Myeloneuritis Due to Acute Organophosphate (DDVP) Intoxication

Given the importance of agriculture and widespread use of pesticides, intoxication due to organophosphate insecticides is common in Turkey. Organophosphorus compounds may cause late-onset distal polyneuropathy occurring 2 or more weeks after the acute exposure. An 18-year-old woman and a 22-year-old man were admitted to the hospital with weakness, paresthesia, and gait disturbances at 35 and 22 days, respectively, after ingesting dimethyl-2,2-dichloro vinyl phosphate (DDVP). Neurological examination revealed weakness, vibration sense loss, bilateral dropped foot, brisk deep tendon reflexes, and bilaterally positive Babinski sign. Electroneurography demonstrated distal motor polyneuropathy with segmental demyelination associated with axonal degeneration prominent in the distal parts of both lower extremities.     

A case of CIDP with horizontal gaze-evoked nystagmus and ataxia]

A 46-year-old man developed mild to moderate weakness in the distal muscles of lower limbs and then had gradually progressive weakness and sensory loss in four limbs. He subsequently developed difficulty in walking over a few months. Examination showed severe distal muscle weakness and atrophy, but mild proximal weakness in four limbs. Superficial sensation was decreased in both distal limbs and his vibratory sense was mildly decreased in bilateral feet. All tendon reflexes were absent. Furthermore, he showed four-limb and truncal ataxia with bilateral horizontal gaze-evoked nystagmus in both directions. Nerve conduction study revealed sensorimotor neuropathy, and sural nerve biopsy showed mixed axonal damage and demyelination. Cerebrospinal fluid protein levels were raised 212 mg/dl. Lumbar spine MRI showed marked cauda equina enhancement with gadolinium. Anti-ganglioside antibodies were negative but serum antineuronal antibodies without known antigen specificity were found. Neurootological findings indicated bilateral horizontal gaze-evoked nystagmus was caused by spinocerebellar damage. We diagnosed this case was CIDP with cerebellar ataxia. After administration of high dose steroid therapy, intravenous methylprednisolone 1000 mg/day, his symptoms including ataxia and polyneuropathy were apparently improved.     

Chronic inflammatory demyelinating polyneuropathy followed by systemic lupus erythematosus and Sj?gren syndrome: a case report]

We report a 60-year-old man with chronic inflammatory demyelinating polyneuropathy (CIDP) accompanying systemic lupus erythematosus (SLE) and Sj?gren syndrome (SS). He was admitted to our hospital because of progressive weakness and dysesthesia in the distal parts of the bilateral upper and lower extremities. We diagnosed the illness as CIDP and treated him with steroids, plasma filtration and high-dose intravenous immunoglobulin therapy (IvIg). Consequently, his symptoms improved gradually and he was discharged. However, 2 years after the first admission, he experienced dyspnea on effort and chest X-ray demonstrated right pleural effusion. Consequently, he gradually developed gait disturbance, loss of taste, and severe dysesthesia in his lower limbs. Therefore, he was admitted again. On neurological examination, mild weakness was detected in all limbs distally. Deep tendon reflexes were absent. The sensation of position and vibration was diminished in the fingers and toes. He could not walk by himself and demonstrated an ataxic gait with a wide base. Examination of cranial nerves demonstrated no abnormalities, except for loss of taste. Serological examination demonstrated positive auto-antibodies including antinuclear, anti-DNA, and anti-SS-A antibodies. Urinalyses showed albuminuria and microscopic hematuria. Cerebrospinal fluid (CSF) was clear and colorless, containing 3 mononuclear cells per cubic mm, with a protein of 275 mg/dl. Magnetic resonance images (MRIs) of the brain and entire spine were normal. Neurophysiological studies demonstrated an absence of sensory nerve action potentials in the right arm and markedly slow conduction velocities, conduction block in the ulnar forearm segment and absence of F waves in all limbs. The renal biopsy revealed lupus nephritis. The lip biopsy demonstrated chronic sialoadenitis consistent with SS, altough patient did not show symptoms of arthritis or vasculitis. It is well known that mononeuritis multiplex and acute demyelinating polyneuropathy accompany SLE. However there are few reports that describe the occurrence of CIDP in patients with SLE, and the association of "CIDP-like" neuropathy in patients with SS. Our case suggests that the autoimmune disease associated with SLE and SS may induce "CIDP-like" inflammatory demyelinating polyneuropathy.     

