Thirty five floppy children seen during two year period, were subjected to clinical examination, electroneuromyography and muscle biopsy. The muscle biopsy was sent for routine histology, histochemistry and electron microscopy. Using muscle pathology as the 'gold standard' for diagnosis, the aetiological entities were spinal muscular atrophy (16), congenital muscular dystrophy (6), mitochondrial myopathy (3), congenital fibre type disproportion (2), acid mutase deficiency (1) and benign congenital hypotonia (6). Mental subnormality, seizures, ptosis and ophthalmoplegia suggested mitochondrial disease (n=2). Macroglossia, hepatomegaly and cardiomegaly along with the dive bomber effect on electromyography were useful clues to the diagnosis of Pompe's disease (n=1). Positive decremental test established the diagnosis of congenital myasthenia in one patient. Contrary to most previously published reports, infantile onset of spinal muscular atrophy did not always spell a poor prognosis on follow up. 'Floppy infant syndrome' has varied etiology. Comprehensive evaluation including clinical, electrophysiological and detailed histological examination is necessary for proper diagnosis and prognosis of this heterogenous entity.

This study was carried out in sixty adult patients of either sex, to assess the efficacy of bupivacaine infiltration in different concentrations to prevent haemodynamic responses during craniotomy. The patients were randomly divided into 4 groups of15 each and scalp was infiltrated along the line of skin incision using either normal saline or 0.5, 0.25 or 0.125 bupivacaine. Patients were premedicated with morphine and promethazine and were anaestetised using conventional general anaesthesia technique including 02, N20 vecuronium and morphine. Ventilation was controlled so as to maintain PaCO2 between 25-30 mm Hg. EKG, heart rate and direct arterial pressure were monitored at various events during craniotomy. In control group there was a statistically significant increase in systolic arterial pressure and heart rate after scalp infiltration, scalp incision, during scalp flap reflection, burr hole formation, giglisaw operation and dural incision. In bupivacaine groups there was no significant alteration in arterial blood pressure or heart rate through out the period of study. On comparing the four groups it was observed that rise in arterial pressure and heart rate was significantly more in control group. Similarly a significantly more increasein systolic arterial pressure was observed in bupivacaine (0.125) group as compared to other trial groups. We recommend the haemodynamic changes during craniotomy.

Twelve patients (M:F 9:3) who fulfilled diagnostic criteria of chronic inflammatory demyelinating polyneuropathy (CIDP) were seen at NIMHANS over a period of three years (1987-1990). Their ages ranged from 20 yrs to 71 yrs and the mean duration of symptoms was 30 months (range 3 months to 6 yrs). Symptoms at the onset were dependent on the duration of disease. These included paraesthesia (7), weakness (4) and ataxia in lower limbs (1). Salient features on examintion were: distal weakness (10), proximal weakness (6), impaired touch and pain (12), impaired joint position and vibration sense (6), distal areflexia (12), bilateral impaired hearing (2) and thickened nerves (4). Electrophysiological evidence of demyelination was present in all and albumino cytological dissociation in CSF was noted in 55 of the patients. Sural nerve biopsy revealed significant loss of myelinated fibres in all the five patients studied. Increase in endo and perineural collagen, remyelination and schwann cell proliferation were also seen. Inflammatory infiltrates were conspicuously absent. Steroids were given in 10 patients. The therapeutic response was good in 5 and moderate in 5. Two patients had remitting relapsing course. Response to steroids could not be predicted on the basis of clinical or laboratory features. The recent diagnostic criteria and their therapeutic relevance are discussed.

Nineteen patients (14 males and 5 females), who had tetanus were evaluated for polyneuropathy by clinical examination, neuroelectromyography and sural nerve biopsy. Three patients (16) had clinically evident distal symmetrical polyneuropathy. Therewas a significant prolongation of mean median motor distal latency (p<0.02) peroneal motor distal latency (p<0.001) and reduction of sural sensory nerve action potentials (p<0.001) in these patients in comparison to controls. Electromyographic sampling of tibialis anterior muscle revealed wide variation in motor unit action potential duration and amplitude. Denervation phenomena including fibrilations and positive sharp waves were present in 4 patients (21) and complex repetitive discharges were present in 9 patients (47.4). Sural nerve histology was performed in 9 patients. It revealed mild to moderate loss of myelinated fibres in 4 cases (44.4). There is evidence that in tetanus there is a distal predominantly sensory polyneuropathy.

We report a South Indian family in which two living members were affected by a progressive neurogasrointestinal disorder characterized by dysmotility. Evidence for mitochondrial dysfunction was provided by increased arterial blood lactate, and ragged red fibres in muscle biopsy. Our report is believed to be the first description of the autosomal recessively inherited multisystem mitochondrial disease, mitochondrial neurogastrointestinal encephalopathy (MNGIE), from India.

This paper is a detailed description of an improvised operative technique used by us, for ventral screw fixation in a case of type II odontoid fracture.

Chronic subdural haematoma (SDH) complicating chronic idiopathic thrombocytopenic purpura (ITP), in an adult is extremely rare. There are only four such case reports available in the literature and all these patients had surgical evacuation of the haematoma. We report an elderly female with chronic ITP presenting with a chronic SDH which resolved spontaneously. This is possibly the first report of its kind in the world literature. The possible pathogenesis is discussed and pertinent literature reviewed.

A case of histologically verified ventro-laterally placed enterogenous cyst in the upper cervical region is reported.

A 38 year male suffering from cardioembolic stroke developed acute left hemiplegia and cortical sensory loss, which included graphanesthesia, impairment of two point discrimination and tactile inattention. CT scan revealed haemorrhagic infarction inright corona radiata and anterior limb of internal capsule. On day 13, cortical SEPS were absent and there was bifrontal and left parietal hypoperfusion on SPECT studies. Both the sensory loss, and SEP improved on day 28, which correlated with improvement in regional cerebral blood flow (rCBF). The cortical sensory loss, therefore, can also occur in subcortical lesion and may be due to cerebral diaschisis.

Two adult male patients having lumbar canal stenosis with prolapsed disc, developed claudicatory priapism along with classical symptoms of claudication, are reported in this article. The first patient complained of severe claudicatory pain in anal canal along with other symptoms. The second patient had a protruded bony rim at the lower margin of L4 vertebra in addition to the prolapsed disc. All the symptoms totally subsided after relieving the compression.

Germ cell tumours are rare intracranial tumours. The basal ganglionic location of these tumours comprise a rare group. Bilateral involvement of the basal ganglia by germ cell tumours is distinctly rare and have only been reported in Japanese patients. We present the first report of bilateral basal ganglionic germ cell tumour in a non-Japanese patient.