A case of distal lower motor neuron syndrome associated with IgM anti-GM1 antibodies

A 34-year-old man had noted progressive weakness in his right hand. On admission at age 39, cranial nerves were not involved. Fasciculations were observed in his upper limb girdles. Neurological examination revealed severe wasting and weakness of arms and the right hand, whereas mild in the left hand. The deep tendon reflexes were absent in the upper extremities, but normal in the lower extremities. No sensory disturbances were observed. Motor and sensory nerve conduction velocities were normal, and multifocal conduction block was not observed. EMG showed neuropathic changes in all 4 limbs and sternocleidomastoideus muscles. Serum immunoelectrophoresis failed to detect an M protein. High-performance thin-layer chromatography with immunostaining revealed that his serum IgM reacted with GM1, but not reacted with GM2, GD1a, GD1b, and asialo-GM1.     

Association of antibodies to ganglioside complexes and conduction blocks in axonal Guillain‐Barré syndrome presenting as acute motor conduction block neuropathy

A close relationship between acute motor conduction block neuropathy and antibodies against the complex of GM1 and GalNAc‐GD1a has been reported. This study investigates the hypothesis that conduction block at the early phase of axonal Guillain‐Barré syndrome (GBS) is also associated with such ganglioside complexes. Sera were obtained from seven French patients with initial evidence of isolated conduction blocks that resolved or progressed to acute motor axonal neuropathy. Serum IgG to asialo‐GM1 and gangliosides of LM1, GM1, GM1b, GD1a, GalNAc‐GD1a, GD1b, GT1a, GT1b, and GQ1b as well as their complexes were measured. Five of seven patients progressed within the first month of disease to AMAN. One patient had IgG antibodies against the complex of asialo‐GM1 and each of the other ganglioside antigens. Another patient carried IgG antibodies against GM1 complex with GM1b, GD1a, and GT1a as well as asialo‐GM1 complex with GD1a and GT1a. None had IgG antibodies against GM1/GalNAc‐GD1a complex. Six patients had IgG against single antigens GM1, GD1a, GalNAc‐GD1a, GD1b, and asialo‐GM1. In three patients, a reduced reaction against GM1/GalNAc‐GD1a complex was observed. The presence of conduction block in axonal GBS is not always associated with anti‐GM1/GalNAc‐GD1a complex antibodies.     

An acute axonal polyneuropathy affecting intrinsic hand muscles following Campylobacter infection--a case report]

A 24-year-old carpenter had the shakes and fever on March 13, 1990. He suffered from watery diarrhea on March 14 and 15. He left muscle weakness in his thumbs and fingers when he drove nails with a hammer on March 24. The weakness reached maximum by the 3rd day of illness. He was admitted to our hospital on day 4. Neurological examination revealed symmetrical weakness localized in the intrinsic hand muscles (MRC grade 2-4). The deep tendon reflexes were preserved. Sensation was intact except for mild disturbance of superficial sense on both plantar areas. Campylobacter jejuni was cultured from his stool. A complement fixation test indicated serologically preceding C. jejuni infection. Whereas maximum motor nerve conduction velocities were not reduced and distal latencies were not prolonged, compound muscle action potential recorded in the thenar and hypothenar muscles were remarkably reduced on day 5. Needle EMG showed neuropathic changes in four limbs. Sensory nerve conduction velocities and action potentials were normal. The weakness gradually improved in association with increased compound muscle action potentials in the thenar and hypothenar muscles. His muscle symptom fully resolved 2 months after the onset of his illness. Thin-layer chromatogram with immunostaining revealed that serum IgG from this patient reacted with GM1, GD1a, GD1b, but did not react with GM2 and GT1b. Enzyme-linked immunosorbent assay showed that anti-GM1, GD1a, GD1b antibodies titer (IgG) decreased concurrently with the clinical improvement.     