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)     

A case of Down's syndrome with chronic inflammatory demyelinating polyradiculoneuropathy

A 19-year-old male patient with Down's syndrome accompanied by relapsing and steroid-dependent chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) was reported. He had been well until the beginning of June 1988, and he rapidly developed a gait disturbance and symmetrical numbness in his arms and legs at the end of that month. He also suffered from difficulty in swallowing and dyspnea soon afterward. A physical examination revealed the specific clinical features of Down's syndrome, but no particular signs. Neurological examination showed the following abnormal findings; fixed nystagmus, diminished gag reflex, flaccid tetraparesis, and distal dominant dysesthesia of upper and lower extremities. Deep tendon reflexes were diminished and Babinski's sign was negative. No autonomic dysfunction was detected. Routine hematology and biochemistry screening test revealed normal level except for increasing GPT and IgG. Protein and gamma globulin values in cerebrospinal fluid (CSF) were elevated without pleocytosis. Nerve conduction velocities were slighty reduced. Pulse therapy and corticosteroid treatment improved on clinical course and CSF results. CIDP probably results from an abnormal immune responsiveness. Down's syndrome has often been taken for autoimmune abnormality. It is of great interest whether there is a close connection of autoimmune abnormality between Down's syndrome and CIDP. This might be the first case report of Down's syndrome associated with CIDP, judging from our survey of the literature in Japan and other countries.     

A variant of Guillain-Barré syndrome with prominent bilateral peripheral facial nerve palsy--facial diplegia and paresthesias]

A patient with Facial diplegia and paresthesias, a rare regional variant of Guillain-Barré syndrome (GBS), is described. A 37-year-old woman developed paresthesias in the distal limbs and subsequently bifacial weakness. She had had a preceding episode of laryngitis. Neurological examination showed severe facial diplegia with loss of taste sensation on the tip of the tongue. Limb muscle power was preserved. Deep tendon reflexes were generally absent. She complained of paresthesias in the distal limbs, but sensory examination was normal. Cerebrospinal fluid showed albuminocytological dissociation. Electrophysiological studies revealed severe facial nerve involvements and demyelinative findings in her limbs. Intravenous immunoglobulin rapidly improved the abnormal sensation in her limbs. Although facial diplegia gradually lessened after the therapy, mild residual weakness was noted 6 months after the neurological onset. To diagnose facial diplegia and paresthesias, it is important to clarify the findings common to typical GBS such as the antecedent illness, acute and monophasic course, distal paresthesias, areflexia, cerebrospinal fluid albuminocytological dissociation, and demyelinative conduction abnormalities in the limbs.     

Neurosarcoidosis with girdle sensation and polyradiculoneuropathy masquerading as Guillain-Barré syndrome]

We herein report two patients with neurosarcoidosis presenting girdle sensation in the trunk and polyradiculoneuritis. The first patient, a 53-year-old woman, manifested subacute progressive paresthesia in all four limbs and below the Th 3 level with girdle sensation from the thorax to lower abdomen and mild weakness in the left upper limb and the bilateral lower limbs. The patient was diagnosed to have sarcoidosis based on a biopsy of the scalenus anticus lymph nodes. The second patient, a 63-year-old woman, showed an acute onset of weakness and paresthesia in all four limbs and girdle sensation from the Th 5 to Th 8 level. On examination, she demonstrated diminished tendon reflexes in all four limbs, mild to severe weakness in all four limbs, paresthesia in all four limbs and below the Th 5 level. Although Guillain-Barré syndrome was initially suspected in this patient, the presence of girdle sensation led us to examine the possibility of neurosarcoidosis. Her examination demonstrated an abnormal accumulation of gallium in the bilateral hilar lymph nodes and mediastinum on scintigraphy, an elevated CD 4/CD 8 ratio in the bronchoalveolar lavage fluid, a negative tuberculin reaction, and elevated serum lysozyme level. These findings thus fulfilled the clinical criteria for sarcoidosis. None of the two patients showed any abnormalities in the thoracic cord MRI. In the first patient F wave was not evoked in either upper or lower limbs, while in the second patient temporal dispersion on M wave was observed in the right median and both ulnar nerves. We therefore consider the girdle sensation to have not been caused by myelopathy but instead by polyradiculopathy. When sarcoid peripheral neuropathy masquerades as Guillain-Barré syndrome, then the presence of girdle sensation may help diagnosis of neurosarcoidosis.     

