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Biomedical subjects

M M Mehndiratta

Publications and source records attributed to M M Mehndiratta.

At least 19 recordsLinked to original sources
PubMed2006

Epilepsy in India: nuptiality behaviour and fertility.

PURPOSE: The study was conducted to assess the marital status and fertility in adults with epilepsy. METHODS: The study group consisted of 240 subjects with epilepsy aged 15 and above, Unmarried males (m) 55, unmarried females (f) 73 and married males 48, married females 64. Data was collected according to standard protocol regarding age, sex, age at marriage and onset of seizure, effect of epilepsy on marriage, type of seizure and control, fertility pattern and abortions. The study group was followed up for 30 months. The data was compared with latest available state and national data using Student's 't'-test, chi square, Fisher Z-test. RESULTS: The mean age of married subjects was 26.19 + 6.2 years and of the unmarried was 24.94 +/- 7.1 years. People with epilepsy had significantly (p < 0.05) higher mean age at marriage, significantly (p < 0.05) lower rate of marriage (m 46.6% f 46.72%), significantly (p < 0.05) higher divorce rate and withheld marriage. Females compared to males had higher divorce rate (f 5.84%) and withheld marriage (m 5.45% f 10.96%). The marriage rate of subjects with onset of epilepsy in the first decade (m 38.71% f 34.09%) and second decade (m 33.33% f 35.42%) was significantly lower (p < 0.05) than that of subjects with age at onset of epilepsy greater than 20 years (m 66.67% f 71.11%). Subjects with epilepsy not in remission had significantly (p < 0.05) higher mean age at marriage, lower marriage rate and lower fertility per person-year of marriage than those in remission. Majority (95.54%) did not disclose epilepsy before marriage. Total fertility rate (TFR) in males (4.56) and females (5.45) with epilepsy was comparable to general population. Abortions per pregnancy were 4.76%. CONCLUSION: Subjects with epilepsy had lower marriage prevalence rate, delayed marriage (especially females), withheld marriage and higher divorce rate compared to general population. Marriage rate was lower in people with age at onset of epilepsy less than 20 years and in whom seizures were not in remission. Majority of people with epilepsy did not disclose epilepsy before marriage. Though fertility was not affected in people with epilepsy as compared to general population, males had lower fertility than females.

Adolescent↗
PubMed2005

Adrenomyeloneuropathy.

Adrenomyeloneuropathy is a rare X-linked inherited disorder of peroxisomes characterized by the accumulation of very long chain fatty acids (VLCFA) in the central and peripheral nervous system, adrenal glands and testis leading to the dysfunction of these organs and systems. The phenotypic expression is highly variable, childhood cerebral adrenoleukodystrophy and adrenomyeloneuropathy being the main variants. Here we report a case of adrenomyeloneuropathy presenting initially as Addison's disease, which remained undiagnosed for many years with many rare features. We could not locate any other publication on this subject in the indexed journals of literature published from India.

Adolescent↗
PubMed2004

IgA monoclonal gammopathy of undetermined significance with peripheral neuropathy.

Monoclonal gammopathy of undetermined significance (MGUS) and peripheral neuropathy may be causally linked. In most cases, the M-protein is of IgG or IgM type. Peripheral neuropathy associated with IgA MGUS is uncommon, and there are limited reports. Here, we report a case of a 55-year-old male who was diagnosed to have symmetrical sensorimotor peripheral neuropathy associated with IgA MGUS with deposits of IgA-monoclonal protein in the myelin sheath.

Humans↗
PubMed2004

Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy.