Sensorimotor demyelinating neuropathy with IgM antibody against gangliosides GD1a, GT1b and GM3

We report a patient with sensorimotor demyelinating neuropathy with high-titer IgM antibody against gangliosides GD1a, GT1b and GM3. The patient was a 65-year-old male who was hospitalized with chief complaints of muscular weakness of all limbs and numbness of the hands and feet. Nerve-conduction studies revealed reduced conduction velocities of the motor nerves with increased temporal dispersion and loss of sensory nerve action potentials. Treatment with steroids was ineffective. IgM antibody against GD1a, GT1b and GM3, which are known to be the ligands for myelin-associated glycoprotein (MAG), might have played a role in the demyelination in this patient by inhibiting adhesion between myelin and axonal membrane.     

Motor-dominant neuropathy with multifocal conduction block

Patient 1 was a 39-year-old man; patient 2, a 42-year-old woman; patient 3, a 78-year-old man. Leading symptoms were chronic asymmetrical weakness in all three cases, which started in a distal portion of the upper extremities. Muscle atrophy was often less prominent than would be expected from the power of the muscle. Fasciculations were observed in two patients and the initial symptom of patient 2 was painful cramp of the right thumb. Patient 1 initially had mild transient dysesthesia of the right fingers. The other two patients had no sensory symptoms or signs. General laboratory tests revealed no particular abnormalities except that patient 3 had mild diabetes mellitus, although the type of neuropathy in patient 3 was quite different from diabetic neuropathy. Total protein concentrations in the cerebrospinal fluid were 34, 32 and 43 mg/dl in three patients, respectively (normally, less than 40 mg/dl). Motor nerve conduction studies revealed conduction block in more than one nerve in every case. Conduction velocities were generally normal in those segments of nerve where conduction block was not detected. Serum anti-ganglioside antibodies were investigated by Enzyme-linked immunosorbent assay (ELISA). Glycolipids used as the antigen include GM1, GM2, GM3, GD1b, GD3, GT1b, GQ1b, GA1 and galactocerebroside. Strong IgM antibody activity against GM1, GD1b and GA1 was noted in patient 1. Weaker but significant IgM antibody activities against GM1 and GA1 were detected in patient 2 and 3. Thin-layer chromatography immunostaining also confirmed these results. Muscle biopsy in patient 1 revealed a lot of target fibers and profuse polyglucosan bodies in the axons of intramuscular nerves.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case report of Bickerstaff's brainstem encephalitis with positive anti GQ 1 b, GT 1 a, GM 1 ganglioside antibodies]

Patient was an 18-year-old female student. After she had symptoms of common cold for 3 days, she developed somnolence, diplopia, dysarthria, urinary disturbance and ataxia. On admission neurological examination revealed coma with mydriasis, ophthalmoplegia, ptosis and weakness of the upper limbs. Light reflex, corneal reflex and oculocephalic test were all negative. Deep tendon reflexes were brisk and extensor toe signs were positive bilaterally. She did not have nuchal rigidity. Laboratory test revealed normal cerebrospinal fluid with negative myelin basic protein. Brain MRI, brainstem evoked potentials presented no abnormality. EMG revealed normal conduction velocity and no conduction block. EEG had diffuse theta and delta slowing. Culture of the stool represented no Campylobacter jejuni. At the fifth day of admission consciousness level improved, and other neurological findings disappeared in about 6 weeks. She had anti GQ 1 b, GT 1 a(IgG, IgM) and anti GM 1(IgM) antibodies in the serum. We made a diagnosis of Bickerstaff's brainstem encephalitis from these neurological symptoms and clinical course. The main lesion was present in the brainstem from midbrain to medulla oblongata in the midline. High titer of anti GT 1a antibody may be related to the ophthalmoplegia as noted in Miller Fisher syndrome. As a result of EMG and stool culture, it denied the complication of Guillain-Barré syndrome. We had no proof of the reason of the presentation of anti GM 1 antibody.     