A case of bilateral brachial plexopathy after median sternotomy]

A 53-year-old man with bilateral brachial plexopathy after median sternotomy for cardiac surgery was described. When he was awakened six days after cardiac surgery, he experienced weakness and paresthesia of both upper limbs. Neurological examination revealed moderate to severe atrophy and weakness of right biceps, brachioradialis, left triceps, and distal muscles of both upper limbs, and paresthesia around the right thumb and the left hypothenar. Deep tendon reflex of the right biceps, triceps, brachioradialis and left triceps was absent. In addition, he showed Horner's syndrome at the left side. The results of needle EMG and nerve conduction study indicated the damage of the right upper trunk and left middle and lower trunks of the brachial plexus. Although brachial plexopathy following median sternotomy has previously been reported in Western literatures, there is only a single report of unilateral brachial plexopathy in Japan. This is the first report in Japan of the bilateral brachial plexopathy following median sternotomy, and suggests that brachial plexopathy should be recognized as a complication of median sternotomy.     

SENSORY‐MOTOR NEUROPATHY,MENTAL RETARDATION,SEIZURES AND KERATOCONUS: A NEW GENETIC SYNDROME?

We describe two siblings, V.A. 30 years old and V.U. 26 years old, affected by an atypical genetic syndrome. Paternal grandfather and grandmother were first cousins. Clinical history revealed, since the first infancy, generalized and partial seizures, drug resistance, and a mental retardation more severe in V.U. Neurological examination showed in V.A. congenital bilateral twisted feet, absence of tendon reflexes, reduction of vibration sense, calf enlargement and fasciculations in lower limbs. Mild ataxia, mild distal wasting more marked in the lower limbs with foot drop, absence of tendon reflexes, reduction of vibration sense and pes cavus were observed in V.U. Cutaneous hyperkeratosis was also present. Electrophysiological study showed in both, axonal sensory-motor neuropathy, confirmed by sural nerve biopsy, performed in V.A. In the same patient the muscle biopsy excluded a mitochondrial dysfunction. Ophthalmologic evaluation demonstrated normal fundus oculi and the presence of keratoconus, monolaterally in V.U. and bilaterally in V.A. Laboratory investigations disclosed mild increase of CK. Furthermore, brain magnetic resonance, brainstem and somatosensory evoked potentials, urinary oligosaccharides, fragile X syndrome analysis, Arylsulfatase-A and lactate levels were normal. Conclusion : The two patients are affected by sensory-motor neuropathy, mental retardation, seizures and keratoconus. This symptom association is not present in medical literature.     

Ataxic form of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

We reported a 64-year-old male with an eight-month history of gait disturbance and sensory impairment. The patient initially noticed unsteadiness of gait and numbness in his feet, and these symptoms progressed until he was unable to walk without assistance five months later. Vibratory sensation and position sense were markedly diminished, and deep tendon reflexes were absent in all extremities. Motor conduction velocities were slow with prolonged distal latencies, and sensory nerve action potentials (SNAP) were not elicited. Sural nerve biopsy revealed a mild loss of myelinated fibres and segmental demyelination. Cerebrospinal fluid showed normal cell count with protein 526 mg/dL. Anti-GM1, anti-GM2 and anti-GA1 antibodies in serum were positive. We diagnosed chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) presenting ataxia. Steroid therapy provided immediate improvement of symptoms and signs. This case suggests that CIDP should be considered as one of the potential causes of ataxic neuropathy.     

