[A case presenting with Raeder's syndrome-like symptoms due to vertebral artery aneurysm].

A 50-year-old man complained of headache around his left orbit, left frontal pain and paresthesia associated with left incomplete Horner syndrome. MRI demonstrated a mass at the level of medulla oblongata. Left vertebral angiogram revealed an aneurysm of left vertebral artery. Following the removal of the aneurysm, these Raeder's syndrome-like symptoms improved. Therefore, they were probably caused by a compression of the spinal tract of the trigeminal nerve and the central sympathetic tract by the aneurysm. This is the first report of Reader's syndrome-like symptoms caused by vertebral artery aneurysm, thus indicating that MRI and cerebral angiogram are necessary for differential diagnosis of this syndrome.

[Magnetic resonance imaging of a case of central nervous system tuberculosis with tuberculous arachnoiditis and multiple tuberculomas].

A 62-year-old woman developed headache, vomiting and fever. On admission to hospital, she showed an imparied level of consciousness, diplopia on left lateral gaze, bilateral hearing loss and left hemiparesis. Cranial contrast computed tomography (CT) revealed basal meningeal enhancement. Lumbar cerebrospinal fluid (CSF) showed an increase in cell count (80/mm3) and total protein (3000 mg/dl), and a decrease in glucose (65 mg/dl) in comparison with blood sugar (173 mg/dl). Polymerase chain reaction was positive for Mycobacterium tuberculosis in the CSF. She was diagnosed as having tuberculous meningitis and was treated with anti-tuberculous chemotherapy. Her level of consciousness recovered and other clinical signs improved gradually the first month after admission. However, in spite of the combination of anti-tuberculous chemotherapy and steroid therapy, her combination of anti-tuberculous chemotherapy and steroid therapy, her consciousness level worsened again in association with paraplegia at the sixth week after admission and magnetic resonance imaging (MRI) revealed multiple tuberculomas, spinal arachnoiditis and spinal cord infarction. On T2-weighted imaging some of the tuberculomas showed a central hyperintense area (a central bright core) with an isointense periphery, which was surrounded by a hyperintense area. The lesion appeared hypointense with an isointense rim on T1-weighted imaging, showing a ring enhancement on post-contrast T1W imaging. The spinal cord infarction was situated at the third thoracic cord, which corresponded to the borderline of spinal artery perfusion. This is a rare case of progression of spinal arachnoiditis and spinal cord infarction during anti-tuberculous chemotherapy, and who had tuberculoma with a central bright core on MRI.

[A case of rapidly progressive T cell type malignant lymphoma which started with multiple cranial neuropathy].

A 35-year-old man had suffered from recurrent right trigeminal nerve palsy and flaccid paraparesis for about five months. Cerebrospinal fluid (CSF) showed a marked increase of protein (400 mg/dl) and mononuclear cells (146/mm3), but there were no malignant cells. Antibiotic therapy remitted his inguinal and mediastinal lymph nodes swelling, and trigeminal nerve palsy had recovered spontaneously. Then he developed left trigeminal and facial nerve palsy, mononeuropathy multiplex, and cauda equina syndrome. Nerve conduction studies revealed delayed velocity and reduction of amplitude. Enhanced magnetic resonance imaging showed increased signal intensity in bilateral trigeminal nerves, left internal auditory meatus, and meninges of the basal cistern. Also, there were two mass lesions in cauda equina. They were operated by orthopedist, and were not malignant. After that, CSF cells of malignant lymphoma were elevated and revealed T cell type (large cell). Then the patient exacerbated in bulbar palsy and died. When there is lymph node swelling with multiple neurological deficits, despite remission of lesions and signs, biopsies should be positively pursued early in the patient's clinical course.

[A case of psittacosis with psychiatric symptoms, abnormal EEG, and abnormal SPECT].

A 40-year-old woman was admitted to our hospital with disorientation, delusional idea, and sensory disturbance of lower face after having a fever and cough for 5 days. A chest X-ray examination revealed an infiltration in the left lower lobe field. Psittacosis was diagnosed because the serum antibody titer against chlamydia psittaci was elevated. Electroencephalogram showed right temporal lobe spikes. SPECT showed decreased accumulation of 99mTc-HMPAO in the right frontal and temporal lobe. Computed tomography scan of the brain was normal. This patient was diagnosed as ornithsis Encephalitis.