Acute demyelinating sensorimotor polyneuropathy in B‐cell lymphoma with IGM autoantibodies against glycolipid GD1B

A 20‐year‐old man developed weakness without sensory complaints ten days after rubella. Examination showed limb weakness and brisk tendon reflexes but no sensory abnormalities. Laboratory investigations revealed IgG and IgM anti‐Rubella and increased CSF protein content (0,8 g/L). Electrophysiological examination showed partial motor conduction blocks in eight nerves and normal sensory conductions even across the sites of CB. Brain and spinal cord MRI and SEPs were normal. The patient was treated with four plasmaphereses and fully recovered in six months. Conduction blocks gradually improved with increasing duration and abnormal temporal dispersion in proximal CMAPs. GBS has been rarely reported after rubella. Anti‐myelin basic protein antibodies have been found in a patient with a relapsing motor neuropathy following rubella vaccination. As antibodies cross‐reacted with a viral protein, molecular mimicry has been proposed as a pathophysiological mechanism. In our patient we did not find anti‐MBP antibodies and antibodies to‐glycolipids (GM1, GM2, GA1, GD1a, GD1b, GQ1b, sulfatides, galactocerebroside) were also negative. Indirect immunofluorescence after incubation of patient's serum on rabbit sciatic nerve and human sural nerve and roots was negative. Our patient confirms the occurrence of GBS following Rubella and shows some uncommon features: 1) hyperactive deep tendon reflexes; 2) demyelination selectively involving motor fibres; and 3) widespread early conduction blocks in intermediate nerve segments.     

Immunostaining of motor nerve terminals by IgM M protein with activity against gangliosides GM1 and GD1b from a patient with motor neuron disease

We demonstrated the binding of an IgM monoclonal protein, obtained from a patient with motor neuron disease, with known antibody activity against gangliosides GM1, GD1b, and asialo GM1, to neuromuscular junctions in guinea pig gastrocnemius muscle, using an indirect immunofluorescence technique. Staining disappeared after delipidation of muscle sections. Denervated muscle sections showed no labeling at the neuromuscular junction after incubation with the patient's serum. This indicates presynaptic binding of the IgM M protein and supports the concept that IgM monoclonal antibody to nerve terminal determinants may underlie a motor neuron disorder.     

A case of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome with positive anti-galactocerebroside (Gal-C) IgM antibody]

A 49-year-old man presented with hoarseness, dysphagia, muscle atrophy and weakness of deltoid, trapezius, sternocleidomastoid, rhomboid, anterior serratus, infraspinatus and supraspinatus. Anti-Gal-C IgM antibody was positive in the serum. The other antiganglioside antibodies (GM1, GM2, GM3, GD1a, GD1b, GD3, GT1a, GT1b, GQ1b, GA1, GalNAc-GD1a, GM1b) were negative. Patient contracted pneumonia but whether it was due to mycoplasma was not evident. Plasmapheresis improved his clinical state including a decrease of the antibody. This case was diagnosed pharyngeal-cervical-brachial variant of Guillain-Barré syndrome, and anti-Gal-C antibody seemed to be correlated with the pathogenesis of this syndrome. Gal-C is a major glycolipid of myelin and the cell membrane of the myelin-forming cell (oligodendrocytes and Schwann cells) and is free of specific localization and distribution. The mechanism how the anti-Gal-C IgM antibody induced bulbar paralysis and the symptoms localizing neck and upper limbs remains to be known.     

