Teruyuki Kurihara

Polymyositis and dermatomyositis associated with malignancy: a 30‐year retrospective study

Background Polymyositis and dermatomyositis in association with malignancy are paraneoplastic syndromes, but the incidence, treatment and factors that predict associated cancer and its prognosis all remain unclear.

An autopsy case of mitochondrial encephalomyopathy: biochemical and electron microscopic studies of the brain

A 29-year-old single woman had recurrent stroke-like episodes. She developed loss of consciousness, myoclonic seizures, and lactic acidosis. She died at the age of 30. A muscle biopsy study revealed mitochondrial myopathy, and the postmortem biochemical analysis demonstrated decreased cytochrome c oxidase activity in the skeletal muscles by 20% of normal control. The brain had multiple ischemic lesions in the cerebral cortex without major vascular occlusions. We present this case as an autopsy case of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) with a partial deficiency of cytochrome c oxidase. The analytical electron microscopic study of the calcified small vessels in the globus pallidus revealed increased calcium, phosphorus and iron. No accumulation of chromium, nickel or zinc was noted in this case, which was different from the previously reported cases of basal ganglia calcification.

Trophic effect of angiotensin II, vasopressin and other peptides on the cultured ventral spinal cord of rat embryo*

We studied trophic effects of angiotensin II, vasopressin, cholecystokinin, and oxytocin on explanted ventral spinal cord cultures from 13- and 14-day-old rat embryos. There was a significant neurite promoting effect of the spinal cord cultures by using angiotensin II, vasopressin, and cholecystokinin. Cholecystokinin had the most potent effect at any concentrations. The minimum effective concentration was 10(-8) M in angiotensin II and vasopressin and 10(-12) M in cholecystokinin, respectively. The effect of angiotensin II and vasopressin was dependent on concentrations. However, the rate and grade of neurite appearance did not correlate with the concentrations of cholecystokinin. Oxytocin had no neurotrophic effect at any concentrations. Our results demonstrated that angiotensin II, vasopressin and cholecystokinin have neurotrophic effects on the ventral spinal cord in cultures, and may be candidates for therapeutic trials of amyotrophic lateral sclerosis.

Ischemic and hemorrhagic brain stem lesions mimicking diabetic ophthalmoplegia

Two patients with diabetes mellitus, one of them with an isolated third cranial nerve palsy and the other with an isolated sixth cranial nerve palsy, are presented. MRI investigations including diffusion-weighted MRI revealed a small ischemic brain stem lesion in the former and a small hemorrhagic brain stem lesion in the latter. In the former case wallerian degeneration of the nerve fascicle within the mesencephalon was also detected. These cases indicate that vascular accidents of the brain stem may masquerade as fascicular or infranuclear disturbance of the oculomotor or abducens nerve; therefore, it is important to include brain stem lesions into the differential diagnosis of isolated ophthalmoplegia. Thorough investigation by MRI including diffusion-weighted MRI is helpful for correct diagnosis.

Histological and Immunohistological Changes of the Skeletal Muscles in Older SJL/J Mice

SJL/J mice have been studied as the model animals for autoimmunological diseases. Recently it was clarified that SJL/J mice have a defect of dysferlin. Human limb girdle muscular dystrophy 2B and Miyoshi myopathy also have a defect of dysferlin. In this study we present the histological and immunohistological changes in the natural course. Histological study revealed that SJL/J mice had inflammatory, degenerative changes, and neurogenic changes in later ages. As for interstitial inflammatory cells, the macrophages were dominant in any age, and in the T cell subset, the CD4+ T cells were more abundant than the CD8+ T cells, and few B cells were seen. The laboratory data showed a high level of creatine kinase in all ages. It is suspected that the inflammatory changes were induced by the primary immunological abnormality or by the defect of dysferlin in SJL/J mice.

Ulcerative colitis presenting as sensorineural deafness, brainstem encephalopathy, and white matter lesions.

Background:Several rare neurologic complications of ulcerative colitis have been reported. Review Summary:We report a 69-year-old Japanese woman who developed bilateral sensorineural deafness, 2 attacks of bilateral ophthalmoplegia, and bilateral facial nerve palsy in association with ulcerative colitis. Laboratory data showed elevated cerebrospinal fluid (CSF) protein without pleocytosis, abnormal auditory brainstem evoked potentials, and multiple high signal lesions on magnetic resonance imaging of the brainstem and cerebral deep white matter. Her symptoms improved with corticosteroid therapy except for sensorineural deafness and an exacerbation of cerebral deep white matter lesions without any new clinical signs. Conclusion:Immunologic mechanisms may have led to her central and peripheral nervous system findings in addition to her colon disorder.

Clinical significance of hypointensity in the motor cortex on T2-weighted images

1. Thornton CA, Ballow M. Safety of intravenous immunoglobulin. Arch Neurol 1993;50:135-136. 2. Vera-Ramirez M, Charlet M, Parry GJ. Recurrent aseptic meningitis complicating intravenous immunoglobulin therapy for chronic inflammatory demyel ina t ing polyradiculo-neuropathy. Neurology 1992;42: 1636-1637. 3. Watson JDG, Gibson J, Joshua DE, Kronenberg H. Aseptic meningitis associated with high dose intravenous immunoglobulin therapy. J Neurol Neurosurg Psychiatry 1991;54:275-276. 4. Constantinescu CS, Chang AP, McCluskey LF. Recurrent migraine a n d in t ravenous immune globulin therapy . N Engl J Med 1993;329:583-584. 5. Silbert PL, Knezevic WV, Bridge DT. Cerebral infarction complicating intravenous immunoglobulin therapy for polyneuritis cranialis. Neurology 1992;42:257-258. 6. Ropper AH, Adelman L. Early Guillain-Barre syndrome without inflammation. Arch Neurol 1992;49:979-981. 7. Dalakas MC. High-dose intravenous immunoglobulin and serum viscosity: risk of precipi ta t ing thromboembolic events. Neurology 1994;44:223-226.