[Polymyositis followed by myasthenia gravis].

A 63-year-old woman was admitted to the hospital because of swelling of both forearms and muscle weakness in the limbs. Laboratory examination revealed abnormally high levels of serum creatine kinase, glutamic-oxaloacetic transaminase, glutamic-pyruvic transaminase, and lactate dehydrogenase. Polymyositis was diagnosed, and prednisolone was administered. Her condition had been well controlled on prednisolone until several months before admission, when bilateral blepharoptosis and diplopia developed. An edrophonium test was positive: muscle weakness was transiently alleviated. High titers of anti-acetylcholine receptor antibodies were found. A chest CT scan showed a mass in the anterior part of the mediastinum. We diagnosed myasthenia gravis associated with thymoma. After an extended thymectomy, the patient's condition improved. When muscle weakness recurs after remission of polymyositis, myasthenia gravis should be considered.

[A case of myotonic dystrophy with morning headache following sleep apnea syndrome].

We reported a 39-year-old man with myotonic dystrophy. He suffered from morning headache. Respiratory function tests showed restrictive pattern and arterial gas analysis showed hypoxia and hypercapnia with respiratory acidosis (PaCO2 50.8 mmHg, PaO2 63.8mmHg, pH 7.317, SaO2 89.8%). Polysomnograph showed central apneas exclusively in light sleep (stage 1 and 2). O2 saturation fell at most to as low as 50% during the apneas. We conclude that sleep apnea should be consider in patients with myotonic dystrophy associated with morning headache.

[A case of secondary carnitine deficiency due to anorexia nervosa and severe liver damage].

We reported an 18-year-old woman with anorexia nervosa and liver damage who showed carnitine deficiency. Her unbalanced diet had little carnitine, and severely damaged liver could not synthesize carnitine. Since total parenteral nutrition (TPN) and tube-feed formulas contain little or no carnitine, the patient had progressive weakness and muscle atrophy. Muscle biopsy revealed variability of fiber diameter, type 1 fiber atrophy and copious amount of lipid granules. Carnitine deficiency must be diagnosed in the early stage by muscle biopsy or muscle carnitine levels, and treated with oral intake of L-carnitine.

[Clinical analysis of benign transient shuddering-like involuntary movement in elderly people].

We described 9 patients who developed acute onset benign transient shuddering-like involuntary movement in elderly people. There was no consciousness disturbance or sensorimotor dysfunction. There were finger tremor, dysarthria, or gait disturbance in some patients. Asterixis was observed in the upper extremities in 2 patients. Duration of an involuntary movement was less than 1 second, and the series of the involuntary movements continued for several days. This state appeared acutely and disappeared within 0.5 -4 days in the natural course without any sequelae. Oral administration of clonazepam was very effective to suppress this tremulousness. Seven of 9 patients developed recurrence. Although the cause of this syndrome remains unknown, we believe that this condition is clinically important for differential diagnosis of acute onset involuntary movements in elderly people.

[Multiple system atrophy with macro square wave jerks and pendular nystagmus].

A 51-year-old woman was admitted to our hospital because of gait disturbance and dysuria. Neurological examination revealed limb and truncal ataxia, orthostatic hypotension, cogwheel rigidity in all limbs, generalized hyperreflexia without pathological reflex, and horizontal gaze nystagmus. She became progressively worse and bedridden at age 52. Then she developed abnormal eye movements. Electrooculogram revealed vertical, horizontal or oblique macro square wave jerks and pendular nystagmus. Macro square wave jerks appeared during fixation and disappeared with eye closure or in the dark room. Macro square wave jerks were characterized by a duration of about 200 msec and an amplitude of 10 to 15 degrees. Pendular nystagmus with a duration of several seconds and amplitude of 5 to 15 degrees appeared when she changed her fixation or the point of fixation disappeared. Macro square wave jerks and pendular nystagmus were mildly suppressed after the intramuscular injection of 100 mg of phenobarbital, the oral intake of sodium valproate of 600 mg/day or baclofen of 60 mg/day. They were almost completely depressed after the intravenous injection of 3 mg of diazepam or the oral intake of clonazepam of 1.5 mg/day. We suggested that both macro square wave jerks and pendular nystagmus in this patient might be caused by the dysfunction of GABAergic system in the saccadic eye movement system.