An acute axonal form of Guillain-Barré syndrome with antibodies against gangliosides GM1 and GD1b--a case report

We reported a case of Guillain-Barré syndrome with autoantibodies against gangliosides GM1 and GD1b, which has not been reported yet. A 25-year-old man was admitted with a 7-day history of acute progressive weakness in the extremities. Two weeks before admission he had suffered from an episode of watery diarrhea. Neurological examination revealed areflexia and tetraparesis without any sensory impairment. Respiration and cranial nerves were not involved. The CSF protein level was 157 mg/dl with normal cellularity on the 14th illness day. The maximum M potential amplitude was 0.1 mV, which was 2% of the lower limit of normal, whereas motor and sensory nerve conduction velocities were normal on day 22. Repeated electrophysiological studies suggested that the predominant process was axonal degeneration. Although he received plasmapheresis at the acute phase, four months after onset he continued to have distal dominant limb weakness with wasting. High-performance thin-layer chromatography with immunostaining revealed that his serum IgG reacted with GM1, GD1b, and asialo-GM1. Enzyme-linked immunosorbent assay showed that anti-glycolipids antibodies titers decreased, concurrent with clinical course. Immunoabsorption study demonstrated that antibodies with anti-GM1 activity were absorbed with liposomes containing purified GD1b, indicating that autoantibodies bind to the galactosyl (beta 1-3) N-acetylgalactosaminyl moiety which is shared by GM1, GD1b, and asialo-GM1. There have been several reports of lower motor neuron diseases with monoclonal IgM antibody to GM1, GD1b, and asialo-GM1. This case suggested that the transient rise in these anti-carbohydrate antibodies may be involved in the pathogenesis of acute axonal degeneration of motor nerve as well as paraproteinemic motor neuron diseases.     

Case of a pharyngeal-cervical-brachial variant of Guillain-Barré syndrome without pharyngeal palsy

We report a case of a 3-year-old boy with acute muscle weakness that initially affected neck and all four limbs but later vanished from the lower limbs. Pharyngeal palsy was not observed during the course. All deep tendon reflexes were absent. Peripheral nerve conduction studies showed a demyelination pattern in each limb. The patient received intravenous high-dose corticosteroid hormone, followed by two immunoglobulin therapies. His muscle strength gradually improved after treatment and was almost completely restored four months later. We ultimately diagnosed the condition as the pharyngeal-cervical-brachial variant of Guillain-Barré syndrome, in consideration of the patient's muscle weakness of the neck and four limbs, the greater degree of weakness of the upper limbs versus the lower limbs. His clinical presentation was atypical for the pharyngeal-cervical-brachial variant of Guillain-Barré syndrome, as he presented no pharyngeal muscle weakness or anti GT1a antibodies, typical manifestations of the condition.     

A case of mortor neuron syndrome with onset 9 months after electrical injury]

A 72-year-old man noticed progressive weakness of both upper limbs, more severe on the left side, 9 months after an electric shock of a 20,000 V alternating current. He had diffuse scars of superficial burns with skin graft in four limbs, more on the right side. A neurological examination revealed diffuse muscle atrophy, weakness and fasciculation in both upper limbs, predominantly on the left side, hyper-reflexia in four limbs with mildly exaggerated jaw jerk, left Babinski sign, and mild decrease of touch and pain sensation in the right C6 and C7 segments. Painful dysesthesia was present in the left hand and right lower limb. The search for serum antibodies against GM1, GM2, GM3, GD1a, Gd1b, GQ1b, GA1, and GT1b was negative. No abnormality except mild cervical spondylotic changes was evident in the magnetic resonance imaging of the brain and spinal cord. The upper limb motor evoked potentials (MEPs) were not elicited by the left cortical stimulation and the central motor conduction time by the right cortical stimulation was remarkably prolonged in the upper limb MEPs. Nerve conduction study showed a delay of motor conduction velocity and distal latency in the right median and bilateral ulnar nerves with low amplitude and delayed velocity of sensory nerves of those nerves. Needle EMG revealed diffuse ongoing denervation potentials in bilateral upper limbs and giant motor unit potentials in the right triceps and first dorsal interossei muscles. These findings indicate that the delayed motor neuron syndrome induced by electrical shock is characteristic for having demyelination as well as axonal changes in both central and peripheral nervous systems.     

