Lähettäjä: Soijuv Lähetetty: 26.5.2006 17:18
Tutkijoiden mukaan neuroborrelioosi on yleinen eikä sitä tunnisteta useinkaan oikein. Tanskalaisessa tutkimuksessa seurattiin (vuosina 1985 - 1990) 187 neuroborrelioosia sairastavaa henkilöä. Suurimmalla osalla (160) esiintyi hermojuuritulehduksesta johtuvia oireita esim. vatsan ja/tai kaulan-hartioiden alueella.
Tapausselostuksen naisella esiintyi kovaa, polttavaa kipua vatsan alueella. Kipu säteili selkään ja kylkiin. Kivut pahenivat öisin. Kipu tuntuu joillakin vyömäisenä vartalon ympärillä.
Myös Bleiweissin mukaan yksi borrelioosin oireista saattaa esim. olla lihasheikkous, ristiselkäkipu, lihaskrampit,välilevyn esiinluiskahdus, spinaali stenoosi,vatsalihasten tulehdus jne.
The Clinical and Epidemiological Profile of Lyme Neuroborreliosis in
Denmark 1985-1990
A prospective study of 187 patients with Borrelia burgdorferi specific
intrathecal antibody production
by Klaus Hanson and Anne-Mette Lebech (from the Borrelia Laboratory,
Department of Infection-Immunology, Statens Seruminstitut, Copenhagen,
Denmark)
"Case 2ll: painful radiculitis without focal neurological signs
" A 59-year old female was admitted to a surgical department in August 1990
with a 10 d history of severe deep burning pain in the right abdominal flank
irradiatiing to the back and right thigh. A kidney stone was suspected but a
pyelogram was normal. Over the following two months she was readmitted to a
department of internal medicine with continuous excruciating burning pain
with pronounced nocturnal exacerbation. Three weeks before admission she had
noticed a 10x15 cm large, well-demarcated, slightly painful non-vesicular
erythema in the right abdominal flank. During her second admission she was
treated with morphine, aciclovir and carbamazepine as a herpes zoster
neuralgia was suspected. None of these drugs were effective.....
"Painful sensory radiculitis:
" Radiculitic pain was a major symptom and present in 160
patients....The intensity of radicular pain increased significantly with
the age of the individual..The onset was in most instances subacute,
occurring overnight. Pain often began in the region of a previous erythema
migrans, then migrated and finally became most pronounced axially in the
back typically between the shoulder blades but also in the neck or lumbar
region. Some patients had migrating pain almost over the whole body. The
pain was described as being of a type never experienced before and was
easily distinguished from ordinary back pains "The intensity of the pain often varied
from day to day and typically showed severe nocturnal exacerbations
......Pain was described by most patients as severe, burning deep and/or
superficial as if located in the skin, and often accompanied by patchy
areas of unpleasant hyper- and dysaesthesiae . Occasionally a belt-like
sensation around the trunk was described. Severe pain was refractory to
morphine. Forty-six patients had radiculitic pain only and never developed
focal motor signs. In 54 patients the sensory radiculitis either
diminished or disappeared even before therapy after 2-16 weeks."
"Before the final diagnosis of neuroborreliosis was established, the
painful condition was often thought to be due to other diseases, e.g.herpes
zoster neuralgia, cervical or lumbar nerve root compression, facet
syndrome, brachial plexus neuropathy, polymyalgia rheumatica, myocardial
infarction or kidney concrements (stones). In one female a cholesystectomy
(gall bladder removal) was performed , three patients underwent biopsy of
the temporal lobe , three and i.v. pyelogram (radiograph of the ureter and
renal pelvis), seven a bone scintigraphy and 17 a myelogram , before the
final diagnosis . Routine examination of the CSF obtained during the
myelography unexpectedly revealed pleocytosis leading to the correct
diagnosis.
" Some patients presented with an agitated mental state. This was
probably due to severe pain condition and deprivation of sleep for days to
probably due to severe pain condition and deprivation of sleep for days to
weeks. Thirteen patients were initially suspected of being hysterical and
several were examined by a psychiatrist because of the apparent
diproportion between thier dramatic complaints and the lack of signs of the
disease.
....Two had paresis of the abdominal wall. The distribution of paretic
muscles usually indicated involvement of multiple nerve roots. Tendon
reflexes were attenuated or absent only corresponding to the muscle
weakness.........spastic bladder paresis.....clinical signs of meningitis
are remarkably rare....
"We conclude that neuroborreliosis is a common, clinically
characteristic but still underrecognized neurological syndrome.
.....differential diagnosis: Guillain-Barre syndrome, herpes zoster
radiculitis, lymphocytic meningitis of other aetiologies, meningeal
carcinomatosis (widespread dissemination of carcinoma in the body), CNS
lymphoma, sarcoidosis, multiple sclerosis and other encephalopathies."
------------------------------------------------------------------------------------------------------
When to Suspect Lyme Disease by Dr. John Bleiweiss
".......Muscle weakness occurs in some LD patients, More commonly,
exertional performance is limited by shortness of breath, poor
coordination, musculoskeletal discomfort, or fatigue. Exercise without or
early in treatment can precipitate a flare of fatigue, especially the
following day.
Low back ache can arise from sacroilitis, paravertebral lumbosacral
muscle strain/spasm, and herniated vertebral discs. In a few patients,
spinal stenosis is encountered.
Inflammation of the abdominal wall muscles, in particular the rectus
abdominus is not an infrequent problem. Usually the tender sites are
focal, involving more often than not, the lateral borders of the rectus
abdominus muscle. Nausea is often present as well. The sites are best
located when the patient is performing a Valsalva maneuver or doing a
partial sit up. Failure to appreciate the abdominal myositis may lead to
avoidable extensive GI workup. ........"
OIRE: HERMOJUURITULEHDUS/VATSAKIVUT
Valvojat: Jatta1001, Borrelioosiyhdistys, Bb
OIRE: HERMOJUURITULEHDUS/VATSAKIVUT
Viimeksi muokannut Bb, Pe Maalis 06, 2009 21:43. Yhteensä muokattu 1 kertaa.
Lähettäjä: Soijuv Lähetetty: 26.5.2006 17:18
Tutkijoiden mukaan neuroborrelioosi on yleinen eikä sitä tunnisteta useinkaan oikein. Tanskalaisessa tutkimuksessa seurattiin (vuosina 1985 - 1990) 187:ää Neuroborrelioosia sairastavaa henkilöä. Suurimmalla osalla (160) esiintyi hermojuuritulehduksesta johtuvia oireita esim vatsan ja/tai kaulan-hartioiden alueella.
Tapausselostuksen naisella esiintyi kovaa, polttavaa kipua vatsan alueella. Kipu säteili selkään ja kylkiin. Kivut pahenivat öisin. Kipu tuntuu joillakin vyömäisenä vartalon ympärillä.
Myös Bleiweissin mukaan yksi borrelioosin oireista saattaa esim. olla lihasheikkous, ristiselkäkipu, lihaskrampit, välilevyn esiinluiskahdus, spinaali stenoosi, vatsalihasten tulehdus jne
The Clinical and Epidemiological Profile of Lyme Neuroborreliosis in
Denmark 1985-1990
A prospective study of 187 patients with Borrelia burgdorferi specific
intrathecal antibody production
by Klaus Hanson and Anne-Mette Lebech (from the Borrelia Laboratory,
Department of Infection-Immunology, Statens Seruminstitut, Copenhagen,
Denmark)
"Case 2ll: painful radiculitis without focal neurological signs
" A 59-year old female was admitted to a surgical department in August 1990
with a 10 d history of severe deep burning pain in the right abdominal flank
irradiatiing to the back and right thigh. A kidney stone was suspected but a
pyelogram was normal. Over the following two months she was readmitted to a
department of internal medicine with continuous excruciating burning pain
with pronounced nocturnal exacerbation. Three weeks before admission she had
noticed a 10x15 cm large, well-demarcated, slightly painful non-vesicular
erythema in the right abdominal flank. During her second admission she was
treated with morphine, aciclovir and carbamazepine as a herpes zoster
neuralgia was suspected. None of these drugs were effective.....