Acute relapsing sensory neuropathy following upper respiratory infections--a case report]

A 44-year-old man developed numbness in his hands and feet and an unsteady gait following an upper respiratory symptoms. The symptoms progressed rapidly and reached maximum in a few days, followed by a gradual improvement over a few weeks, but his gait remained unsteady. His symptoms progressively increased over 14 years, with 11 relapses of similar episodes. Neurologic examination showed normal limb strength. Deep tendon reflexes were absent. Touch, cold, and pinprick sensation were impaired in his hands and feet. There was a profound loss of position and vibration sense in all extremities. He demonstrated an unsteady and broad-based gait. Electrophysiologic and histologic examination on the peripheral nerves revealed evidences of axonal degeneration without demyelination involving the sensory nerves.     

A case of cervical myeloradiculopathy with positive serum anti-GT1a antibody]

A 19-year-old man with cervical myeloradiculopathy is reported. He was admitted to our hospital because of acute muscle weakness of upper limbs, which developed two weeks after respiratory tract infection. Neurologic examination revealed prominent muscular weakness of upper limbs. Deep tendon reflexes showed hyporeflexia in upper limbs and hyperreflexia in lower limbs. Serum IgG anti-GT1a antibody was detected by thin-layer chromatography and immunoblotting. Neck MRI revealed T2-weighted high intensity legions and swelling in spinal cord at third to sixth cervical segment. The muscular weakness and the cervical legion in MRI improved two weeks after steroid treatment. These findings indicate the involvement of cervical pyramidal tract as well as that of cervical roots in the patient. Neurological symptoms of the present case differed from those of pharyngeal-cervical-brachial variant (PCB) of Guillain-Barré syndrome, although serum anti-GT1a antibody was positive.     

Two brother cases of late-onset familial amyloidotic polyneuropathy in Kyoto]

Measurement of variant Met30 transthyretin is diagnostic for a patients with familial amyloidotic polyneuropathy (FAP) type I. The elder brother first noticed numbness of the feet at 64 years of age, and developed weakness of the legs. A few years later, he noticed numbness of the hands, and he was admitted to the hospital at 67 years of age. He was emaciated and had hoarseness and macroglossia. He had moderate muscle atrophy and weakness of all extremities with distal predominance. Deep tendon reflexes were hypoactive in the upper limbs and absent in the lower limbs. There was marked sensory loss of pain and temperature in all 4 limbs distally, and position sense was also impaired. He had mild orthostatic hypotension, severe cardiomegaly and arrhythmia. The younger brother noticed cold sensation of the feet and sexual impotence at 59 years of age. Two years later, he had numbness of the feet and developed weakness of the legs. At 65 years of age, he was admitted to the hospital because of the micturition syncope. He was emaciated and had macroglossia. He had moderate muscle atrophy and weakness of all extremities with distal predominance. Deep tendon reflexes were absent. There was marked sensory loss in the extremities which was predominant in pain and temperature. He had severe orthostatic hypotension (112/70 mmHg in supine position, 50/30 mmHg on standing). Plasma NE value was low and showed poor response to standing. He had neither cardiomegaly nor arrhythmia. Their parents were supposed to have no neurological symptom and were not related with any other Japanese foci of FAP.(ABSTRACT TRUNCATED AT 250 WORDS)     

Multifocal axonal motor neuropathy associated with anti-ganglioside antibodies]