Frequency and clinical correlates of anti–neural IgM antibodies in neuropathy associated with IgM monoclonal gammopathy

We studied the frequency and clinical correlates of different IgM specificities in 75 patients with neuropathy associated with IgM monoclonal gammopathy. Patients were tested for IgM reactivity with the myelin-associated glycoprotein, PO, neurofilaments, and tubulin by immunoblot; with GM1, asialo-GM1, GM2, GD1a, GD1b, sulfatide, and chondroitin sulfate C by enzyme-linked immunosorbent assay; and with brain and nerve glycolipids by overlay highperformance thin-layer chromatography. Forty-two patients (56%) had high titers of IgM antibodies to MAG; 4 (5%), to sulfatide (1 also to myelin-associated glycoprotein); 4 (5%), to the 200-kd neurofilament (2 also to myelin-associated protein); and 1 each, to GD1b and chondroitin sulfate C. No reactivity was found in 26 patients (35%). More patients with anti–myelin-associated glycoprotein IgM (62%) than with unknown IgM reactivity (31%) had a predominantly sensory neuropathy (p < 0.025). Nerve conduction findings were consistent with a demyelinating neuropathy in 77% of patients reactive to myelin-associated glycoprotein and 24% with unknown reactivity (p < 0.0001) and the mean conduction velocity of peroneal nerve was lower in the former group (22.9 m/sec) than in the latter group (39.6 m/sec) (p < 0.000001). Patients with anti–sulfatide IgM had a sensorimotor neuropathy with morphological evidence of demyelination while anti–neurofilament IgM was not associated with homogeneous findings. Patients with anti–GD1b or anti–chondroitin sulfate C IgM had a predominantly motor impairment. The frequent occurrence of anti–neural IgM antibodies in neuropathy associated with IgM gammopathy, and their frequent, though not constant association with similar neuropathy features, support their possible pathogenetic role in the neuropathy.     

Anti-GM1 IgM antibodies in motor neuron disease and neuropathy

We found anti-GM1 IgM antibodies in 23% of 56 patients with motor neuron disease (MND), in 19% of 69 patients with neuropathy, and in 7% of 107 controls with other neurologic and nonneurologic diseases. Most of these patients had anti-GM1 IgM antibody titers of 1:80 or less; slightly higher antibody titers (up to 1:640) were found in 3 patients, 1 with MND and 2 with neuropathy, and very high titers (1:20,480) in a patient with MND and an IgM kappa M protein that reacted with GM1, GD1b, and asialo GM1. Six other patients with anti-GM1 IgM that also bound to GD1b. Reactivity with GD1b did not correlate with anti-GM1 titers but was only present in patients with MND or neuropathy. Anti-GM1 IgM antibodies may be a normal constituent of the human antibody repertoire but their frequency and, in some cases, their levels are higher in patients with MND and neuropathy. The origin and the pathogenetic role of these antibodies in neural impairment remain to be established.     

Acute motor conduction block neuropathy pattern occurring in the course of an acute inflammatory demyelinating polyradiculoneuropathy

OBJECTIVE: To describe the case of a young woman with the diagnosis of acute inflammatory demyelinating polyradiculoneuropathy (AIDP), who during the course of the disease developed an electrophysiologic pattern of acute motor conduction block neuropathy (AMCBN). METHODS: Electrophysiologic techniques including needle EMG, standard motor and sensory nerve conductions studies, and somatosensory evoked potentials were carried out over the four months after symptom onset. RESULTS: The results of four neurophysiological studies, performed on Days 14, 26, 35 and 125 after symptomatic onset are reported. All immunological determinations including antiganglioside antibodies (GM1, GM2, GM3, asialoGM1, GD1a, GD1b, GD3, GQ1b and GT1b) were negative. The patient had a favorable evolution following treatment with intravenous immunoglobulins (IVIg). CONCLUSIONS: We conclude that the electrophysiologic hallmark of AMCBN may occur in the course of AIDP. Serial investigation including proximal, intermediate and distal segments of all nerves from upper and lower limbs is essential for its detection.     

