Loss of motor function due to damage to nervous system is an important cause of disability among neurologically ill patient. Recent work in man and experimental animals has suggested that motor control is not only a function of cerebral cortex but also depends significantly on circuits in the spinal cord. A variety of mechanisms for natural recovery from motor paralysis have been proposed. These include dendritic sprouting, synaptogenesis, restoration of axonal transport, remyelination, unmasking of alternative pathways, removal of the effect of diaschisis, alteration in neurotransmitters and bilaterality of brain function. However in many a situations these are inadequate to provide functional independence to patients. A number of rehabilitation strategies for motor retraining have therefore been used. These are traditional exercise programs, neuromuscular re-education techniques, corrective surgical interventions, EMG biofeedback and use of pharmacological agents. More recent use of treadmill training with body weight support system and functional electrical stimulation have provided promising results. The basic underlying mechanism for the use of these strategies, their efficacy and limitations require critical evaluation. Neural transplantation may open new avenues for these patients who are incapacitated due to motor dyscontrol.

Forty two patients of chronic liver disease (CLD) were studied to evaluate the diagnostic utility of brainstem auditory evoked potentials (BAEP) and visual evoked potentials (VEP) with respect to development of hepatic encephalopathy (HE). The evoked potential (EP) evaluation was coincided with electroencephalography (EEG). A sequential analysis of electrophysiologic modifications occurring in response to dietary protein challenge or large-volume paracentesis was planned by carrying out the study in two phases. The first phase patients did not undergo provocation procedures. They were divided into group 1 (28 patients) and group 2 (14 patients) on the basis of respective absence or presence of neuropsychologically recognizable HE. Phase II included 13 patients of which 10 were given high-protein diet and 3 underwent ascitic tap. A control group of matched healthy individuals was also studied with the 3 step paradigm of electophysiologic assessment comprising VEP, BAEP and EEG. The EEG abnormalities were detected in 10.7 percent of non-HE and 64.2 percent of HE patient groups, without a statistically significant difference between the P100 mean latency scores of both these groups. Evaluation of BAEP revealed abnormalities in 17.8 percent patients of group 1 and 42.8 percent of group 2 enrolled under phase I, and 30.76 percent patients of phase II. The chief abnormality in the non-HE group was significantly higher mean I-V interpeak latency (IPL) scores compared to controls. All the 3 interpeak latencies I-III, III-V and I-V were abnormally prolonged in the HE patients group. There was no significant prolongation of various mean latencies after the provocation procedures in phase II patients, despite an appreciable upward trend in the BAEP interpeak latencies. The mechanisms underlying EP abnormalities in CLD are discussed. It is concluded that BAEP is a useful neurophysiologic device to follow up patients with CLD at risk of lapsing into overt HE.

Recent literature from different countries has revealed variable results of interferon treatment in subacute sclerosing panencephalitis (SSPE). Twelve patients with SSPE were treated at NIMHANS by concurrent use of intravenous and intrathecal interferon ALFA-2a, manufactured by recombinant DNA technology. The diagnosis of SSPE was based on clinical profile, EEG, CT and presence of antibody radio-immunoassay measles IgG antibodies in CSF. The total dose of interferon by intrathecal as well as intravenous route varied from 15 million to 60 million units. Clinical improvement as demonstrated by a decrease in scores on Neurological disability index occurred in three. Two of them relapsed five months after stopping the therapy but one patient maintained improvement two years after stopping treatment. There was no significant change in six patients. There were 3 deaths. One of them was autopsied. A decrease in titres of antimeasles antibody in CSF was noted in two patients who showed clinical improvement but in others there was no consistent correlation. No significant changes occurred in EEG during the treatment period. No serious side effect of interferon were encountered. Two patients had iatrogenic meningitis which could be treated successfully. Use of intravenous combined with intrathecal interferon is not promising in SSPE.

The relative frequency of carpal tunnel syndrome (CTS) among the patients referred for electrophysiological studies was studied. The case material included both inpatients and outpatients with various peripheral nerve disorders during the study period. CTS constituted 7 percent of all the peripheral nerve disorders and 83.6 percent of entrapment neuropathies. Eighty four patients were referred with the diagnosis of CTS and only 49 percent could be confirmed electrophysiologically. The presentation was that of acral and /or brachial paraesthesiae or pain.

Factors associated with stroke were studied by combination of morbidity and mortality analysis for the period 1986 to 1990. Strokes constituted 6.5-7.8 percent of total neurological registration over a five year period at NIMHANS, Bangalore. The morbidity pattern revealed that stroke increases with increasing age and was predominantly constituted by ischaemic strokes (73.1 percent). Stroke in the young constituted 27.8 percent of the cases. Analysis of one year mortality series revealed that 32.5 percent of deaths were in the age group of less than 40 years. Hypertension, Alcoholism and smoking were the major risk factors associated with stroke mortality to the extent of 32 percent, 12 percent and 11 percent of cases respectively. Aneurysms contributed for 10.0 percent of total deaths among the various other conditions associated with stroke. The case fatality rate was in the order of 25.1 percent. The present report emphasizes the need for well designed analytical studies for identifying and qualifying risk factors along with the focus on primary preventive measures through risk approach for preventing morbidity and mortality from stroke in India.

POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M protein and Skin changes) syndrome in a patient with solitary osteolytic plasmacytoma has been reported in this communication. He presented with a demyelinating polyneuropathy, lytic lesion in the left femur and hypogonadism. Serum protein electrophoresis, urine Bence-Jones protein and multiple bone marrow aspirations were negative for the presence of a myeloma. A biopsy from the lytic lesion confirmed the diagnosis of plasmacytoma.

A manifesting female carrier of Duchenne muscular dystrophy (DMD) with no prior family history of DMD is described. She presented with proximal muscle weakness, enlarged calf muscles and an elevated serum creatine phosphokinase (CPK). Histological myopathic changes and immunoperoxidase examination for dystrophy in muscle biopsy with antidystrophin serum proved to be valuable in the diagnosis of DMD in a manifesting female carrier.

Binswanger's disease is a form of vascular dementia, the diagnosis of which till recently was made only at necropsy. With the advent of MRI, the diagnosis can be made ante-mortem on the basis of Clinico-radiological correlation. One such case is reported.

We present a 19 years old male with bilateral sensorineural deafness. Primary optic atrophy and bilateral symmetrical, distal sensorimotor neuropathy. The illness began at the age of five years as a progressive disease without any family history.