"Painful sensory radiculitis:
" Radiculitic pain was a major symptom and present in 160
patients....The intensity of radicular pain increased significantly with
the age of the individual..The onset was in most instances subacute,
occurring overnight. Pain often began in the region of a previous erythema
migrans, then migrated and finally became most pronounced axially in the
back typically between the shoulder blades but also in the neck or lumbar
region. Some patients had migrating pain almost over the whole body. The
pain was described as being of a type never experienced before and was
easily distinguished from ordinary back pains "The intensity of the pain often varied
from day to day and typically showed severe nocturnal exacerbations
......Pain was described by most patients as severe, burning deep and/or
superficial as if located in the skin, and often accompanied by patchy
areas of unpleasant hyper- and dysaesthesiae . Occasionally a belt-like
sensation around the trunk was described. Severe pain was refractory to
morphine. Forty-six patients had radiculitic pain only and never developed
focal motor signs. In 54 patients the sensory radiculitis either
diminished or disappeared even before therapy after 2-16 weeks."
"Before the final diagnosis of neuroborreliosis was established, the
painful condition was often thought to be due to other diseases, e.g.herpes
zoster neuralgia, cervical or lumbar nerve root compression, facet
syndrome, brachial plexus neuropathy, polymyalgia rheumatica, myocardial
infarction or kidney concrements (stones). In one female a cholesystectomy
(gall bladder removal) was performed , three patients underwent biopsy of
the temporal lobe , three and i.v. pyelogram (radiograph of the ureter and
renal pelvis), seven a bone scintigraphy and 17 a myelogram , before the
final diagnosis . Routine examination of the CSF obtained during the
myelography unexpectedly revealed pleocytosis leading to the correct
diagnosis.
" Some patients presented with an agitated mental state. This was
probably due to severe pain condition and deprivation of sleep for days to
probably due to severe pain condition and deprivation of sleep for days to
weeks. Thirteen patients were initially suspected of being hysterical and
several were examined by a psychiatrist because of the apparent
diproportion between thier dramatic complaints and the lack of signs of the
disease.
....Two had paresis of the abdominal wall. The distribution of paretic
muscles usually indicated involvement of multiple nerve roots. Tendon
reflexes were attenuated or absent only corresponding to the muscle
weakness.........spastic bladder paresis.....clinical signs of meningitis
are remarkably rare....
"We conclude that neuroborreliosis is a common, clinically
characteristic but still underrecognized neurological syndrome.
.....differential diagnosis: Guillain-Barre syndrome, herpes zoster
radiculitis, lymphocytic meningitis of other aetiologies, meningeal
carcinomatosis (widespread dissemination of carcinoma in the body), CNS
lymphoma, sarcoidosis, multiple sclerosis and other encephalopathies."
-----------------------------------------------------------------------------
When to Suspect Lyme Disease by Dr. John Bleiweiss
".......Muscle weakness occurs in some LD patients, More commonly,
exertional performance is limited by shortness of breath, poor
coordination, musculoskeletal discomfort, or fatigue. Exercise without or
early in treatment can precipitate a flare of fatigue, especially the
following day.
Low back ache can arise from sacroilitis, paravertebral lumbosacral
muscle strain/spasm, and herniated vertebral discs. In a few patients,
spinal stenosis is encountered.
Inflammation of the abdominal wall muscles, in particular the rectus
abdominus is not an infrequent problem. Usually the tender sites are
focal, involving more often than not, the lateral borders of the rectus
abdominus muscle. Nausea is often present as well. The sites are best
located when the patient is performing a Valsalva maneuver or doing a
partial sit up. Failure to appreciate the abdominal myositis may lead to
avoidable extensive GI workup. ........"
________________________________________________________________
Lähettäjä: Soijuv Lähetetty: 26.5.2006 17:33
Lisää artikkeleita hermojuuritulehduksesta.
Clin Rheumatol 2001;20(6):447-50
Abdominal wall weakness and lumboabdominal pain revealing neuroborreliosis: a report of three cases.
Mormont E, Esselinckx W, De Ronde T, Hanson P, Deltombe T, Laloux P.
Cliniques Universitaires UCL de Mont-Godinne, Universite Catholique de
Louvain, Yvoir, Belgium.
The authors report three cases of thoracic radiculoneuropathy disclosing
neuroborreliosis. All three patients had low back and abdominal pain and two
had marked abdominal wall paresis. EMG confirmed a motor involvement of the
lower thoracic roots and CSF analysis revealed a lymphocytic meningitis in
all
three cases. Antibodies against Borrelia burgdorferi were present in both the
serum and the CSF. A favourable outcome was obtained in all three patients
with
appropriate antibiotherapy. The differential diagnosis of this misleading
presentation is discussed.
PMID: 11771534 [PubMed - in process
__________________________
"....In patients from an
area with a high incidence of Lyme disease it is recommended to exclude
neuroborreliosis even in patients with known diabetes mellitus in order to
avoid the misdiagnosis of diabetic thoracoabdominal radiculopathy. This has
occured in 2 of our patients. ..."
Title:[Thoraco-abdominal manifestation of stage II Lyme neuroborreliosis]
Authors:Pfadenhauer K, Schonsteiner T, Stohr MSource:Nervenarzt 1998
Apr;69(4):296-9
Organization:Neurologische Klinik mit klinischer Neurophysiologie,
Zentralklinikum Augsburg.
Abstract:
In the past little attention was paid to the thoracoabdominal manifestation
of
Lyme radiculoneuritis, because paralysis of the abdominal wall muscles was
considered to be a very uncommon clinical manifestation of Lyme
neuroborreliosis. In a group of 90 patients suffering from early stage Lyme
neuroborreliosis we found abdominal wall weakness in 11 cases. In the
majority
of patients thoracoabdominal radiculoneuritis was located in the lower
thoracic
segments (Th 7-12) and involved more than 3 segments (62%) mostly bilaterally
(69%). Abdominal wall paralysis was mostly bilateral (91%) and involved
always
the lower half of the abdominal wall. It was very severe in 18%.
Electromyographic studies were done in the paraspinal and abdominal wall
muscles showing fibrillation potentials and positive sharp waves in 86% and
50%, respectively. Diagnosis of thoracoabdominal radiculoneuritis in Lyme
neuroborreliosis may be difficult and diagnostic errors may occur. Therefore
we
recommend to look carefully for paralysis of the abdominal wall, which can
easily be overlooked on routine neurological examination. In patients from an
area with a high incidence of Lyme disease it is recommended to exclude
neuroborreliosis even in patients with known diabetes mellitus in order to
avoid the misdiagnosis of diabetic thoracoabdominal radiculopathy. This has
occured in 2 of our patients.
Language: Ger
Unique ID: 98269524
___________________
".......Expanding abdominal girth can be an unusual
manifestation of the polyradiculoneuropathy associated with Lyme disease..."
title:Lyme polyradiculneuropathy presenting as increasing abdominal girth
[Bantam medical dictionary-"polyradiculoneuropathy-any disorder of the
peripheral nerves in which the brunt of the disease falls on the nerve roots
where they emerge from the spinal cord.]
from: Neurology 1990; 40:373-375
authors: Daffner, Saver, et al
Article abstract: "We describe a patient with documented Lyme disease whose
major complaint was increasing abdominal distention. Electrophysiologic
studies demonstrated denervation of the lower thoracic paraspinal muscles and
the rectus abdominis. Expanding abdominal girth can be an unusual
manifestation of the polyradiculoneuropathy associated with Lyme disease.