Clinical phenotype of patients with neuropathy associated with monoclonal gammopathy: a comparative study and a review of the literature

The objective of this study was to investigate if the clinical and electrophysiological phenotype of patients with polyneuropathy associated with IgM monoclonal gammopathy (IgM-PNP) is related to the presence of antibodies against gangliosides or myelin-associated glycoprotein (MAG). We compared clinical and nerve conduction study (NCS) characteristics of 11 IgM-PNP patients with antibodies against asialo-GM1 or gangliosides (GM1, GD1a, GD1b, GM2 or GQ1b) to 11 consecutive IgM-PNP patients with anti-MAG neuropathy and to 9 IgM-PNP patients without antibodies against either MAG or gangliosides. Patients with anti-ganglioside antibodies could not be differentiated from those with anti-MAG antibodies based on clinical characteristics. However, within the group of anti-ganglioside antibody positive patients, antibodies against GD1b and GQ1b were associated with a purely sensory neuropathy (p = 0.002), while asymmetric weakness with symmetric sensory loss was associated with anti-asialo-GM1 antibodies. In conclusion, polyneuropathy associated with IgM monoclonal gammopathy and anti-ganglioside antibodies clinically resembles anti-MAG neuropathy. Pure sensory neuropathy and marked asymmetry may suggest the presence of anti-ganglioside rather than anti-MAG antibodies.     

CIDP: DESCRIPTION OF FOUR PARTICULAR CASES

Chronic inflammatory demyelinating polineuropathy (CIDP) is an acquired polineuropathy, clinically heterogeneous, and is associated with demyelination of spinal roots and peripheral nerves with a monophasic, progressive or relapsing course.
We describe four patients affected by CIDP with different clinical pictures.
The first patient, a 54-year-old man, complained of a 10 year paresthesia, with a slight wasting and weakening of the right leg, where he showed the absence of deep tendon reflexes and a reduction of the vibration sense.
The second patient, a 21-year-old woman, had distal paresthesia and mild weakness restricted to the left hand. Deep tendon reflexes were reduced and vibration sense was normal.
The third patient, a 41-year-old man, with a 5 year history of muscle cramps more frequent in the upper limbs, showed mild weakness of the right forearm, absence of distal deep tendon reflexes, and a reduction of vibration sense in the lower limbs.
The last patient, a 20-year-old man, showed a marked increase of CPK level, a severe and progressive proximal and distal hypotrophy in the upper and lower limbs, and respiratory distress. Deep tendon reflexes were absent.
Nerve conduction studies showed a reduction of sensory and motor velocity, which was not homogeneous among the patients and among the explored nerves of each patients. Proximal motor conduction blocks were present only in three cases. CSF parameters were normal and antibodies anti nerve were absent in all patients.
The first three patients were treated with IVIg with a clear clinical and electrophysiological improvement, the last patient showed a moderate improvement, only after cyclosporin A treatment .
These four cases confirm that CIDP is clinically heterogeneous with a variable spectrum of symptoms and severity, from a minimal clinical evidence to a severe clinical picture as in our fourth patient.     

A case of Guillain-Barré syndrome (GBS) following human parvovirus B19 infection]

