[Isolated angiitis of the central nervous system presenting as subcortical hemorrhage--a case report of benign type].

A 47-year-old woman had an episode of severe headache for a few days. She suddenly experienced right leg weakness and sensory loss. A CT scan revealed subcortical hematoma at the left parietal lobe on admission. Cerebral angiography showed multiple vascular irregularities such as segmentally narrow or sausage-like dialatated areas. Her laboratory studies were entirely normal including antinuclear antibody and coagulation tests. The diagnosis of isolated angiitis of the CNS was made and she responded well to the low dose corticosteroid therapy. Repeated cerebral angiography two months after the onset demonstrated most of the areas of segmental irregularity had improved with a few unchanged areas. Some cerebral angiitis do exist that respond well to corticosteroid therapy, therefore, early diagnosis and treatment are suggested in cases of angiitis.

[A patient of late-onset nemaline myopathy with mononuclear cell infiltration].

We described a 50-year-old woman with late-onset nemaline myopathy with focal mononuclear cell infiltrates in her muscle biopsy. She developed difficulty in climbing stairs, and elevating her arms for 5 months after the onset of the disease. On admission, neurological examination revealed moderate weakness and atrophy in the proximal limb and neck muscles. Laboratory studies were within normal limits except mildly elevated serum CK and aldolase levels. Electromyography showed myopathic changes in the right triceps and quadriceps muscles examined. A biopsy from the left biceps brachii muscle revealed increased variation in fiber size, with numerous basophilic atrophic fibers. There were some foci of mild mononuclear cell infiltration. Most of basophilic fibers contained nemaline bodies on modified trichrome stain. On electron microscopy numerous nemaline bodies were present in fibers with marked myofibrillar degeneration. Azathioprine and prednisolone administration was not effective to improve her condition. As mononuclear cell infiltration has been occasionally described in the previously reported patients of adult-onset nemaline myopathy, inflammatory process may have some roles in formation of nemaline bodies on the way of acute myofibrillar degeneration.

[Chronic inflammatory demyelinating polyneuropathy in childhood].

Clinical, electrophysiological and histopathological findings in 6 children with steroid-responsive acquired demyelinating neuropathy are presented. The clinical features and nerve conduction findings are basically similar to those of chronic inflammatory demyelinating neuropathy (CIDP) in adults, although early-onset cases had prominent pes cavus deformity and thickened nerves, which are rare findings in acquired neuropathies in adults. The diagnostic criteria of adult CIDP can be adopted for most of the cases, however, repeated electrophysiological tests may be required to identify multifocality of the nerve lesion, especially when conduction block is not apparent before treatment. The biopsied sural nerves showed many thinly-myelinated fibers, subperineurial and endoneurial edema, and cellular infiltrations. Varied fascicular involvements were common. Two cases with almost complete or considerable loss of myelinated fibers in the biopsied sural nerve revealed good clinical response to steroid therapy. The degree of nerve degeneration in the sural nerve thus, may not be helpful to estimate the prognosis and the responsiveness to treatment. Therapeutic trials should be employed when the main conduction findings are those of demyelinating neuropathies, even if genetically-determined neuropathy is suggested from the clinical pictures.

Spontaneous remission of acromegaly after pituitary apoplexy following head trauma.

A 34-year-old man with acromegaly was observed, and the clinical course and various endocrinological deficiencies occurring before, during and after the development of pituitary apoplexy were evaluated. Following the attack, the patient experienced acute reductions in growth hormone (GH) and other pituitary hormones, adrenohypophyseal insufficiency and transient diabetes insipidus which appeared subsequent to glucocorticoid therapy. The level of growth hormone in serum and cerebrospinal fluid (CSF) was measured synchronously, and the respective regressions of GH levels in serum and CSF were observed. The results demonstrated the utility of measuring hormonal levels in CSF in the clinical evaluation of pituitary apoplexy in a patient with a functioning pituitary tumor.

In vivo effect of methylcobalamin on the peripheral nerve structure in streptozotocin diabetic rats.

To study in vivo effect of methylcobalamin (CH3-B12) on the peripheral nerve structures, rats with experimental diabetes induced by streptozotocin were administered with daily intramuscular injection of CH3-B12 (500 microgram/kg) for 16 weeks. By isolated nerve fiber studies, CH3-B12-treated diabetic rats showed less incidence of paranodal demyelination as an early sign of segmental demyelination than non-treated diabetic rats. From morphometrical analysis on sural nerves, the reduction in the density of myelinated nerve fibers, nerve fiber size and axon size of myelinated fibers was definitely protected in treated diabetic rats. The results suggested that continuous treatment with CH3-B12 had an ameliorative effect on the peripheral nerve lesions in experimental diabetic neuropathy.