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy is an uncommon progressive or relapsing paralysing disease caused by inflammation of the peripheral nerves. If the hypothesis that it is due to autoimmunity is correct, removal of autoantibodies in the blood by plasma exchange should be beneficial. OBJECTIVES: To evaluate the efficacy of plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. SEARCH STRATEGY: We searched the Neuromuscular Disease Group Register (December 2003), and MEDLINE (January 1966 to January 2003), EMBASE (January 1980 to January 2003), CINAHL (January 1982 to December 2002) and LILACS (January 1982 to January 2003). We also scrutinised the bibliographies of the trials, and contacted the trial authors and other disease experts. SELECTION CRITERIA: Randomised or quasi-randomised controlled trials in participants of any age comparing plasma exchange with sham treatment or no treatment. A trial showing no significant difference in the benefit from plasma exchange with intravenous immunoglobulin has been included in a separate Cochrane review. DATA COLLECTION AND ANALYSIS: Two authors selected the trials, extracted the data and assessed methodological quality independently. Where possible data were combined according to the methods of the Cochrane Neuromuscular Disease Review Group. MAIN RESULTS PRIMARY OUTCOME MEASURE: one crossover trial including 18 participants showed 2 (95% confidence interval (CI) 0.8 to 3.0) points more improvement after four weeks in an 11-point disability scale with plasma exchange (10 exchanges over four weeks) than with sham exchange. Rapid deterioration after plasma exchange occurred in eight of 12 who had improved. SECONDARY OUTCOME MEASURES: when the results of this trial and another with 29 participants treated in a parallel group design trial were combined, there were 31 points (95% CI 16 to 45) more improvement in an impairment scale after plasma exchange (six exchanges over three weeks) than after sham exchange. There were significant improvements in both trials in an electrophysiological measure, the proximally evoked compound muscle action potential, after three or four weeks. Non-randomised evidence indicates that plasma exchange induces adverse events in 3% to 17% of procedures. These are sometimes serious. REVIEWERS' CONCLUSIONS: Evidence from two small trials showed that plasma exchange provides significant short-term benefit in about two-thirds of patients with chronic inflammatory demyelinating polyradiculoneuropathy but rapid deterioration may occur afterwards. Adverse events related to difficulty with venous access, use of citrate and haemodynamic changes are not uncommon. More research is needed to identify agents which will prolong the beneficial action of plasma exchange.

Humans↗
PubMed2004

Hallucinations in Parkinson's disease--a study of forty three patients.

OBJECTIVE: To see the prevalence of hallucinations in patients with Parkinson's disease and to compare the various parameters between hallucinators and non-hallucinators. METHODS: Forty-three patients of Parkinson's disease were included. Detailed motor assessment was done with UPDRS scales. Assessment was done for the presence of depression and sleep disturbances. The patients were enquired for the presence of depression and sleep disturbance. The patients were enquired for the presence of hallucinations. Hallucination types and associated emotional experience were probed into. Comparative analysis was subsequently done between hallucinators and non-hallucinators. RESULTS: The mean age of the patients was 61.47 years while mean duration of symptoms of PD was 4.30 years. The mean UPDRS II and III scores were 15.18 and 38, respectively. Fifteen patients (34.9%) had experienced hallucinations. The hallucinations described were visual as well as auditory in nature. Majority of these patients (12 out of 15, 80%) had not volunteered about their hallucinations on their own. On analysis of various parameters between the hallucinators and non-hallucinators, it was observed that hallucinators were older and had a higher mean duration of symptoms of PD. The patients with hallucinations had a higher severity of motor symptoms and signs. Hallucinators more commonly had depression and sleep disturbances. Mean dosage of L-dopa equivalent dose was higher in patients with hallucinations as compared to those without hallucinations. On statistical analysis, however, only two parameters were found to be different in a statistically significant manner. These were the duration of illness and the Hoen and Yahr scale (p < 0.05). Also, hallucinations occurred independent of dopaminergic drugs. CONCLUSION: Hallucinations are common source of distress but are often neglected. One should always probe actively into the presence of hallucinations.

Delusions↗
PubMed2003

Clobazam monotherapy in drug naïve adult patients with epilepsy.