"Neurologic manifestations of Lyme disease most typically include
meningitis, cranial neuropathies, especially facial palsies, and
polyradiculopathies. Encephalitis, cerebellar ataxia, chorea, and transverse
myelitis [as though the spinal cord has been severed] are less common
features.
Most often, radiculoneuropathic symtoms are composed of pain, dysthesias,
and
limb weakness. We report a patient presenting with an unusual manifestation
of
a Lyme polyradiculoneuropathy ---dramatic abdominal distention.
"Case report-A 60 year-old, right handed man, devoted to muscle
enlarging
and toning exercise, developed excessive fatigue, spiking fever, and chills
while summering on Cape Cod. After a few weeks he defervesced but complained
of "sandpaper and tingling" dysesthesias involving the chest, back , and
abdomen bilaterally, and the right thigh. Four weeks after the onset of his
illness, he noted progressive abdominal distention despite no alteration in
diet and continued efforts to adhere to his exercise regime. This formerly
trim and fit man became increasingly upset over his growing abdomen. In the
course of his illness, his pants waist size increased from 34 to 42,
requiring
him to purchase a new wardrobe. Previously, he performed 100 sit ups every
morning; now, he could
do none. There was no significant alteration in dietary or bowel habits.
Cathartics did not reduce his abdominal bloating. Six weeks after his
symptoms
began, he awoke with frank right facial weakness and was referred for
neurologic consultation.
"He was unaware of exposure to ticks, but recalled many insect bites. A
pruritic, erythematous rash on the right forearm, which he attributed
to poison ivy , had occurred several months prior to presentation. Medical
history was notable for nephrolithiasis, glaucoma, and vocal cord polyps.
There was no family history of neurologic impairment.
"Examination disclosed a well-developed man with normal vital signs.
His
abdomen was protuberant, but soft and nontender, without organmegaly. Anal
spincter tone was normal. Mental status was normal.
Cranial nerve exam demonstrated moderate weakness of the right side of the
face, including the brow and lower face, with impaired taste sensation of the
right anterior tongue. Motor exam was normal. There was no ataxia. DTRs
were
1+. Plantar responses were equivocal bilaterally. Sensation was preserved
for
joint position, vibration, and light touch. He reported dysesthetic
sensations
when the skin over his chest and abdomen was stroked...........
"Discussion, Our patient exhibited many of the salient features of 2-nd
stage Lyme disease, including dysesthesias, facial palsy, and csf
pleocytosis.
Systemic symptoms, as he experienced weeks prior to the onset of his
neurologic
complaints, and the absence of a documented history of erythema migrans, are
NOT UNCOMMON in Lyme disease.
[references for this last statement----
1. Pachner, Steere. The triad of neurologic manifestations of Lyme disease:
meningitis, cranial neuritis, and radiculoneuritis. Neurology 1985;35:47-53.
2. Finkel, MF. Lyme disease and its neurologic complications. Arch of
Neuro
1988;45:99-104.
3. Reik, Burgdorfer, Donaldson. Neurologic abnormalities in
Lyme disease without erythema chronica migrans. Am J Med. 1986;81:73-78]
__________________________
"Elavated IgM and IgG titers against B. burgdorferi in serum and SCSF
confirmed
the diagnosis.
"The patient's most persistent complaint during his illness was abdominal
distention with associated discomfort and weakness. Although his abdominal
girth increased markedly and he lost the ability to perform sit-up exercises,
he exhibited neither significant weight gain nor change in eating or bowel
habits. Common causes of abdominal distention such as ileus, colonic
obstruction....were ruled out by an extensive workup.
"The most parsimonious explanation for this patient's expanding abdominal
girth is a lower thoracic polyradiculoneuropathy secondary to
neuroborreliosis.
Supporting this interpretation are EMG/NCS that revealed denervation of
thoracic paraspinal muscles as well as the rectus abdominis and the gradual
resolution of his symptoms and signs after effective antibiotic
treatment.......
"Polyradiculopathies rarely have been reported to lead to marked
abdominal
distention. Diabetes occasionally causes a syndrome of thoraco-abdominal
neuropathy involving multiple thoracic roots. However, this entity most
typically produces sensory loss and pain and only rarely weakness of
abdominal
or intercostal muscles. One explanation is that the oblique and rectus
abdominis muscles, innervated by T7-L1 and T7-T12, respectively, receive a
multisegmented supply and thus rarely manifest clinical weakness. We believe
that our patient is the first fully documented example of
polyradiculoneuropathy of Lyme disease causing abdominal weakness and
distention. While approximatly 1/3 of Lyme disease patients with neurologic
manifestations develop peripheral neuropathic symptoms, these typically
include
a sensory radiculoneuropathy of the thorax and a sensory-motor
radiculoneuropathy of the extremities. The thoracic radiculopathy, which
preferentially involves the lower thoracic roots, most commonly presents as
intense pain or pressure within a few dermatomes, at times associated with
hypoesthesia.
"...Ackerman et al noted that 1 patient of 100 had paresis of abdominal
muscles, apparently
without sensory disturbances or cranial neuropathy. This series was
collected
between 1956 and 1983, before serologic confirmation of diagnosis was
available, so the diagnosis of Bannwarth's syndrome was rendered on clinical
grounds..
"The fundamental pathophysiologic mechanisms of Lyme
polyradiculoneuropathy remain uncertain. .....We suspect that if patients
with
the more common thoracic dysesthesias were subjected to electrophysiologic
studies of paraspinal and rectus abdominis muscles, subtle, clinically
imperceptible abnormalities would be demonstrated."
____________________________________
"........the patient experienced pain in her left
shoulder and progressive weakness of both upper extremities, along
with persistent low dorsal pain, with numbness in the area of the
abdominal wall in which she had previously experienced girdling pain.
She also had difficulty voiding. ...."
American Journal of Medicine
-----------------------------------------------------------------------------
Volume 103(6): December 1997: pp 544-547
-----------------------------------------------------------------------------
Respiratory Failure due to Lyme Meningoradiculitis
[Brief Clinical Observations]
Sigler, Stephen MD; Kershaw, Paul MD; Scheuch, Robert MD; Sklarek,
Howard MD; Halperin, John MD
Southampton Hospital, Southampton, New York (Sigler; Kershaw; Scheuch;
Sklarek).
Neurology Department, North Shore University Hospital, Manhasset, New
York (Halperin).
-----------------------------------------------------------------------------
A well-documented case of a severe, progressive paralytic syndrome,
with respiratory failure and residual paralysis, due to Lyme
meningoradiculitis is presented here for the first time in the
American literature. The importance of early diagnosis of Lyme disease
is emphasized, with a high index of suspicion in an endemic area and
use of sensitive immunodiagnostic technology. The diagnostic value of
differential cerebrospinal fluid and serum antibody studies for IgG
and IgM is emphasized. The usefulness of electrodiagnostic studies in
ruling out acute inflammatory demyelinating polyradiculoneuropathy is
described.
More than 50% of patients with Lyme disease present with the
characteristic localized rash known as erythema chronicum migrans.
Others may present with multiple skin lesions or a "summer flu"
syndrome of headache, myalgias, and fever, indicating early bacterial
dissemination. If Borrelia burgdorferi infection is not diagnosed and
adequately treated in this early phase, the disease may progress, and
secondary manifestations, such as heart block or meningitis, may
occur. As many as 15% to 20% of infected patients may develop
neurologic manifestations, of which the most common is meningitis with
or without superimposed cranial or peripheral neuropathies. [1]
The major causes of a progressive paralytic syndrome in Lyme disease
are described by Finkel as encephalitis, myelitis, cranial neuritis,
and radiculoneuritis or meningoradiculitis. [2] Lyme encephalitis and
myelitis are rare. Seventh cranial nerve palsies, with or without
meningitis, are the form of paralytic neuropathy most frequently seen
by physicians. In lymphocytic meningoradiculitis
(Garin-Boujadoux-Bannwarth syndrome), more common in Europe than in
the United States, there is, in addition to a mononuclear meningitis,
frequently an asymmetric motor, sensory, or mixed neuropathy, with
electrophysiologic evidence suggesting predominantly axonal
involvement. [3] Although several papers indicate that serious
paralysis may occur in this syndrome, [2-4] detailed case descriptions
are rare. [5,6] There is only one report, in the European literature,
of diaphragmatic paralysis in Lyme disease, in a patient who
subsequently succumbed to a pulmonary embolism. [7]
The following case history is the first report in the American
literature of a well-documented case of Lyme lymphocytic
meningoradiculitis in which there was respiratory paralysis due to
disseminated peripheral nerve involvement. Prolonged intubation and
subsequent tracheostomy were required owing to respiratory failure.