We report a patient with asymmetrical patchy weakness of the limbs, and with autoantibodies against gangliosides GM1, GD1b, asialo GM1. Although electrophysiological studies did not reveal conduction block, treatment with prednisolone resulted in clinical improvement. A 52-year-old man was admitted to Kyoto University Hospital, because of gait disturbance. Neurological examination revealed a patchy distribution of weakness in the limbs. Deep tendon reflex was normal at the right knee, and was depressed at the right biceps. Other deep tendon reflexes were absent. There was a slight decrease in vibratory sensation in the distal portions of the lower extremities. Routine laboratory studies, heavy metal screen, vitamin, cryoglobulin, coproporphyrin and delta-amino levulinic acid in urine, and the protein value of the cerebrospinal fluid were normal. Head and neck MRI, and myelography were normal. Immunofixation electrophoresis showed IgM lambda M-protein in serum. Thin-layer chromatography with immunostaining showed his serum IgM reacted with GM1, GD1b, and asialo GM1. ELISA (Enzyme Linked Immunosorbent Assay) demonstrated high titers of anti GM1, GD1b and low titer of anti asialo GM1. Motor conduction studies showed no demonstrable conduction block, normal conduction velocities and the low amplitudes of CMAP. Sensory conduction studies showed no abnormalities except for slightly decreased amplitude of SNAP in sural nerve. Electromyography showed active denervation in extensor digitorum communis muscle, tibialis anterior muscle and left biceps brachii muscle. Muscle biopsy specimen revealed large and small group atrophy and there was perivascular mononuclear infiltration at one point.(ABSTRACT TRUNCATED AT 250 WORDS)     

Hyperexcitability restricted to the lower limb motor system in a patient with stiff-leg syndrome

We report a 29-year-old man who presented with a 2-year history of progressive stiffness and painful spasms limited to the bilateral lower limbs, exaggerated by auditory and tactile stimuli. His deep tendon reflexes were slightly increased in both lower extremities. His plantar response was flexor. His serum and cerebrospinal fluid were negative for anti-glutamic acid decarboxylase antibodies. Electromyography of antagonist muscle pairs in his distal lower limbs revealed a failure of reciprocal inhibition. We used transcranial magnetic stimulation with a paired-pulse paradigm, delivered to the cortical area of the upper and lower limbs, and revealed significantly enhanced facilitation only in the area of his lower limbs, but not that representing his upper limbs. His symptoms were improved substantially by 20mg/day of oral diazepam. To our knowledge this is the first report of a patient with hyperexcitability limited to the lower limb motor system in a patient with stiff-leg syndrome.     

Nitrous oxide anesthesia-associated myelopathy

BACKGROUND: The role of nitrous oxide exposure in neurologic complications of subclinical cobalamin deficiency has been reported, but few cases are well documented. OBSERVATION: Two weeks after surgery for prosthetic adenoma, a 69-year-old man developed ascending paresthesia of the limbs, severe ataxia of gait, tactile sensory loss on the 4 limbs and trunk, and absent tendon reflexes. After a second surgical intervention, the patient became confused. Four months after onset, the patient had paraplegia, severe weakness of the upper limbs, cutaneous anesthesia sparing the head, and confusion. Moderate macrocytosis, low serum B12 levels, and a positive Schilling test result led to the diagnosis of pernicious anemia. Results of electrophysiologic examinations showed a diffuse demyelinating neuropathy. Magnetic resonance imaging of the spinal cord disclosed hyperintensities of the dorsal columns on T2-weighted images. CONCLUSIONS: Pernicious anemia can result in severe neurologic symptoms with only mild hematologic changes. The role of nitrous oxide anesthesia in revealing subclinical B12 deficiency must be emphazised. Magnetic resonance imaging of the spinal cord might be helpful in making the diagnosis.     

A case of hereditary motor and sensory neuropathy of neuronal type with retardation of motor development

An atypical case of hereditary motor and sensory neuropathy of neuronal type with retardation of motor development was described. The patient was a 15-year-old boy who had suffered from distal muscle weakness with atrophy of four limbs and deformities of hands and feet since age 6 months. These symptoms were slowly progressive. He had never walked. His parents were not consanguinous. His parents and two siblings were unremarkable on neurological examination and on nerve conduction studies. On neurological examination, he showed severe degree of muscle weakness and atrophy in the distal upper and lower limbs, moderate degree of muscle weakness and atrophy in the proximal upper limbs and slight degree of made weakness and atrophy in the proximal upper limbs. Deep tendon reflexes in four limbs were decreased or absent. Vibration sensation was moderately decreased in the distal parts of four limbs. On the nerve conduction studies, no sensory nerve potential was recorded in the median, ulnar and sural nerves bilaterally. Motor nerve conduction velocity of the right tibial nerve was 21 m/sec and the amplitude of the compound muscle action potential (M-wave) was 0.15 mV, and no M-wave was elicited with the electrical stimulation of the median, ulnar and peroneal nerves. Neelde EMG showed fibrillation potentials and giant spikes with a reduction of the number of motor units. On sural nerve biopsy, the densities of both myelinated and unmyelinated fibers were severely decreased.(ABSTRACT TRUNCATED AT 250 WORDS)     