Human parvovirus B19 (HPV-B19) infection is recently known to produce variable clinical manifestations, but neurological disorder associated with this infection is uncommon. We reported a case of GBS associated with transient lupus-like status following HPV-B19 infection. This is the first report describing an adult case of GBS following HPV-B19 infection. A healthy female, aged 33, developed erythema infectiosum simultaneously with her 5-year-old daughter. On the same day, she noticed leg fatigue, which worsened in the following days. On the day 11th, she became unable to walk. Mild pancytopenia, liver injury, proteinuria, hypocomplementemia, and increased anti-nuclear antibody (ANA) were revealed at a hospital. Polymerase chain reaction detected HPV-B19 DNA in the serum. On the day 17th, she was admitted to our hospital because of moderate generalized weakness and mild sensory disturbance, which were symmetrical and distal-dominant. The deep tendon reflexes were absent. Analysis of cerebrospinal fluid showed albuminocytologic dissociation. Other laboratory data were normal except positive ANA. Enzyme-linked immunosorbent assay (ELISA) showed high titer of the serum IgM antibody to GM1 and GD1b. Serum anti HPV-B19 IgM and IgG tested by ELISA were also positive. She improved gradually after 2 courses of double filtrated plasma pheresis.     

A case of brachial amyotrophic diplegia accompanied with Sj?gren's syndrome presenting good response to immunotherapies in the early course of the disease]

A 74-year-old woman suffered from progressive muscle atrophy and weakness of her arms since she was seventy two years old. Before referral to our department, she was diagnosed as having cervical spondylotic myeloradiculopathy and received spinal fusion. Though spinal decompression was successful, muscle weakness of her upper limbs were progressive even after the surgery. On admission, neurological examinations revealed marked atrophy and weakness of her bilateral upper limbs with absent deep tendon reflexes showing man-in-the-barrel syndrome. Her lower extremities had normal muscle strength, but fasciculations were seen in her all four limbs. Electrophysiologically, motor nerve conduction velocity was almost normal but the amplitude was remarkably decreased, conduction block was not detected, and electromyography showed neurogenic patterns on her all extremities. Spinal progressive musclar atrophy (SPMA) accompanied with Sj?gren's syndrome was the likely diagnosis. Because 50 kDa anti-neuronal antibodies were found in her serum, we assumed that anterior horn cells were impaired by an autoimmune mechanism. Thus we treated her with corticosteroid pulse therapy, plasma exchange (PE) and intravenous immunoglobulin infusion therapy (IVIG). Although steroid pulse therapy only had a minimal effect, PE and IVIG promoted a remarkable improvement on her weakness, and the effect lasted for about three months. This is the first case of SPMA with Sj?gren's syndrome which showed good response to PE and IVIG in the early course of the disease. We considered that some SPMA-like motor neuron syndrome accompanied with autoimmune features may require immunomodulating therapies.     

A case of adult T cell leukemia/lymphoma with motor and sensory polyneuropathy]

A 62-year-old man was admitted to our hospital because of two months continuing paresthesia and muscle weakness of distal portions of the four limbs. On general physical examination, skin lesions, lymphadenopathy and hepatosplenomegaly were not found. Neurological examination revealed moderate weakness in the bilateral distal muscles of the lower limbs and left distal muscles of the upper limbs, and slight weakness in the right distal muscles of the upper limbs and the bilateral proximal muscles of the four limbs. Hand grasping powers were 24 kg and 2 kg on the right and left, respectively. The biceps, triceps and radial reflexes were decreased on the right, but normal on the left. The Achilles tendon reflex was decreased on the right and absent on the left. Paresthesia and superficial sensory disturbance were observed with glove and stocking distribution, which was more severe on the left side. The vibration and position senses were slightly decreased in the distal part of the lower limbs. On the laboratory examinations, serum anti-HTLV-I antibody was positive and no abnormal lymphocytes were observed in peripheral blood. Cerebrospinal fluid findings were normal, and anti-HTLV-I antibody was negative. Motor and sensory conduction velocities were normal or slightly decreased in all of the limb nerves examined, but the amplitudes of the compound muscle action potentials and the sensory nerve action potentials were asymmetrically decreased. Needle EMG showed fibrillation potentials and giant spikes with a reduction in number of motor unit potentials. The histological examination of the biopsied sural nerve revealed severe axonal degeneration without evidence of vasculitis or infiltration of abnormal lymphocytes.(ABSTRACT TRUNCATED AT 250 WORDS)