This case dramatically demonstrates the potentially lethal effects of
what is ordinarily a benign illness and should sensitize the physician
to the importance of early diagnosis and treatment of Lyme disease. It
also illustrates the helpfulness of electrophysiologic studies in
differential diagnosis and the usefulness of CSF antibody measurement
in determining the presence of CNS B burgdorferi infection and its
response to antibiotics.
CASE REPORT
This 59-year-old woman was admitted to the hospital with a 1-week
history of weakness of the upper extremities. She had been well until
this time, except for a history of meningococcemia with meningitis at
age 21. Six weeks prior to admission, she had a 5 x 8 cm red swelling
of the entire dorsum of her left foot around what appeared to the
examining doctor to be an infected insect bite. There was no "bulls
eye" appearance to the rash and no history of tick attachment,
although she admitted to gardening recently. Two weeks after the
erythematous rash subsided with short-term antibiotic treatment
(erythromycin for 5 days), the patient experienced severe low dorsal
back pain with bilateral radiation to the mid-abdominal area,
following mild exercise. There was no headache, photophobia, or neck
stiffness at any time. Radiographs of the dorsal spine showed no
evidence of compression fracture.
Over the next 2 weeks the patient experienced pain in her left
shoulder and progressive weakness of both upper extremities, along
with persistent low dorsal pain, with numbness in the area of the
abdominal wall in which she had previously experienced girdling pain.
She also had difficulty voiding.
Physical examination revealed mild tachypnea, with clear lungs to
auscultation. She denied headache, and her neck was supple. Cranial
nerve examination was unremarkable with no evidence of facial paresis.
No weakness of neck flexion was noted. There was marked weakness of
deltoid, biceps, and brachioradialis muscles bilaterally, left
(strength 1/5) more than right (strength 2/5). Deep tendon reflexes
were trace in both arms, 2+ in both legs, and plantar responses were
flexor bilaterally. The patient was able to stand and ambulate with
assistance. There was hypesthesia involving the T4-T12 dermatomes and
weakness of the rectus abdominis muscles bilaterally.
There was a 500 cc urine residual on catheterization. Vital capacity
was 1 L, with a negative inspiratory force (NIF) of -35. Arterial
blood gases demonstrated hypoxemia, with pH 7.41, pO2 64.1, pCO2 41.3,
and O2 saturation 92%. Chest roentgenogram was remarkable for
elevation of the left hemidiaphagm. Magnetic resonance imaging of the
cervical and dorsal spine were negative except for mild posterior disc
bulging at the C5-6 level with minimal effacement of the anterior
thecal sac.
Complete blood cell count and chemistry panel were normal. Lumbar
puncture revealed clear fluid under normal pressure with 54 white
blood cells (all lymphocytes), 30 red blood cells, a negative gram
stain and cultures for bacteria, fungi, and tuberculosis, glucose of
40 (blood sugar 90), and protein of 209. Cerebrospinal fluid (CSF)
cytology was negative for malignant cells; there were frequent
reactive lymphocytes, monocytes, and plasma cells. There were four
oligoclonal bands in the spinal fluid, and myelin basic protein was
negative, a combination of findings that has been reported in Lyme
lymphocytic meningoradiculitis. [8]
Lyme serology (Table I) by antibody capture assay (IMUGEN, Norwood,
Massachusetts) revealed, in the CSF, IgM > 10.4, IgG > 19.1.
Corresponding serum values were IgM > 10.4, IgG 1.8; the high
CSF/serum IgG ratio (>19.1:1.8 ) was considered indicative of
intrathecal antibody production. [9] IgG Western Blot showed bands at
65, 57, 41, 39, and 30kD, positive by CDC/ASTPHLD criteria. With the
positive ELISA and Western blot, active CSF, and high CSF specific
antibody, the patient was felt to have Lyme meningoradiculitis.
Initial nerve conduction studies (Table II) demonstrated slightly
prolonged median F wave latencies. The presence of numerous cells in
the CSF, urinary retention, preservation of lower extremity reflexes,
and the prominent radicular-type pain mitigated against acute
inflammatory demyelinating polyradiculoneuropathy (AIDP). However,
because of the early F wave changes and fairly rapid progression of
her neurologic symptoms, the patient was started on high-dose
intravenous (IV) gammaglobulin (0.4 g/kg/day for 5 days), an
alternative to plasmapheresis for the treatment of Guillain-Bare
syndrome, [10] in addition to IV ceftriaxone 2 g daily for Lyme
meningoradiculoneuritis.
[Help with image viewing]
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Table II. Nerve Conduction Studies
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Despite the administration of IV ceftriaxone and high-dose intravenous
gammaglobulin, the patient's motor weakness and paresthesias failed to
improve, and her respiratory status dramatically deteriorated over the
next 3 days. There were increasing tachypnea and dyspnea, with VC
falling to 500 cc and NIF to -20. On the fourth hospital day she
required intubation and ventilatory support. Follow-up nerve
conduction studies at that time (Table II) were normal except for an
unobtainable right median F wave and slightly reduced lower extremity
compound muscle action potential amplitudes. Sensory nerve action
potential amplitudes and latencies were normal in all studies.
Because of failure of the patient to ventilate adequately after
intubation for 14 days, a tracheotomy had to be performed, which
remained in place until the gradual improvement of her respiratory
mechanics permitted its removal 2 weeks later. Over this same period
there was gradual improvement of strength in the deltoids, biceps,
brachioradialis and abdominal wall muscles, but persistent hypesthesia
of the abdominal wall; voiding normalized. Repeat lumbar puncture
after 3 weeks of IV antibiotics revealed 100 lymphocytes, with protein
having fallen to 91. Lyme antibody capture assay of the spinal fluid
(Table I) revealed a persistently high IgG, with a dramatic fall in
IgM, from >10.4 to 1.2. Over the same period, there had been a lesser
drop in serum IgM, from >10.4 to 4.9.
The patient had a total of 8 weeks of IV antibiotics, a more prolonged
course than the usual 3 to 4 weeks because of the severity of her
symptomatology and increasing CSF pleocytosis. Twelve weeks after the
onset of this patient's neurologic symptoms, examination revealed some
recovery of upper extremity strength. Upper extremity strength was on
the left 2/5 (previously 1/5), and on the right 3/5 (previously 2/5).
Nerve conduction studies were normal, but electromyelography (EMG)
revealed abnormal spontaneous activity and long duration polyphasic
motor units in the C5 and C6 myotomes, consistent with acute and
chronic denervation. After 6 months of intensive physical therapy, she
had completely recovered muscle strength and bladder control, though
abdominal wall hypesthesia persisted. She is able to garden again.
DISCUSSION
This patient presented with typical Lyme lymphocytic
meningoradiculitis, or Garin-Bujadoux-Bann-warth syndrome,
characterized by meningitis (in this instance clinically asymptomatic
but with CSF lymphocytosis and protein and specific IgG elevation) and
painful asymmetric motor and sensory radiculoneuropathy. This
multilevel involvement led to profound respiratory compromise possibly
due to phrenic nerve involvement (elevated left hemidiaphragm on chest
xray) as well as weakness of thoracically and cervically innervated
accessory muscles of respiration, severe bilateral upper extremity
weakness, hypesthesia in the thoracic distribution, and urinary
retention. Her respiratory failure and significant neurologic residua
demonstrate the severity with which the disease can attack the nervous
system, a severity rarely reported in this country.
