A case control study and one year follow-up of registered epilepsy cases in a resettlement colony of North India, a developing tropical country.

A population-based case control study and monthly follow-up of 121 registered epilepsy cases was conducted during 1995-1997 in a resettlement colony of Chandigarh, India. History of various tentative risk factors, e.g. trauma, febrile seizures, family history of seizures, alcohol intake and other possible causes was elicited. An age- and sex-matched control was selected from the neighbouring families for each case. A discordant pair analysis was done for matched case/controls. History of head injury, febrile seizures and developmental delay was observed exclusively in cases (none present in controls). Odds for epilepsy were higher among people who had positive family history (O.R.= 2.1, chi2 = 5.5, C.I. = 1.1-4.3). All cases were followed up and interviewed for history of seizures and drug intake. Fourteen cases could not be followed up completely. Ninety-four (88%) of the remaining 107 cases did not have any seizures during the follow-up. Of them, 70 (75%) patients were not on medication, 13 patients were on phenytoin and 11 patients received phenobarbitone. Thirteen cases reported seizures during the follow-up. Four patients out of the latter had mental retardation and were not on medication. Cumulative incidence of epilepsy was estimated to be 0.6/1000 person-year exposure.

Davidenkow's syndrome : a clinical, electrophysiological and histomorphometric evaluation.

Davidenkow's syndrome is being reported in three members of a family. The affected subjects had weakness and wasting of the deltoids and the distal muscles of the lower limbs. The onset of the disorder was in the middle of the second decade and the affected subjects retained the capability of independent mobility till the fifth decade. Neuroelectromyographic studies revealed the presence of a demyelinating neuropathy. Sural nerve histology was characterised by loss of myelinated fibres and segmental demyelination.

Visual and auditory evoked responses in acute severe hepatitis.

Evoked responses have not been studied in patients with acute severe hepatitis (ASH) with or without hepatic encephalopathy. This prospective study was undertaken to find out diagnostic as well as prognostic value of visual evoked responses (VER), and brain stem auditory evoked responses (BAER) in patients with ASH with or without encephalopathy. Visual evoked responses and BAER were studied in 20 patients (14 males and six females) with ASH. The patients were diagnosed as having severe hepatitis if acute hepatitis was associated with raised serum bilirubin and serum transaminases, and if they had a prothrombin time index of < 50%. After a detailed neuropsychiatric examination of each patient, the study sample was divided into two groups of 10 patients: ASH without encephalopathy (ASH-WOE), and ASH with encephalopathy (fulminant hepatic failure, FHF). The median P100 latencies of FHF patients were significantly increased compared with controls and patients in the ASH-WOE group. Abnormal P100 latencies, exceeding 95th percentile values of the controls, were present in one patient in the ASH-WOE group and six patients in the FHF group. The median interpeak latencies I-III, III-V and I-V were significantly prolonged in the FHF group. Interpeak latencies III-V were also increased significantly in patients in the ASH-WOE group. While abnormal BAER were seen frequently in both groups, VER abnormalities were largely confined to patients in the FHF group. In the FHF group, six out of 10 patients survived and exhibited clinical improvement in the status of hepatic encephalopathy. Evoked responses were repeated after 2-3 weeks of recovery in these patients and VER abnormalities showed a tendency to normalize, thereby suggesting a prognostic implication. The incidence of abnormal VER in hepatic encephalopathy complicating ASH far exceeded that of abnormal BAER. Markedly prolonged P100 latencies in FHF patients indicate poor prognosis.

Auditory function after lumbar puncture.

This study was undertaken to evaluate the incidence and degree of hearing loss by clinical and audiometric analysis after lumbar puncture and to determine the site of involvement in brainstem auditory evoked response (BAER) studies. We also wanted to determine whether the degree of hearing loss is related to the size of the spinal needle used. Twenty patients were randomised for lumbar puncture into 2 groups, one using 20G and the other using 24G needle. Audiometry and brainstem auditory evoked response studies were carried out before and 24 hours after lumbar puncture. There was no statistically significant change in the hearing levels (on audiometry), before and after lumbar puncture, in cases belonging to both the groups (20G and 24G). There was no statistically significant change in the latency of each wave (on BAER) before and after lumbar puncture in cases belonging to both the groups (20G and 24G). No significant change either in hearing level on audiometry or in latency of BAER waves was noted when the two groups (20G, 24G) were compared with each other. In the present study, there was no indicator to suggest that the size of the lumbar puncture needle influenced auditory function.

