14 patients with the clinical diagnosis of early onset cerebellar ataxia with retained tendon reflexes (EOCA) were evaluated by multimodal evoked potential (EP) studies. The results were compared with observations made on 10 patients with Friedreich's ataxia (FA) and 16 patients with olivopontocerebellar atrophy (OPCA). Ten patients (71.4) with EOCA had abnormality of at least one of the EP parameters. Brainstem auditory evoked response (BAER) studies were abnormal in 50, followed by abnormalities in posterior tibial somatosensory evoked potential (SSEP) (46.1), visual evoked potential (VEP) and median SSEP (30.8 each). Only the abnormality of posterior tibial SSEP had a positive correlation with the duration of disease. The abnormalities in SSEP were more frequent in EOCA than in OPCA and were comparable to that of FA patients. On the contrary, abnormalities of VEP and BAER in EOCA patients were significantly less common (especially VEP) than in FA patients and were almost comparable to OPCA. Thus, on the basis of conventional evoked potential studies, EOCA patients seem to share some common features of FA and OPCA patients. There was no single EP feature to characterize EOCA as a distinct entity.

In a series of forty eight consecutive patients with parenchymatous mass lesions in the perirolandic area the central sulcus was identified intraoperatively in forty six. In patients in whom the mass lesion was seen on the surface, the relationship of the lesion to the somatosensory cortex and its underlying white matter was precisely determined. When the lesion was subcortical, the relationship of the mass lesion to the central sulcus on the MR image together with the central sulcus identified peroperatively, again, helped to determine the exact site of the tumour. Knowledge of the exact location of the tumour in relation to the central sulcus helped in the radical excision of seventy seven percent of these tumours. The morbidity following the operation was minimal.

A case of left cerebellar intraparenchymal schwannoma in an adult is being presented along with ten available reports in the literature, differential diagnosis and management of the condition.

Changes in the glycosaminoglycans (GAG), glycoproteins (GP) and lipid components of erythrocyte membrane were studied in two patients of neuroacanthocytosis, as compared to normal, control subjects. Concentration of GAG, decreased in the erythrocytemembrane in patients of neuroacanthocytosis while the carbohydrate components of the glycoproteins showed alterations both quantitatively and qualitatively. The ratio of W6 to W3 fatty acids showed increase in the erythrocyte membrane in neuroacanthocytosis patients while the ratio of cholesterol to phospholipids was decreased.

Three members of one family suffering from Marinesco Sjogren syndrome are being reported. All the three cases had mental retardation, congenital bilateral cataracts and cerebellar ataxia. It is a rare autosomal recessive disorder.

Primary leptomeningeal melanoma presenting as a lesion in the region of the pineal gland is rare. The MRI characteristic of this interesting condition is described and its value in studying spread or other foci is emphasized.

We report a female infant with features suggestive of Oral Facial Digital Syndrome (OFDS) Type I and associated cerebellar anomalies with Dandy-Walker malformation which suggest OFDS Type VI. The phenotypic overlap in this child OFDS type I and TypeVI raises the question as to whether they represent separate genetic entities. This pattern of abnormalities appears to be unique.

A case of spinal extradural cavernous haemangioma without a vertebral lesion is reported. Clinical and radiological features of the lesion are discussed. Relevant literature is reviewed.

A 47 year-old woman with Wegener's granulomatosis presented with an atypical onset of multiple symmetric cranial nerve involvement. The diagnosis was established by clinical examination and laboratory data. To our knowledge, this is the first case of Wegener's granulomatosis presenting with bilateral multiple symmetric cranial neuropathies.