This case emphasizes the importance of a high index of suspicion for
Lyme disease in an endemic area. The erythematous rash surrounding an
insect bite of the foot might have alerted the physician to the
possibility of atypical EM. Too often a skin rash is diagnosed as
cellulitis and treated with a short course of antibiotics while Lyme
disease progresses. An early antibody capture assay for Lyme IgM
antibodies might have avoided misdiagnosis, and should be considered
in the presence of any atypical rash of flu syndrome in an endemic
area.
Further, this case demonstrates the usefulness of serum and CSF
antibody studies in determining the presence of Lyme
meningoradiculitis. Although a high CSF/serum IgG ratio does not
necessarily mean active CNS disease, in the context of the CSF
pleocytosis and elevated protein, it strongly suggests active Lyme CNS
infection. The demonstration of a high Lyme IgM titer in the initial
serum and CSF samples, together with lesser elevations of serum IgG
titers, further support acute Borrelia infection as the source of this
patient's symptomatology. The marked decrease in CSF IgM after
treatment may be evidence for resolution of the CNS infection, despite
the persistent high ratio of CSF to serum IgG, which has been reported
to persist for months to years after successful treatment. [4]
Another lesson from this case is the usefulness of nerve conduction
studies and EMG, complementing clinical presentation and CSF findings,
in distinguishing Lyme meningoradiculitis from AIDP as the cause of
this patient's profound weakness. Although initial F-wave changes
raised the possibility of demyelination, follow-up EMG and nerve
conduction studies were more suggestive of predominantly axonal
involvement. Similar findings have been well documented in the
literature of Lyme neuroborreliosis. [11]
Manuscript submitted March 18, 1997, and accepted in revised form
September 13, 1997.
REFERENCES
1. Steere AC. Lyme disease. NEJM. 1989;321(9):586-596. [Medline Link]
[Context Link]
2. Finkel MF. Progressive paralytic disorders associated with Lyme
disease. Semin Neurol. 1993;13:299-304. [Medline Link] [BIOSIS
Previews Link] [Context Link]
3. Halperin JJ, Little BW, Coyle PK, Dattwyler RJ. Lyme disease-a
treatable cause of peripheral neuropathy. Neurology.
1987;37:1700-1706. [Medline Link] [Context Link]
4. Hansen K, Lebech A-M. The clinical and epidemiological profile of
Lyme neuroborreliosis in Denmark 1985-1990. Brain. 1992;115:399-423.
[Medline Link] [Context Link]
5. Sterman AB, Nelson S, Barclay P. Demyelinating neuropathy
accompanying Lyme disease. Neurology. 1982;32:1302-1305. [Medline
Link] [Context Link]
6. Garin-Boujadoux C. Paralysie par les tiques. J Med Lyon.
1922;71:765-767. [Context Link]
7. Melet M, Gerard A, Voiriot P, Gayet S, et al. Fatal
meningoradiculitis in Lyme disease. Presse Medicale. 1986;15:2075.
[Medline Link] [Context Link]
8. Henriksson A, Link H, Cruz M, Stiernstedt G. Immunoglobulin
abnormalities in cerebrospinal fluid and blood over the course of
lymphocytic meningoradiculitis (Bannwarth's syndrome). Ann Neurol.
1986;20:337-345. [Medline Link] [Context Link]
9. Halperin JJ, Luft BJ, Anand AK, et al. Lyme neuroborreliosis:
central nervous system manifestations. Neurology. 1989;39(6):753-759.
[Medline Link] [PsycINFO Link] [Context Link]
10. Jackson M, Godwin-Austern R, Whitely A. High dose intravenous
immunoglobulin in the treatment of Guillain-Barre syndrome: a
preliminary open study. J Neurol. 1993;240:51-53. [Medline Link]
[BIOSIS Previews Link] [Context Link]
11. Halperin JJ, Luft BJ, Volkman DJ, Dattwyler RJ. Lyme
neuroborreliosis-peripheral nervous system manifestations. Brain.
1990;113:1207-1221. [Medline Link] [Context
Tutkijoiden mukaan neuroborrelioosi on yleinen eikä sitä tunnisteta useinkaan oikein. Tanskalaisessa tutkimuksessa seurattiin (vuosina 1985 - 1990) 187:ää Neuroborrelioosia sairastavaa henkilöä. Suurimmalla osalla (160) esiintyi hermojuuritulehduksesta johtuvia oireita esim vatsan ja/tai kaulan-hartioiden alueella.
Tapausselostuksen naisella esiintyi kovaa, polttavaa kipua vatsan alueella. Kipu säteili selkään ja kylkiin. Kivut pahenivat öisin. Kipu tuntuu joillakin vyömäisenä vartalon ympärillä.
Myös Bleiweissin mukaan yksi borrelioosin oireista saattaa esim. olla lihasheikkous, ristiselkäkipu, lihaskrampit, välilevyn esiinluiskahdus, spinaali stenoosi, vatsalihasten tulehdus jne
The Clinical and Epidemiological Profile of Lyme Neuroborreliosis in
Denmark 1985-1990
A prospective study of 187 patients with Borrelia burgdorferi specific
intrathecal antibody production
by Klaus Hanson and Anne-Mette Lebech (from the Borrelia Laboratory,
Department of Infection-Immunology, Statens Seruminstitut, Copenhagen,
Denmark)
"Case 2ll: painful radiculitis without focal neurological signs
" A 59-year old female was admitted to a surgical department in August 1990
with a 10 d history of severe deep burning pain in the right abdominal flank
irradiatiing to the back and right thigh. A kidney stone was suspected but a
pyelogram was normal. Over the following two months she was readmitted to a
department of internal medicine with continuous excruciating burning pain
with pronounced nocturnal exacerbation. Three weeks before admission she had
noticed a 10x15 cm large, well-demarcated, slightly painful non-vesicular
erythema in the right abdominal flank. During her second admission she was
treated with morphine, aciclovir and carbamazepine as a herpes zoster
neuralgia was suspected. None of these drugs were effective.....
"Painful sensory radiculitis:
" Radiculitic pain was a major symptom and present in 160
patients....The intensity of radicular pain increased significantly with
the age of the individual..The onset was in most instances subacute,
occurring overnight. Pain often began in the region of a previous erythema
migrans, then migrated and finally became most pronounced axially in the
back typically between the shoulder blades but also in the neck or lumbar
region. Some patients had migrating pain almost over the whole body. The
pain was described as being of a type never experienced before and was
easily distinguished from ordinary back pains "The intensity of the pain often varied
from day to day and typically showed severe nocturnal exacerbations
......Pain was described by most patients as severe, burning deep and/or
superficial as if located in the skin, and often accompanied by patchy
areas of unpleasant hyper- and dysaesthesiae . Occasionally a belt-like
sensation around the trunk was described. Severe pain was refractory to
morphine. Forty-six patients had radiculitic pain only and never developed
focal motor signs. In 54 patients the sensory radiculitis either
diminished or disappeared even before therapy after 2-16 weeks."
"Before the final diagnosis of neuroborreliosis was established, the
painful condition was often thought to be due to other diseases, e.g.herpes
zoster neuralgia, cervical or lumbar nerve root compression, facet
syndrome, brachial plexus neuropathy, polymyalgia rheumatica, myocardial
infarction or kidney concrements (stones). In one female a cholesystectomy
(gall bladder removal) was performed , three patients underwent biopsy of
the temporal lobe , three and i.v. pyelogram (radiograph of the ureter and
renal pelvis), seven a bone scintigraphy and 17 a myelogram , before the
final diagnosis . Routine examination of the CSF obtained during the
myelography unexpectedly revealed pleocytosis leading to the correct
diagnosis.