Peripheral neuropathy in tetanus : a clinical, neuroelectrophysiological and histological evaluation.

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.

Hereditary liability to pressure palsy : a clinical electrophysiological and histomorphometric case study.

An 18 years old female patient presenting with a peroneal nerve palsy after trivial pressure has been reported. A diagnosis of hereditary liability to pressure palsy was not suspected until electrodiagnostic studies were performed. These revealed the presence of a demyelinating polyneuropathy with conduction block and differential affection of various segments of the nerves. Sural nerve histology was charcterised by the presence of suasage like swellings and segmental demyelination and remyelination in teased fibres and mild loss of large myelinated fibres.

Effect of sorbinil and ascorbic acid on myo-inositol transport in cultured rat Schwann cells exposed to elevated extracellular glucose.

The effect of long-term (2 weeks) exposure to 0-50 mM glucose and 0-1 mM sorbitol on myo-inositol metabolism was studied in cultured rat Schwann cells. Experiments were carried out to determine the effect of sorbinil and ascorbic acid on myo-inositol uptake in rat Schwann cells cultured in the presence of increased extracellular glucose or sorbitol. myo-Inositol uptake and its incorporation into phospholipids decreased significantly when cells were grown in > or = 30 mM glucose for a period of 2 weeks. This inhibitory effect was partly blocked by sorbinil, an aldose reductase inhibitor, in a dose-dependent fashion. Significant prevention was achieved with 0.5 and 1 mM sorbinil. Ascorbic acid also prevented the reduction in myo-inositol uptake due to excess extracellular glucose, at 3 and 30 microM concentrations, but not at 300 microM. Neither sorbinil nor ascorbic acid could prevent the alterations in myo-inositol transport in cells exposed to high sorbitol levels for the same period of time. These data suggest that glucose-induced alteration of myo-inositol transport in Schwann cells is mediated, at least in part, via sorbitol accumulation. This myo-inositol transport impairment is prevented by sorbinil and also by ascorbic acid. Ascorbic acid may hold a fresh promise for the treatment/prevention of diabetic neuropathy/complications, at least as an adjunct therapy along with known aldose reductase inhibitors.

Evaluation of epilepsy management in a developing country: a prospective study of 407 patients.

407 patients (248 men, 159 women) of epilepsy attending the neurology clinic were evaluated to find out the profile of epilepsy, cost-effectiveness of various investigations, therapeutic regimens and efficacy of referring physicians in a developing country. At the time of onset of seizures 67.2% of patients were in the second and third decade. Generalised tonic clonic seizures were the commonest seizure type seen. Specific aetiology was established in 20.8% cases only. Neurocysticercosis was the commonest cause observed. The main source of referral (50.1%) was general practitioners. Referral diagnosis was incomplete in 52.8% of the cases. Investigations did not alter the diagnosis in 62.5% cases. The EEG was useful in the management of 15.1% cases of epilepsy. The skull x-ray and chest x-ray were abnormal in 1.7% and 2% cases respectively. CT scan revealed abnormality in 39.4% cases. Most of the patients were treated with monotherapy. Phenytoin, phenobarbitone and carbamazepine were the common drug used. Out of 246 cases who were started on anticonvulsant therapy prior to referral, the choice of drug was wrong in 78 (31.7%) cases and dose was inappropriate in 121 (49.2%) cases. It was concluded that most important factor for cost effective management of epilepsy is proper clinical evaluation and education of general physicians in this direction.

Clinical profile of Subacute scelerosing panencephalitis (SSPE).