Variant transthyretin in cerebrospinal fluid in familial amyloidotic polyneuropathy

Structurally abnormal transthyretin is a precursor protein of amyloid fibrils in type I familial amyloidotic polyneuropathy (FAP). This variant transthyretin has an amino acid substitution of methionine for valine at position 30. The purpose of this study was to clarify whether this variant transthyretin also circulates in the cerebrospinal fluid (CSF) of patients with type I FAP. CSF transthyretin of the patients was purified and its primary structure determined. Sequence determination indicated that transthyretin consisted of a mixture of normal and variant transthyretin. Variant transthyretin was present in the CSF of all 5 Japanese FAP patients studied. The CSF concentration of variant transthyretin was high (0.72 +/- 0.15 mg/dl, mean +/- S.D.), suggesting that variant transthyretin is synthesized in the choroid plexus. Variant transthyretin was not present in any of 20 controls. The CSF concentration of total transthyretin in FAP patients was 1.74 +/- 0.42 mg/dl, which was not significantly different from controls.

Neuropsychological function before and after plasma exchange in myasthenia gravis

This study was designed to determine whether there are cognitive and memory impairments in patients with myasthenia gravis (MG). Five female patients with MG were tested on two occasions, 4-6 days apart, prior to plasma exchange and after plasma exchange. All subjects had 2-4 separate plasma exchanges. The subjects were tested with mini-mental state examination (MMS), Zung self-rating depression scale (SDS) and a logical memory test. The MG group was significantly impaired compared to the control group on all tests prior to plasma exchange. The MG patients improved in motor function with plasma exchange. In addition, the mean MMS score improved from 26.4 to 28.2 and the mean SDS score improved from 0.46 to 0.39 and the mean immediate logical memory score improved from 5.8 to 8.4 and mean delayed logical memory score improved from 5.8 to 8.0. These improvements were all significant (P < 0.05). The results support the hypothesis that MG has central cholinergic effects, manifested by cognitive dysfunction.

A case of Guillain-Barré syndrome associated with cerebellar ataxia and positive serum anti-GD1b IgG antibody

Sirs: It is well known that the antibody against ganglioside is detectable in the Guillain-Barré syndrome (GBS) [2, 5, 9]. AntiGD1b IgG antibody has been found in a case of ataxia with disturbance of deep sensation [7]. We encountered a case of GBS associated with cerebellar type ataxia and positive serum anti-GD1b IgG antibody. A 21-year-old woman presented on 9 May 1999, suffering from diarrhoea. On 20 May, she felt weakness in the lower extremities and later developed paraesthesia in the distal part of both extremities and diplopia. On 26 May, she had difficulty in walking. On 3 June, she was admitted to our hospital. On admission, neurological examination revealed normal consciousness. In the cranial nerves, a left abducens paralysis was found. The muscle strength was 3/5 in all extremities. Grip strength was 2 kg on the right side and 3 kg on the left side. All deep tendon reflexes were diminished and pathological reflexes were not observed. Objective sensations were all normal. Rectal sphincter and bladder dysfunction and cerebellar signs were not observed. Laboratory studies disclosed that Campylobacter jejuni was not detected in her faeces. Serum antiGD1b IgG antibody titre was 1:200 (normal < 100). Anti-GM1, GM2, GD1a,GD1b, GT1b and GQ1b antibody titres were all within normal ranges. Cerebero-spinal fluid examination revealed that the cell count was 2/μl, total protein was 217 mg/dl, and sugar was 54 mg/dl (blood sugar 88 mg/dl). Electrophysiological examination revealed that the motor nerve conduction velocity was 37.1 m/s (normal > 43.0) in the right peroneal nerve and 36.6 m/s (normal > 41.0) in the right tibial nerve. Sensory nerve conduction velocity was not detected in the right peroneal nerve and was 39.9 m/s (normal > 46.0) in the right tibial nerve. After admission, she was immediately treated with immuno-absorption therapy (tryptophan calumn; TR-250). After beginning the 1st immuno-absorption therapy, cerebellar speech appeared. Subsequently, disturbance of finger-nose and heel-knee tests, intention tremor, dysmetria and dysdiadochokinesis were observed; however, Romberg’s sign and disturbance of deep sensation were not detected. After 4 courses of immuno-absorption therapy, muscle weakness, paraesthesia and disturbance of eye movement gradually improved, but did not completely disappear. The delayed cerebellar signs also improved by the end of June, and she discharged herself. At this point, serum anti-GD1b IgG antibody titre was still elevated at 1:200. However, following discharge, exacerbation occurred after a few days, including muscle weakness mostly in the upper extremities and paraesthesia. After re-admission another 4 courses of immuno-absorption therapy were administered. At the end of August, her symptoms all subsided, with normal serum anti-GD1b IgG antibody titre (< 1:100). Head MRI was performed twice during her admission, but no abnormal signs were detected in the cerebrum, cerebellum or brain stem. The clinical course is shown in Figure. In the present case, muscle weakness, paraesthesia and diminished deep tendon reflexes occurred, followed by diarrhoea, and LETTER TO THE EDITORS