" Some patients presented with an agitated mental state. This was
probably due to severe pain condition and deprivation of sleep for days to
probably due to severe pain condition and deprivation of sleep for days to
weeks. Thirteen patients were initially suspected of being hysterical and
several were examined by a psychiatrist because of the apparent
diproportion between thier dramatic complaints and the lack of signs of the
disease.
....Two had paresis of the abdominal wall. The distribution of paretic
muscles usually indicated involvement of multiple nerve roots. Tendon
reflexes were attenuated or absent only corresponding to the muscle
weakness.........spastic bladder paresis.....clinical signs of meningitis
are remarkably rare....
"We conclude that neuroborreliosis is a common, clinically
characteristic but still underrecognized neurological syndrome.
.....differential diagnosis: Guillain-Barre syndrome, herpes zoster
radiculitis, lymphocytic meningitis of other aetiologies, meningeal
carcinomatosis (widespread dissemination of carcinoma in the body), CNS
lymphoma, sarcoidosis, multiple sclerosis and other encephalopathies."
-----------------------------------------------------------------------------
When to Suspect Lyme Disease by Dr. John Bleiweiss
".......Muscle weakness occurs in some LD patients, More commonly,
exertional performance is limited by shortness of breath, poor
coordination, musculoskeletal discomfort, or fatigue. Exercise without or
early in treatment can precipitate a flare of fatigue, especially the
following day.
Low back ache can arise from sacroilitis, paravertebral lumbosacral
muscle strain/spasm, and herniated vertebral discs. In a few patients,
spinal stenosis is encountered.
Inflammation of the abdominal wall muscles, in particular the rectus
abdominus is not an infrequent problem. Usually the tender sites are
focal, involving more often than not, the lateral borders of the rectus
abdominus muscle. Nausea is often present as well. The sites are best
located when the patient is performing a Valsalva maneuver or doing a
partial sit up. Failure to appreciate the abdominal myositis may lead to
avoidable extensive GI workup. ........"
________________________________________________________________
Lähettäjä: Soijuv Lähetetty: 26.5.2006 17:33
Lisää artikkeleita hermojuuritulehduksesta.
Clin Rheumatol 2001;20(6):447-50
Abdominal wall weakness and lumboabdominal pain revealing neuroborreliosis: a report of three cases.
Mormont E, Esselinckx W, De Ronde T, Hanson P, Deltombe T, Laloux P.
Cliniques Universitaires UCL de Mont-Godinne, Universite Catholique de
Louvain, Yvoir, Belgium.
The authors report three cases of thoracic radiculoneuropathy disclosing
neuroborreliosis. All three patients had low back and abdominal pain and two
had marked abdominal wall paresis. EMG confirmed a motor involvement of the
lower thoracic roots and CSF analysis revealed a lymphocytic meningitis in
all
three cases. Antibodies against Borrelia burgdorferi were present in both the
serum and the CSF. A favourable outcome was obtained in all three patients
with
appropriate antibiotherapy. The differential diagnosis of this misleading
presentation is discussed.
PMID: 11771534 [PubMed - in process
__________________________
"....In patients from an
area with a high incidence of Lyme disease it is recommended to exclude
neuroborreliosis even in patients with known diabetes mellitus in order to
avoid the misdiagnosis of diabetic thoracoabdominal radiculopathy. This has
occured in 2 of our patients. ..."
Title:[Thoraco-abdominal manifestation of stage II Lyme neuroborreliosis]
Authors:Pfadenhauer K, Schonsteiner T, Stohr MSource:Nervenarzt 1998
Apr;69(4):296-9
Organization:Neurologische Klinik mit klinischer Neurophysiologie,
Zentralklinikum Augsburg.
Abstract:
In the past little attention was paid to the thoracoabdominal manifestation
of
Lyme radiculoneuritis, because paralysis of the abdominal wall muscles was
considered to be a very uncommon clinical manifestation of Lyme
neuroborreliosis. In a group of 90 patients suffering from early stage Lyme
neuroborreliosis we found abdominal wall weakness in 11 cases. In the
majority
of patients thoracoabdominal radiculoneuritis was located in the lower
thoracic
segments (Th 7-12) and involved more than 3 segments (62%) mostly bilaterally
(69%). Abdominal wall paralysis was mostly bilateral (91%) and involved
always
the lower half of the abdominal wall. It was very severe in 18%.
Electromyographic studies were done in the paraspinal and abdominal wall
muscles showing fibrillation potentials and positive sharp waves in 86% and
50%, respectively. Diagnosis of thoracoabdominal radiculoneuritis in Lyme
neuroborreliosis may be difficult and diagnostic errors may occur. Therefore
we
recommend to look carefully for paralysis of the abdominal wall, which can
easily be overlooked on routine neurological examination. In patients from an
area with a high incidence of Lyme disease it is recommended to exclude
neuroborreliosis even in patients with known diabetes mellitus in order to
avoid the misdiagnosis of diabetic thoracoabdominal radiculopathy. This has
occured in 2 of our patients.
Language: Ger
Unique ID: 98269524
___________________
".......Expanding abdominal girth can be an unusual
manifestation of the polyradiculoneuropathy associated with Lyme disease..."
title:Lyme polyradiculneuropathy presenting as increasing abdominal girth
[Bantam medical dictionary-"polyradiculoneuropathy-any disorder of the
peripheral nerves in which the brunt of the disease falls on the nerve roots
where they emerge from the spinal cord.]
from: Neurology 1990; 40:373-375
authors: Daffner, Saver, et al
Article abstract: "We describe a patient with documented Lyme disease whose
major complaint was increasing abdominal distention. Electrophysiologic
studies demonstrated denervation of the lower thoracic paraspinal muscles and
the rectus abdominis. Expanding abdominal girth can be an unusual
manifestation of the polyradiculoneuropathy associated with Lyme disease.
"Neurologic manifestations of Lyme disease most typically include
meningitis, cranial neuropathies, especially facial palsies, and
polyradiculopathies. Encephalitis, cerebellar ataxia, chorea, and transverse
myelitis [as though the spinal cord has been severed] are less common
features.
Most often, radiculoneuropathic symtoms are composed of pain, dysthesias,
and
limb weakness. We report a patient presenting with an unusual manifestation
of
a Lyme polyradiculoneuropathy ---dramatic abdominal distention.
"Case report-A 60 year-old, right handed man, devoted to muscle
enlarging
and toning exercise, developed excessive fatigue, spiking fever, and chills
while summering on Cape Cod. After a few weeks he defervesced but complained
of "sandpaper and tingling" dysesthesias involving the chest, back , and
abdomen bilaterally, and the right thigh. Four weeks after the onset of his
illness, he noted progressive abdominal distention despite no alteration in
diet and continued efforts to adhere to his exercise regime. This formerly
trim and fit man became increasingly upset over his growing abdomen. In the
course of his illness, his pants waist size increased from 34 to 42,
requiring
him to purchase a new wardrobe. Previously, he performed 100 sit ups every
morning; now, he could
do none. There was no significant alteration in dietary or bowel habits.
Cathartics did not reduce his abdominal bloating. Six weeks after his
symptoms
began, he awoke with frank right facial weakness and was referred for
neurologic consultation.
"He was unaware of exposure to ticks, but recalled many insect bites. A
pruritic, erythematous rash on the right forearm, which he attributed
to poison ivy , had occurred several months prior to presentation. Medical
history was notable for nephrolithiasis, glaucoma, and vocal cord polyps.
There was no family history of neurologic impairment.
"Examination disclosed a well-developed man with normal vital signs.
His
abdomen was protuberant, but soft and nontender, without organmegaly. Anal
spincter tone was normal. Mental status was normal.