Thirty nine confirmed cases of SSPE were analysed retrospectively to study the clinical profile and to look for any atypical presentations. There were 29 males and 10 females with the age ranging from 4-19 (mean 11.5 × 3.5) years. Sixteen patients (41.0 percent) had received measles vaccination. Definite history of measles was available in 33 (84.6 percent) cases. Onset of symptoms was after 10 years of age in 58.9 percent of cases. Majority (56.42 percent) had presented within six months of onset of symptoms but 5 patients (12.8 percent) had symptoms for more than 3 years prior to the presentation. Decreased scholastic performance (58.97 percent) and myoclonus (33.33 percent) were the common presenting symptoms. Generalized tonic clonic seizures (2 cases), visual deterioration (2 cases) and altered sensorium (1 case) were the unusual presenting features. Ophthalmological manifestations were seen in 6(15.4 percent) patients. One patient had diminution of hearing in earlier stages illness. Mean age of onset of symptoms was significantly different (p < 0.01) in vaccinated (13.10 × 2.84 years) and non-vaccinated (9.87 × 3.44 years) group. Oligoclonal bands in cerebrospinal fluid (CSF) revealed a positivity in 79.4 percent of cases. Antimeasles antibody titres in serum and CSF were significantly raised in 31 cases (79.5 percent). Electroencephalogram (EEG) revealed periodic complexes in 38 cases (97.4 percent).

Electrophysiological assessment of early hepatic encephalopathy in chronic liver disease.

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.

POEMS syndrome associated with solitary, osteolytic plasmacytoma : a case report.

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.

Churg-Strauss syndrome presenting as mononeuritis multiplex : a case report.

A 25 year old female presented with mononeuritis multiplex and pulmonary symptoms. Investigations revealed the presence of marked peripheral blood eosinophilia. Skin and nerve biopsies were consistent with a diagnosis of Churg Strauss Syndrome. These were characterized by vasculitis of the small arteries and veins and perivascular eosinophilic infiltrates. The patient showed good response to cyclophosphamide and steroids.

Ipsilateral cerebral events.

Eleven patients of ipsilateral seizures or ipsilateral neurological deficit were studied under the heading of ipsilateral cerebral events. Ipsilateral localisation was based on electroencephalogram (EEG) and computed tomography (CT) scan or both. Group A had 4 patients who had predominantly motor neurological deficit and ipsilateral pathology was evident on CT scan. Surgery re-confirmed the lesion in one case. Group B had 7 patients who had partial motor seizures with or without generalisation. EEG showed ipsilateral focus in all but only 3 patients revealed ipsilateral lesion on CT scan. All patients showed improvement on medical treatment except one case which underwent surgery. Various hypotheses are put forward for the ipsilateral phenomenon and the mechanism implicated are discussed.

Evoked potentials in hereditary spastic paraplegia.

Pattern reversal visual evoked response (VER) and monaural stimulation of brainstem auditory evoked responses (BAER) were recorded from both sides in 25 patients (males 19; females 6) with hereditary spastic paraplegia (HSP). Their age ranged from 15-52 (mean +/- SD; 25.2 +/- 22.5) years and duration of symptoms 6 months-9 (mean +/- SD; 4.2 +/- 3.6) years. A prolonged P100 latency was seen in 6 patients and BAER abnormality in 13. None of the patients had clinical evidence of brainstem involvement. It is suggested that VER and BAER abnormalities are due to segmental demyelination and fiber loss in central conduction pathways and could serve as an important tool for the diagnosis of this disorder.

Polymyositis : a clinical profile.

Clinical data of 132 patients of polymyositis seen over a period of ten years was retrospectively analysed. Important features included a mean age of 32.31 years with a range of 8 years to 70 years. 40.62 percent patients were of primary idiopathic polymyositis while 53.13 percent presented with disease of more than six months duration. Pelvic girdle weakness was demonstrated in 31 cases (96.87 percent). Creatin phosphokinase (CPK) values were consistently elevated in 81.25 percent of these cases. Electromyography in majority showed mixed neurogenic and myopathic pattern. Muscle biopsy was performed in 23 patients and all revealed changes characteristic of polymyositis. Follow up was available in 18 cases.

Neurocryptococcosis in acquired immunodeficiency syndrome.

Two cases of Neurocryptococcosis in AIDS are described. The first patient presented with generalised lymphadenopathy, hepatosplenomegaly and raised intracranial tension. The clinical manifestations of second patient included prolonged fever and hepatosplenomegaly. Both the patients had evidence of disseminated cryptococcosis. The distinctive clinical manifestations, investigative abnormalities and modification of therapy of neurocryptococcosis manifestations in AIDS are discussed.

Serum vitamin E estimation in patients of spinocerebellar degeneration.

Serum Vitamin E was estimated in twenty two consecutive patients of spinocerebellar degeneration. Serum vitamin levels were found to be normal in all these patients. Role of Vitamin E in spinocerebellar syndrome is discussed.