Ipsilateral hemiparesis after putaminal hemorrhage due to uncrossed pyramidal tract

OBJECTIVE: Previous case reports supported the presence of the uncrossed pyramidal tract in exceptional patients. However, most of these case reports have not fully discussed involvement of the motor cortex controlling the ipsilateral limbs. DESIGN AND METHOD: The authors investigated a 62-year-old man who developed right hemiparesis after right putaminal hemorrhage by using MRI, transcranial magnetic stimulation, functional MRI (fMRI), and sensory evoked potentials. He had moderate weakness including the face, spasticity with brisk deep tendon reflexes and Babinski sign, and impaired vibration and position sense, all on the right side. RESULT: A MRI study showed hemorrhage in the right putamen and the wedge-shaped medulla. A fMRI study during a sequential finger opposition task showed activation in the motor cortex ipsilateral to the finger movements, but not on the contralateral side. Sensory evoked potentials showed cortical response ipsilateral to the side of stimulation. CONCLUSION: The pyramidal tract and the dorsal column-medial lemniscus pathway did not cross in the medulla in this patient. In view of the presence of the abnormal shape in the medulla and congenital scoliosis, a congenital factor might be responsible for the uncrossed pyramidal tract and dorsal column-medial lemniscus in this patient.     

A case of motor neuron disease with presenile dementia showing bilateral degeneration of the pyramidal tract on cranial MRI]

A 58-year-old man developed dysarthria followed by a personality change. Subsequently, he developed muscle weakness and atrophy of the left upper and lower limbs, leading to repeated falls when he tried to walk. Neurological examination showed mild dementia, dysarthria, dysphagia, atrophy and fasciculation of the tongue, and muscle weakness and atrophy of all four extremities, particularly on the left side. Deep tendon reflexes were slightly diminished in the upper limbs and slightly exaggerated in the lower limbs without Babinski's sign. Cranial MRI revealed marked atrophy of the medial portions of the temporal lobes, more striking on the right, and T2-weighted imaging revealed symmetrical high-intensity signals from the posterior limbs of the internal capsules to the cerebral peduncles in the midbrain, extending to the pons on the left. 125I-IMP SPECT showed diffuse reduction of RI uptake in the frontal and temporal lobes, which was more marked on the right. We diagnosed this is a case of motor neuron disease with presenile dementia, which Mitsuyama et al. proposed as a new clinical entity, as well as a rare example of bilateral degeneration of the pyramidal tract on cranial MRI.     

A case of Hopkins syndrome with onset at puberty]

The patient was a 15-year-old man who developed weakness of left leg 6 days after an acute asthmatic attack. Neurological examination revealed severe muscle weakness and atrophy at L5-S1 level and mild muscle weakness and atrophy at L2-4 level in the left leg. Deep tendon reflexes were normal in the upper limbs and slightly brisk in the lower limbs except for absence of Achilles tendon reflex on the left. His sensation was normal. Needle EMG revealed neurogenic changes in both the left (L2-S1) and right (L2-4) leg muscles. Motor nerve conduction study of the left tibial and peroneal nerves revealed a marked reduction of amplitude and mild reduction of MCV. F-wave was not evoked in either nerves. Sensory nerve conduction study of the left sural nerve was normal. The titers of anti-viral antibodies in the paired sera showed no significant changes in any viruses examined including echovirus, enterovirus, coxsackievirus and poliovirus type 1, 2 and 3. The serum IgE was elevated (1,300 IU/ml) and mite antigen-specific IgE was strongly positive. Spinal cord MRI revealed no abnormality in either thoracic or lumbar spinal cord. This patient was diagnosed as a rare case of Hopkins syndrome with onset at puberty.