Cranial nerve exam demonstrated moderate weakness of the right side of the
face, including the brow and lower face, with impaired taste sensation of the
right anterior tongue. Motor exam was normal. There was no ataxia. DTRs
were
1+. Plantar responses were equivocal bilaterally. Sensation was preserved
for
joint position, vibration, and light touch. He reported dysesthetic
sensations
when the skin over his chest and abdomen was stroked...........
"Discussion, Our patient exhibited many of the salient features of 2-nd
stage Lyme disease, including dysesthesias, facial palsy, and csf
pleocytosis.
Systemic symptoms, as he experienced weeks prior to the onset of his
neurologic
complaints, and the absence of a documented history of erythema migrans, are
NOT UNCOMMON in Lyme disease.
[references for this last statement----
1. Pachner, Steere. The triad of neurologic manifestations of Lyme disease:
meningitis, cranial neuritis, and radiculoneuritis. Neurology 1985;35:47-53.
2. Finkel, MF. Lyme disease and its neurologic complications. Arch of
Neuro
1988;45:99-104.
3. Reik, Burgdorfer, Donaldson. Neurologic abnormalities in
Lyme disease without erythema chronica migrans. Am J Med. 1986;81:73-78]
__________________________
"Elavated IgM and IgG titers against B. burgdorferi in serum and SCSF
confirmed
the diagnosis.
"The patient's most persistent complaint during his illness was abdominal
distention with associated discomfort and weakness. Although his abdominal
girth increased markedly and he lost the ability to perform sit-up exercises,
he exhibited neither significant weight gain nor change in eating or bowel
habits. Common causes of abdominal distention such as ileus, colonic
obstruction....were ruled out by an extensive workup.
"The most parsimonious explanation for this patient's expanding abdominal
girth is a lower thoracic polyradiculoneuropathy secondary to
neuroborreliosis.
Supporting this interpretation are EMG/NCS that revealed denervation of
thoracic paraspinal muscles as well as the rectus abdominis and the gradual
resolution of his symptoms and signs after effective antibiotic
treatment.......
"Polyradiculopathies rarely have been reported to lead to marked
abdominal
distention. Diabetes occasionally causes a syndrome of thoraco-abdominal
neuropathy involving multiple thoracic roots. However, this entity most
typically produces sensory loss and pain and only rarely weakness of
abdominal
or intercostal muscles. One explanation is that the oblique and rectus
abdominis muscles, innervated by T7-L1 and T7-T12, respectively, receive a
multisegmented supply and thus rarely manifest clinical weakness. We believe
that our patient is the first fully documented example of
polyradiculoneuropathy of Lyme disease causing abdominal weakness and
distention. While approximatly 1/3 of Lyme disease patients with neurologic
manifestations develop peripheral neuropathic symptoms, these typically
include
a sensory radiculoneuropathy of the thorax and a sensory-motor
radiculoneuropathy of the extremities. The thoracic radiculopathy, which
preferentially involves the lower thoracic roots, most commonly presents as
intense pain or pressure within a few dermatomes, at times associated with
hypoesthesia.
"...Ackerman et al noted that 1 patient of 100 had paresis of abdominal
muscles, apparently
without sensory disturbances or cranial neuropathy. This series was
collected
between 1956 and 1983, before serologic confirmation of diagnosis was
available, so the diagnosis of Bannwarth's syndrome was rendered on clinical
grounds..
"The fundamental pathophysiologic mechanisms of Lyme
polyradiculoneuropathy remain uncertain. .....We suspect that if patients
with
the more common thoracic dysesthesias were subjected to electrophysiologic
studies of paraspinal and rectus abdominis muscles, subtle, clinically
imperceptible abnormalities would be demonstrated."
____________________________________
"........the patient experienced pain in her left
shoulder and progressive weakness of both upper extremities, along
with persistent low dorsal pain, with numbness in the area of the
abdominal wall in which she had previously experienced girdling pain.
She also had difficulty voiding. ...."
American Journal of Medicine
-----------------------------------------------------------------------------
Volume 103(6): December 1997: pp 544-547
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Respiratory Failure due to Lyme Meningoradiculitis
[Brief Clinical Observations]
Sigler, Stephen MD; Kershaw, Paul MD; Scheuch, Robert MD; Sklarek,
Howard MD; Halperin, John MD
Southampton Hospital, Southampton, New York (Sigler; Kershaw; Scheuch;
Sklarek).
Neurology Department, North Shore University Hospital, Manhasset, New
York (Halperin).
-----------------------------------------------------------------------------
A well-documented case of a severe, progressive paralytic syndrome,
with respiratory failure and residual paralysis, due to Lyme
meningoradiculitis is presented here for the first time in the
American literature. The importance of early diagnosis of Lyme disease
is emphasized, with a high index of suspicion in an endemic area and
use of sensitive immunodiagnostic technology. The diagnostic value of
differential cerebrospinal fluid and serum antibody studies for IgG
and IgM is emphasized. The usefulness of electrodiagnostic studies in
ruling out acute inflammatory demyelinating polyradiculoneuropathy is
described.
More than 50% of patients with Lyme disease present with the
characteristic localized rash known as erythema chronicum migrans.
Others may present with multiple skin lesions or a "summer flu"
syndrome of headache, myalgias, and fever, indicating early bacterial
dissemination. If Borrelia burgdorferi infection is not diagnosed and
adequately treated in this early phase, the disease may progress, and
secondary manifestations, such as heart block or meningitis, may
occur. As many as 15% to 20% of infected patients may develop
neurologic manifestations, of which the most common is meningitis with
or without superimposed cranial or peripheral neuropathies. [1]
The major causes of a progressive paralytic syndrome in Lyme disease
are described by Finkel as encephalitis, myelitis, cranial neuritis,
and radiculoneuritis or meningoradiculitis. [2] Lyme encephalitis and
myelitis are rare. Seventh cranial nerve palsies, with or without
meningitis, are the form of paralytic neuropathy most frequently seen
by physicians. In lymphocytic meningoradiculitis
(Garin-Boujadoux-Bannwarth syndrome), more common in Europe than in
the United States, there is, in addition to a mononuclear meningitis,
frequently an asymmetric motor, sensory, or mixed neuropathy, with
electrophysiologic evidence suggesting predominantly axonal
involvement. [3] Although several papers indicate that serious
paralysis may occur in this syndrome, [2-4] detailed case descriptions
are rare. [5,6] There is only one report, in the European literature,
of diaphragmatic paralysis in Lyme disease, in a patient who
subsequently succumbed to a pulmonary embolism. [7]
The following case history is the first report in the American
literature of a well-documented case of Lyme lymphocytic
meningoradiculitis in which there was respiratory paralysis due to
disseminated peripheral nerve involvement. Prolonged intubation and
subsequent tracheostomy were required owing to respiratory failure.
This case dramatically demonstrates the potentially lethal effects of
what is ordinarily a benign illness and should sensitize the physician
to the importance of early diagnosis and treatment of Lyme disease. It
also illustrates the helpfulness of electrophysiologic studies in
differential diagnosis and the usefulness of CSF antibody measurement
in determining the presence of CNS B burgdorferi infection and its
response to antibiotics.
CASE REPORT
This 59-year-old woman was admitted to the hospital with a 1-week
history of weakness of the upper extremities. She had been well until
this time, except for a history of meningococcemia with meningitis at
age 21. Six weeks prior to admission, she had a 5 x 8 cm red swelling
of the entire dorsum of her left foot around what appeared to the
examining doctor to be an infected insect bite. There was no "bulls
eye" appearance to the rash and no history of tick attachment,
although she admitted to gardening recently. Two weeks after the
erythematous rash subsided with short-term antibiotic treatment
(erythromycin for 5 days), the patient experienced severe low dorsal
back pain with bilateral radiation to the mid-abdominal area,
following mild exercise. There was no headache, photophobia, or neck
stiffness at any time. Radiographs of the dorsal spine showed no
evidence of compression fracture.