PURPOSE: Evaluation of the efficacy and side effects profile of Clobazam in a 24-week open-labelled trial involving 26 cases of drug naïve adult patients with epilepsy. METHODS: The study was an open labelled unicentre trial in which only drug naïve cases with epilepsy were included. A total of 26 cases were recruited. One case was dropped because he did not complete the desired follow up. Seizure type and frequency were recorded and follow up was done at 4, 8, 12, 18 and 24 weeks after initiation of therapy. The change in seizure severity, the dose of Clobazam required and development of side effects were recorded. RESULTS: The seizure types included GTCS (n=16), complex partial seizures (n=4), focal motor seizures with secondary generalisation (n=3) and juvenile myoclonic epilepsy (n=2). Out of 25 patients, 16 (64%) became seizure free, while five (20%) had >50% reduction in their seizure frequency. Thus, these 21 patients (84%) were considered to be well controlled. The commonest side effect seen was sedation, which was noted in 4 of the 25 patients (16%). However, in none of these four patients sedation was significant enough to warrant stoppage of therapy. Weight gain, gait ataxia, loss of short-term memory and breakthrough seizures were noted in one patient each. CONCLUSIONS: The efficacy of Clobazam coupled with the lack of significant side effects noted in our study makes it merit consideration as monotherapy in adult patients with epilepsy.

Adolescent↗
PubMed2002

Clinical expression and EEG features of patients with juvenile myoclonic epilepsy (JME) from North India.

We aimed to characterize the clinical profile, EEG features and response to treatment of juvenile myoclonic epilepsy (JME) patients. We studied 103 JME probands with a standard protocol recording age of onset, type, frequency of seizures, EEG data, detailed family history and response to treatment in a superspeciality university hospital in New Delhi. The mean age of onset of disease was 14.01 +/- 3.14 years with a male to female ratio of 1.19 : 1. The myoclonic jerks were present in all the probands, generalized tonic-clonic seizures (GTCS) were present in 75.72% and 11.65% probands had absence seizures. The incidence of febrile convulsion (FC) was higher (9.7%) in our JME probands reflecting some ethnic variation or ascertainment bias. There was a considerable delay (of approximately 5.26 +/- 4.61 years) in the diagnosis of JME in our probands because most of the patients were referred from private physicians who were possibly not familiar with this epileptic syndrome in this part of the world or thought it was a milder variety of GTCS. The family history was positive in 25.24% JME probands among first- and second-degree relatives. An interictal EEG was found to be abnormal in 81 (78.64%) patients with the predominant abnormality being generalized polyspike and wave (PSW) discharges (39.80% probands). The majority of patients (80.58%) showed a good response to treatment with valproate alone. There was a subset of patients (11.65%) who required the addition of other antiepileptic drugs (AEDs) for control of GTCS: 7.76% of JME patients were diagnosed as cases of GTCS by private practitioners before they were registered in our study and their seizures were well controlled on other AEDs (without valproate) prescribed by the referring physicians (carbamazepine-4, phenytoin-2, clobazam-2). It is concluded that the clinical features and EEG data of JME probands were comparable to reports from other parts of the world except for the fact that the incidence of FC was higher in our JME patients. There was a delay in the diagnosis of JME due to unfamiliarity with the epileptic syndrome among private practitioners in this part of the world. There was a subset of JME patients who had complete seizure control on other AEDs besides valproate.

Adult↗
PubMed2002

Neurological mitochondrial cytopathies.

The mitochondrial cytopathies are genetically and phenotypically heterogeneous group of disorders caused by structural and functional abnormalities in mitochondria. To the best of our knowledge, there are very few studies published from India till date. Selected and confirmed fourteen cases of neurological mitochondrial cytopathies with different clinical syndromes admitted between 1997 and 2000 are being reported. There were 8 male and 6 female patients. The mean age was 24.42+/-11.18 years (range 4-40 years). Twelve patients could be categorized into well-defined syndromes, while two belonged to undefined group. In the defined syndrome categories, three patients had MELAS (mitochondrial encephalopathy, lactic acidosis and stroke like episodes), three had MERRF (myoclonic epilepsy and ragged red fibre myopathy), three cases had KSS (Kearns-Sayre Syndrome) and three were diagnosed to be suffering from mitochondrial myopathy. In the uncategorized group, one case presented with paroxysmal kinesogenic dystonia and the other manifested with generalized chorea alone. Serum lactic acid level was significantly increased in all the patients (fasting 28.96+/-4.59 mg%, post exercise 41.02+/-4.93 mg%). Muscle biopsy was done in all cases. Succinic dehydrogenase staining of muscle tissue showed subsarcolemmal accumulation of mitochondria in 12 cases. Mitochondrial DNA study could be performed in one case only and it did not reveal any mutation at nucleotides 3243 and 8344. MRI brain showed multiple infarcts in MELAS, hyperintensities in putaminal areas in chorea and bilateral cerebellar atrophy in MERRF.