[Early-onset benign autosomal dominant limb-girdle myopathy with contractures (Bethlem myopathy) in a Japanese family].

We report a family showing an early-onset benign autosomal dominant limb-girdle myopathy with contractures. The clinical features in our family included; 1) slowly progressive limb-girdle muscle weakness since childhood. 2) contractures of fingers, elbows and ankles, 3) mild motor impairment with preserved activities of daily living. The disease was inherited through an autosomal dominant trait. Muscle pathology revealed variation in fiber size, slightly increased central nuclei, mild endomysial fibrosis, type 1 fiber predominance and type 2 fiber atrophy. The above clinical features were similar to those seen in patients reported by Bethlem (1976) and Mohire (1988), and therefore we made a diagnosis of Bethlem myopathy on this familial patients which was extremely rare in Japan.

[A case of unilateral lingual atrophy and ipsilateral muscular atrophy supplied by trigeminal nerve--in relation to progressive facial hemiatrophy].

A 39-year-old man was admitted to our hospital because of atrophy of the right side of the tongue of 6 years' duration. Neurological examination showed atrophy and fasciculations in the right side of the tongue. Magnetic resonance imaging showed atrophy and high signal intensity areas on T1 and T2 weighted images in the right masseter, temporal, lateral and medial pterygoid muscles. Electromyography showed fibrillations and positive sharp waves at rest, high amplitude polyphasic motor unit potentials with weak contraction in the right tongue and masseter muscles. Examination of autonomic functions suggested postganglionic lesion of right facial sympathetic nerves. We believe that this case may be related to progressive facial hemiatrophy.

[A case of progressive myoclonus epilepsy with the first and second branchial syndrome].

A 22-year-old woman with progressive myoclonus epilepsy associated with the first and second branchial syndrome is described. Clinical features included generalized convulsive seizure, myoclonus, cerebellar ataxia and intellectual deterioration with micrognathia and malformation of auricles. She was initially suspected of mitochondrial encephalomyopathy, but the analysis of muscle biopsy and mitochondrial enzyme activities was negative. Her micrognathia and malformation of auricles were diagnosed as the first and second branchial syndrome. The case of progressive myoclonus epilepsy associated with this syndrome has never been reported, and the relationship between them remains unknown.

[Analysis of hemiparesis with homolateral ataxia by single photon emission CT].

Case 1. A 46-year-old man suddenly developed mild gait disturbance and left hemiparesis. On examination, gross strength was slightly reduced in the left extremities. The finger-to-nose and heel-to-knee tests disclosed moderate dyssynergia and dysmetria on the left side that could not be explained by the muscular weakness. Deep tendon reflexes were more brisk in the left extremities. There was no Babinski sign. Magnetic resonance imaging showed a region of high signal intensity in the right posterior limb of internal capsule with extension into lateral thalamus. The lesion involved the cortico-ponto-cerebellar pathway and partly the dentato-rubro-thalamo-cortical pathway. No lesions were seen in the brainstem. Single photon emission CT with 123I-IMP showed left cerebellar hypoperfusion termed crossed cerebellar diaschisis by Baron et al. Case 2. A 65-year-old female developed weakness of the left extremities and gait disturbance. On examination, there was a horizontal nystagmus on lateral gaze to each side. She showed dysarthria, mild left hemiparesis and slight left hypesthesia. The finger-nose and heel-knees tests revealed moderate dysmetria and dyssynergia on the left side. Deep tendon reflexes were hyperactive in the left extremities with left Babinski sign. CT showed a low density area in the right basis pontis at about middle level. Intravenous digital subtraction angiography revealed a slight stenosis of right vertebral artery, but no other abnormality. The lesion involved the cortico-ponto-cerebellar pathway. Single photon emission CT with 123I-IMP showed left cerebellar hypoperfusion. The right cerebellar blood flow was normal.(ABSTRACT TRUNCATED AT 250 WORDS)