Over the next 2 weeks the patient experienced pain in her left
shoulder and progressive weakness of both upper extremities, along
with persistent low dorsal pain, with numbness in the area of the
abdominal wall in which she had previously experienced girdling pain.
She also had difficulty voiding.
Physical examination revealed mild tachypnea, with clear lungs to
auscultation. She denied headache, and her neck was supple. Cranial
nerve examination was unremarkable with no evidence of facial paresis.
No weakness of neck flexion was noted. There was marked weakness of
deltoid, biceps, and brachioradialis muscles bilaterally, left
(strength 1/5) more than right (strength 2/5). Deep tendon reflexes
were trace in both arms, 2+ in both legs, and plantar responses were
flexor bilaterally. The patient was able to stand and ambulate with
assistance. There was hypesthesia involving the T4-T12 dermatomes and
weakness of the rectus abdominis muscles bilaterally.
There was a 500 cc urine residual on catheterization. Vital capacity
was 1 L, with a negative inspiratory force (NIF) of -35. Arterial
blood gases demonstrated hypoxemia, with pH 7.41, pO2 64.1, pCO2 41.3,
and O2 saturation 92%. Chest roentgenogram was remarkable for
elevation of the left hemidiaphagm. Magnetic resonance imaging of the
cervical and dorsal spine were negative except for mild posterior disc
bulging at the C5-6 level with minimal effacement of the anterior
thecal sac.
Complete blood cell count and chemistry panel were normal. Lumbar
puncture revealed clear fluid under normal pressure with 54 white
blood cells (all lymphocytes), 30 red blood cells, a negative gram
stain and cultures for bacteria, fungi, and tuberculosis, glucose of
40 (blood sugar 90), and protein of 209. Cerebrospinal fluid (CSF)
cytology was negative for malignant cells; there were frequent
reactive lymphocytes, monocytes, and plasma cells. There were four
oligoclonal bands in the spinal fluid, and myelin basic protein was
negative, a combination of findings that has been reported in Lyme
lymphocytic meningoradiculitis. [8]
Lyme serology (Table I) by antibody capture assay (IMUGEN, Norwood,
Massachusetts) revealed, in the CSF, IgM > 10.4, IgG > 19.1.
Corresponding serum values were IgM > 10.4, IgG 1.8; the high
CSF/serum IgG ratio (>19.1:1.8 ) was considered indicative of
intrathecal antibody production. [9] IgG Western Blot showed bands at
65, 57, 41, 39, and 30kD, positive by CDC/ASTPHLD criteria. With the
positive ELISA and Western blot, active CSF, and high CSF specific
antibody, the patient was felt to have Lyme meningoradiculitis.
Initial nerve conduction studies (Table II) demonstrated slightly
prolonged median F wave latencies. The presence of numerous cells in
the CSF, urinary retention, preservation of lower extremity reflexes,
and the prominent radicular-type pain mitigated against acute
inflammatory demyelinating polyradiculoneuropathy (AIDP). However,
because of the early F wave changes and fairly rapid progression of
her neurologic symptoms, the patient was started on high-dose
intravenous (IV) gammaglobulin (0.4 g/kg/day for 5 days), an
alternative to plasmapheresis for the treatment of Guillain-Bare
syndrome, [10] in addition to IV ceftriaxone 2 g daily for Lyme
meningoradiculoneuritis.
[Help with image viewing]
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Table II. Nerve Conduction Studies
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Despite the administration of IV ceftriaxone and high-dose intravenous
gammaglobulin, the patient's motor weakness and paresthesias failed to
improve, and her respiratory status dramatically deteriorated over the
next 3 days. There were increasing tachypnea and dyspnea, with VC
falling to 500 cc and NIF to -20. On the fourth hospital day she
required intubation and ventilatory support. Follow-up nerve
conduction studies at that time (Table II) were normal except for an
unobtainable right median F wave and slightly reduced lower extremity
compound muscle action potential amplitudes. Sensory nerve action
potential amplitudes and latencies were normal in all studies.
Because of failure of the patient to ventilate adequately after
intubation for 14 days, a tracheotomy had to be performed, which
remained in place until the gradual improvement of her respiratory
mechanics permitted its removal 2 weeks later. Over this same period
there was gradual improvement of strength in the deltoids, biceps,
brachioradialis and abdominal wall muscles, but persistent hypesthesia
of the abdominal wall; voiding normalized. Repeat lumbar puncture
after 3 weeks of IV antibiotics revealed 100 lymphocytes, with protein
having fallen to 91. Lyme antibody capture assay of the spinal fluid
(Table I) revealed a persistently high IgG, with a dramatic fall in
IgM, from >10.4 to 1.2. Over the same period, there had been a lesser
drop in serum IgM, from >10.4 to 4.9.
The patient had a total of 8 weeks of IV antibiotics, a more prolonged
course than the usual 3 to 4 weeks because of the severity of her
symptomatology and increasing CSF pleocytosis. Twelve weeks after the
onset of this patient's neurologic symptoms, examination revealed some
recovery of upper extremity strength. Upper extremity strength was on
the left 2/5 (previously 1/5), and on the right 3/5 (previously 2/5).
Nerve conduction studies were normal, but electromyelography (EMG)
revealed abnormal spontaneous activity and long duration polyphasic
motor units in the C5 and C6 myotomes, consistent with acute and
chronic denervation. After 6 months of intensive physical therapy, she
had completely recovered muscle strength and bladder control, though
abdominal wall hypesthesia persisted. She is able to garden again.
DISCUSSION
This patient presented with typical Lyme lymphocytic
meningoradiculitis, or Garin-Bujadoux-Bann-warth syndrome,
characterized by meningitis (in this instance clinically asymptomatic
but with CSF lymphocytosis and protein and specific IgG elevation) and
painful asymmetric motor and sensory radiculoneuropathy. This
multilevel involvement led to profound respiratory compromise possibly
due to phrenic nerve involvement (elevated left hemidiaphragm on chest
xray) as well as weakness of thoracically and cervically innervated
accessory muscles of respiration, severe bilateral upper extremity
weakness, hypesthesia in the thoracic distribution, and urinary
retention. Her respiratory failure and significant neurologic residua
demonstrate the severity with which the disease can attack the nervous
system, a severity rarely reported in this country.
This case emphasizes the importance of a high index of suspicion for
Lyme disease in an endemic area. The erythematous rash surrounding an
insect bite of the foot might have alerted the physician to the
possibility of atypical EM. Too often a skin rash is diagnosed as
cellulitis and treated with a short course of antibiotics while Lyme
disease progresses. An early antibody capture assay for Lyme IgM
antibodies might have avoided misdiagnosis, and should be considered
in the presence of any atypical rash of flu syndrome in an endemic
area.
Further, this case demonstrates the usefulness of serum and CSF
antibody studies in determining the presence of Lyme
meningoradiculitis. Although a high CSF/serum IgG ratio does not
necessarily mean active CNS disease, in the context of the CSF
pleocytosis and elevated protein, it strongly suggests active Lyme CNS
infection. The demonstration of a high Lyme IgM titer in the initial
serum and CSF samples, together with lesser elevations of serum IgG
titers, further support acute Borrelia infection as the source of this
patient's symptomatology. The marked decrease in CSF IgM after
treatment may be evidence for resolution of the CNS infection, despite
the persistent high ratio of CSF to serum IgG, which has been reported
to persist for months to years after successful treatment. [4]
Another lesson from this case is the usefulness of nerve conduction
studies and EMG, complementing clinical presentation and CSF findings,
in distinguishing Lyme meningoradiculitis from AIDP as the cause of
this patient's profound weakness. Although initial F-wave changes
raised the possibility of demyelination, follow-up EMG and nerve
conduction studies were more suggestive of predominantly axonal
involvement. Similar findings have been well documented in the
literature of Lyme neuroborreliosis. [11]
Manuscript submitted March 18, 1997, and accepted in revised form
September 13, 1997.
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