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In 1975, Myklebust proposed a special form of learning disabilities characterizes by impairment of spatial cognition, social interaction and conceptualization, and named it as non-verbal learning disabilities (NLD). Since the brain locus for spatial cognition was thought to be localized in the right hemisphere, NLD was regarded as a disorder of the right hemispheric function. The clinical characteristics of NLD are almost identical to those of Asperger syndrome (AS), which was introduced in English literature in 1981. Since Asperger syndrome is regarded as one of the autistic spectrum disorders, and autism was once regarded as the left hemispheric dysfunction, it has long been believed that NLD and AS are clinically different entities. However, their clinical similarities have raised a growing opinion that these two disorders are probably identical or at least closely related.

We here reported a 24-year-old woman who presented complex partial seizure from 15 years of age. Her brain MRI showed the right perirolandic focal cortical dysplasia (FCD) corresponding to the orofacial division of the primary sensori-motor area. On SPECT (Tc-99 mECD) study, hypoperfusion was demonstrated at the FCD, the right pons and the left cerebellum (crossed cerebellar diaschisis (CCD)). Magnetoencephalography disclosed the current source of the focal spikes was localized on the FCD. To our knowledge, no previous report of a case associated with FCD and CCD was found. We speculated the pathomechanism of the CCD might be related to the persistent epileptogenic firing which inhibited the cerebellar metabolism transsynaptically through cortico (FCD)-ponto-cerebellar pathway.

Development of medical knowledge and technology has greatly improved our understanding of mental retardation. However, clinically applicable treatment of mental retardation is still limited. As described in the definition of mental retardation by the American Association of Mental Retardation, functional maladaptations in various aspects of everyday life are the most integral part of disabilities in mental retardation. The role of pediatric neurologist in the medical care for children with mental retardation is not limited in medical treatment. Pediatric neurologists should play an important role as a coordinators in integrated care. Providing of medical care in special schools for children with mental retardation is among the most important issues, since children spend most of their time either at home or in school. The current situation of "medical care" in school, and its problems are discussed.

Acute necrotizing encephalopathy (ANE) of childhood is a newly proposed disease entity characterized by symmetrically distributed necrotic brain lesions in the thalamus, cerebral white matter, brainstem, and cerebellum. We report a 4-year-old girl with severe psychomotor delay and horizontal gaze palsy as sequelae of ANE at 17 months of age. The computed tomography showed bilaterally symmetrical low density areas in the thalamus and low density areas in the left middle cerebral artery (MCA) region and the posterior cerebral artery (PCA) region. MRI (T 1-weighted) revealed unevenly distributed small low signal intensity areas with scattered high intensity regions in the thalamus bilaterally. The T2-weighted images showed multiple small low intensity areas around high intensity areas, and low signal intensity areas in the left middle cerebral artery (MCA) region and the posterior cerebral artery (PCA) region. In addition to severe psychomotor delay, the patient exhibited a peculiar eye movements. Horizontal ocular movement was impaired, but vertical ocular movement was almost completely normal. As clearly shown by MRI of the brain, the pontine tegmentum, including bilateral abducens nucleus, paramedian pontine reticular formation (PPRF), medial longitudinal fasciculus (MLF), and the facial nerve were affected, but the thalamo-mesencephalic junction, including the rostral interstitial medial longitudinal fasciculus (riMLF) and the nucleus of Cajal, was spared. To our knowledge, this is the first case of ANE associated with this selective ocular movement disorder ever reported. Because of the multiple symmetrical lesions and pons and the asymmetrical lesions of the MCA and PCA regions in the present case, occlusion of a single vessel could not account for the pathology. The pathophysiological mechanism of ANE is unknown. We postulate that some toxic or vasoactive agent caused vasospasm and subsequent breakdown of the blood-brain barrier, especially in the thalamus and pons, resulting in the unique distribution of the lesions and the rare eye movement disorder observed in the present case.

Between April 1990 and March 1991, postoperative adjuvant chemotherapy for resected gastric cancer employing 5-fluorouracil, epirubicin and mitomycin C (FEM) was performed. Forty-two patients subjected to the therapy were considered to have positive serosal invasion and underwent curative operation. FEM therapy consisted of intraoperative intraperitoneal administration of mitomycin C (0.3-0.4 mg/kg) combined with 8 cycles of intravenous bolus injection of epirubicin (20 mg/body) every 2-3 weeks which was started 2 weeks after the operation. Daily oral administration of 5-fluorouracil (150-200 mg/body) was started 2 weeks after the operation and continued for more than 6 months. Thirty-four of the 42 cases were assessable. Major adverse effects were nausea, vomiting, and general fatigue. There were no cardiovascular symptoms. The cumulative two-year survival rate was 74.2%, and follow-up was still under way at this writing.

Among many metabolic disorders, porphyrias and Fabry disease are known to affect autonomic nervous system. In patients with acute intermittent porphyria, hereditary coproporphyria, and variegate porphyria, autonomic symptoms such as abdominal pain, vomiting, hypertension and tachycardia are among the most prominent clinical manifestations. Fabry disease is clinically characterized by severe limb pain, hypohidrosis, angiokeratomas and various autonomic symptoms. In both porphyrias and Fabry disease, pathological changes in the central and peripheral autonomic nervous system have been documented. In porphyrias, a loss of myelinated fibers, axonal degeneration, and segmental demyelination in peripheral autonomic nerves as well as chromatolysis of several brain stem nuclei have been found. In Fabry disease, abnormal amount of the substrates of alpha-galactosidase, i.e. ceramide di- and trihexoside, are found to be accumulated in the central and peripheral autonomic nerves.

The case was a 66-year-old man. Three years ago under a diagnosis of Arteriosclerosis obliterans he received: right aorto-femoral bypass grafting. This time left intermittent clandication was observed and we performed resection of aneurysm and femorofemoral cross over bypass grafting following a diagnosis of right aorto-femoral anastomotic aneurysm accompanied by obstruction of left iliac and femoral artery. Anastomotic aneurysm is one of the complications after prosthetic bypass grafting. In this paper we discussed the causes, precautions and treatment of anastomotic aneurysms.