Adolescent↗
PubMed2001

Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy.

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy is an autoimmune peripheral neuropathy and would be expected to benefit from corticosteroids. Non-randomised studies suggest that corticosteroids are often beneficial. OBJECTIVES: To evaluate the efficacy of corticosteroids for treating chronic inflammatory demyelinating polyradiculoneuropathy. SEARCH STRATEGY: Search of the Cochrane Neuromuscular Disease Group register for randomised trials of corticosteroids treatment for chronic inflammatory demyelinating polyradiculoneuropathy and enquiry from subject experts. SELECTION CRITERIA: Types of studies: All randomised or quasi-randomised trials Types of participants: All patients with chronic inflammatory demyelinating polyradiculoneuropathy who were diagnosed by an internationally accepted definition. Types of interventions: Treatment with any form of corticosteroid or adrenocorticotropic hormone. Types of outcome measures: PRIMARY OUTCOME MEASURE: Change in disability 12 weeks after randomisation. SECONDARY OUTCOME MEASURES: 1. Change in impairment 12 weeks after randomisation. 2. Change in maximum motor nerve conduction velocity or compound muscle action potential amplitude after 12 weeks. 3. Side effects of corticosteroids. DATA COLLECTION AND ANALYSIS: One author extracted the data and the other checked them. MAIN RESULTS: We identified one randomised controlled trial, an open study in which 19 corticosteroid treated patients showed more improvement in impairment than 16 untreated controls after 12 weeks. Experience from large non-randomised studies suggests that steroids are beneficial. REVIEWER'S CONCLUSIONS: A single randomised controlled trial with 35 participants provided weak evidence to support the common opinion derived from non-randomised studies that oral corticosteroids reduce impairment in chronic inflammatory demyelinating polyradiculoneuropathy. Corticosteroids are known to have serious long term side effects. The long term risks and benefits have not been adequately studied.

Adrenal Cortex Hormones↗
PubMed2001

Creutzfeldt-Jakob disease : report of 10 cases from North India.

Creutzfeldt-Jakob disease (CJD) is increasingly being reported over the last three decades as a result of heightened awareness of the disease. Various studies have reported annual incidence of 0.5-1.5 cases of CJD per million of general population. In India, the disease is still under reported. Over the period spanning from 1968-1997, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore recorded 69 cases of CJD from different parts of India in the CJD registry. This paper describes the clinical experience with cases of CJD managed at the Department of Neurology, G.B. Pant Hospital, New Delhi from 1990-1998. In this series, the mean age of the patients was 53.80 (+/- 7.32) years and there were 5 females and 5 males. Myoclonus was present in all the cases and abnormal behaviour with or without other features was the presenting complaint in 7 of the 10 patients, while one patient of CJD had cerebellar ataxia as the presenting feature. One patient with occipital variant of CJD presented with acute onset cortical blindness and myoclonic jerks. One of the patients had acute psychosis precipitated by emotional stress at the onset. Extrapyramidal features were noted in 7 of the 10 patients before death. The mean duration of symptoms from the onset of disease to death was 6.6 (+/- 6.11) months. Classical EEG changes were observed in all the patients, except in one possible case of occipital variant of CJD, where we did not have access to EEG record. Brain biopsy could be undertaken in 3 patients, and in 2 patients the features of subacute spongiform encephalopathy (SSE) were noted.

Adult↗