Eleven cases of Creutzfeldt Jakob Disease (CJD) were diagnosed at NIMHANS, Bangalore, between 1980-1992. The diagnosis was confirmed by necropsy (4), biopsy (2). The remaining cases were diagnosed by clinical and EEG features according to Master's criteria. Among these 11 cases, seven presented with significant psychiatric symptoms. All the seven cases analysed were male with an age range from 40-65 years. The average duration of symptoms before presentation was 2.4 months. The psychiatric symptomsobserved were predominantly mood symptoms, paranoid ideation, hallucinatory behaviour suggesting auditory and visual hallucinations and disturbances of high mental functions. This paper highlights varied psychiatric presentations in CJD patients which is seen in addition to dementia. An attempt is made to correlate psychiatric symptoms and neuropathological lesions found at autopsy among the four subjects to identify probablebiological substrate.

A Neuroepidemiological survey was carried at Malda district, 350 Km away from Calcutta; exactly at midpoint between Siliguri and Calcutta on a rural population of 37,286 (M 18,057; F 19,229; 1981 Census) under three Gram Panchayat areas with the help of non-professionals like Gram Panchayat members, ICDS workers, multipurpose health workers and motivated local youths, based on W.H.O. protocol (1981). House to house survey was carried out. Sensitivity reached 90 and Specificity 86. Total 961 individuals with neurological problems were screened and classified according to simple, but well defined criteria. Single disease was seen in 857 patients and 104 patients had double disease. Crude prevalence rate stands at ( 2856.26 per 1,00,000), epilepsy (305 per 1,00,000), vertigo (24.45 per 1,00,000), mental retardation (42.90 per 1,00,000), paralytic poliomyelitis (53.63 per, 1,00,000), movement disorders (26.81 per 1,00,000), spinal cord disorders (21 per 1,00,000), motor neurone disease (2.7 pre 1,00,000), development of speech and language (34 per 1,00,000). Age specific prevalence disease showed progressive increase in rate with advancing age upto 4th decade followed by a slightly decrease plateau upto 60 years of age and then sharp decline. Age and sex specific disease prevalence shows female dominance with maximum cases in 4th decade.

CT was performed in 51 patients at and caudal to the level of lumbar myelographic block due to disc herniation with or without lumbar canal stenosis. The causes of complete or almost complete myelographic block included disc herniation (27 patients)disc herniation with spinal stenosis (12 Patients), spinal canal stenosis (11 patients) and posterior osteophytes (1 patient). CT helped in detecting the non-visualised vertebral levels, demonstrated a surgically treatable lesion below the level of myelographic block and provided information for preoperative planning.

P300 event related potential was done in 23 chronic epileptics (12M, 11F) to assess subclinical cognitive impairment. No patient had any obvious neurological abnormality or brain damage. All had normal CT Scans and a mini mental scale score of at least 25. Neuropsychological evaluation done included Bender Gestalt test, Bhatia Battery, Wecshler Memory scale. P 300 event related potential was determined using 'Odd ball paradigm'. P300 latency was significantly increased in chronic epileptics (PCO < 001) compared to controls. There was no relation between P300 latency and amplitude with seizure onset, duration frequency. Nor was there any association between the three neuropsychological parameters and P 300 latency. We conclude subclinical cognitivedysfunction is common in epileptics and P300 event related potential could serve as a marker of cognitive impairment.

Infantile spasms is an age specific seizure disorder which occurs in infancy or early childhood either as a part of an epileptic syndrome accompanied by hypsarrhythmia or as chronic spasms involving the axial musculature with mental retardation. Information about infantile spasms in the Indian literature is very sparse. 29 patients of infantile spasms seen over a period of 5 years at NIMHANS were evaluated in this study. EEG was abnormal in all, with classical/modified hypsarrhythmia pattern in 5.14 were cryptogenic and the rest were symptomatic with birth asphyxia as the commonest underlying cause. All were treated with anticonvulsants and in addition 20 children received ACTH/Steroids. They were followed for a mean period of 18 months. The clinical status at the end of the follow-up, serial EEG findings and response in relation to the underlying cause and mode of therapy are discussed.

This is a report of a case of overlap myasthenic syndrome i.e. one which has the feature of both myasthenia gravis and lambert Eaton myasthenic syndrome (LEMS). The patient had first thyrotoxicosis and then was seen with bulbar and limb weakness andEMG showed decrement on repetitive tests at 3Hz. A second admission was for what appeared to be an axonal degeneration neuropathy with respiratory embarrassment. This was based on nerve conduction studies showing low muscle action potentials in all muscles with normal conduction velocities. There was a good response to steroids and relapse on stopping steroids and on the third admission all the electrophysiologic features of LEMS were noted. At his time the patient had bulbar and facial weakness and an excellent response to Prostigmine suggesting associated myasthenia gravis. This is the first overlap myasthenic syndrome reported from India.

Six histologically confirmed cases of esthesioneuroblastoma re reported. Two case had presented with primarily an intracranial mass, two had intracranial extension of a nasal tumour and the remaining two had only nasal growth. Local recurrence in the form of a swelling at the root of nose and metastases to the cervical vertebra, dorsal extradural space and lumbar subarachnoid space were seen in one case each. Aggressive treatment with surgery, radiation therapy and chemotherapy improved the qualityof life in locally recurrent and metastatic disease. CT scan findings and the role of chemotherapy in the primary metastatic esthesioneuroblastoma are discussed.

A rare case of ruptured dermoid in the region of corpus callosum and lateral ventricle is reported. The CT scan appearance of multiple fat density lesions in the subarachnoid space along with a non-enhancing cyst with or without calcification is pathognomonic of a ruptured dermoid cyst. Early intervention and excision of the dermoid cyst with washing of subarachnoid space and systemic steroids help to reduce the morbidity and mortality.

A case of POEMS syndrome is being reported. The case had all the features which are essential for the diagnosis. Agarose gel electrophoresis revealed monoclonal band in gamma region. However, we could not establish osteosclerotic myeloma as a case of this syndrome.

Peripheral neuropathy is an important manifestation of rheumatoid vasculiitis. It occurs in severe, long standing, seropositive, nodular, erosive rheumatoid arthritis (RA). A 56 year old female patient with fulminant RA, who developed a mononeuritismultiplex evolving into an asymmetrical neuropathy is described. The technique and utility of superficial peroneal nerve biopsy in patients with systemic vasculitis is discussed.

Extradural haematomas at more than two sites are extremely rare. Authors present two such interesting cases in the world literature for the first time. Their pathogenesis and prognosis are discussed.

The ninth case of axial intradural melanotic nerve tumour presenting with cervical cord compression is reported. The literature is reviewed and proposed theories on the origin of these tumours are discussed.