Cranial neuropathy in patients with leprosy.
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Cranial Nerve Diseases↗
Biomedical subjects
M Gourie-Devi
Publications and source records attributed to M Gourie-Devi.
Prevalence of neurological disorders in Bangalore, India: a community-based study with a comparison between urban and rural areas.
A population-based neuroepidemiological survey of 102,557 individuals in urban and rural Bangalore in Southern India was conducted to determine the prevalence and pattern of neurological disorders. The study population included subjects from urban (51,502) and rural (51,055) areas, identified through a two-stage stratified random sampling method. Trained social workers administered the screening questionnaire, which had been tested and validated in an earlier pilot study and a neurologist examined the individuals who screened positive. Adults, children (<15 years) and elderly adults (>60 years) constituted 61, 34 and 5% of the study group, respectively. There was a distinct difference in education, occupation and income levels between urban and rural areas with all these parameters being lower in the rural population. In the surveyed population, 3,206 individuals with neurological disorders were detected resulting in crude and age-adjusted prevalence rates of 3,126 and 3,355 per 100,000 population, respectively. The prevalence rate among children, middle-aged (31-40 years) and elderly adults was 2,653, 3,932 and 5,012 per 100,000 population, respectively. The prevalence of neurological disorders among women (3,617) was higher compared with men (2,657). The prevalence rate in urban and rural populations was 2,190 and 4,070/1,00,000, respectively, implying that neurological disorders were twice as frequent in rural areas as in urban areas. The prevalence rates per 100,000 population of the most frequent disorders in the descending order of frequency were: headache (1,119), epilepsy (883), febrile convulsions (330), cerebrovascular disorder (150), and mental retardation (142). This large-scale population-based survey provides data that will be crucial for developing hospital and community-based neurological services in India and other developing countries.
Adolescent↗
(-)-Deprenyl alleviates the degenerative changes induced in the neonatal rat spinal cord by CSF from amyotrophic lateral sclerosis patients.
Previous studies from our laboratory suggest the presence of toxic factor(s) in the cerebrospinal fluid (CSF) of patients with amyotrophic lateral sclerosis (ALS) which induces degenerative changes in the spinal cord neurons. The present work was carried out to investigate the role of (-)-deprenyl in attenuating these degenerative changes. CSF samples from ALS and non-ALS neurological patients were injected into the spinal subarachnoid space of 3-day-old rat pups, followed by a single dose (0.01 mg/kg body weight) of (-)-deprenyl, administered 24 h after CSF injection. After a further period of 24 h, the rats were sacrificed and the spinal cord sections were stained with antibodies against phosphorylated neurofilament (NF, SMI-31 antibody) and glial fibrillary acidic protein (GFAP). Activity of lactate dehydrogenase (LDH) was also measured. (-)-Deprenyl injection resulted in a significant (61%) decrease in the number of SMI-31 stained neuronal soma in the ventral horn of the spinal cord of ALS CSF exposed rats. This was accompanied by a reduction in the astrocytes immunoreactive for GFAP. There was also a significant (35%) decrease in the LDH activity following (-)-deprenyl treatment. These results suggest that (-)-deprenyl may confer neuroprotection against the toxic factor(s) present in ALS CSF.
Amyotrophic Lateral Sclerosis↗
Madras motor neuron disease variant, clinical features of seven patients.
Madras motor neuron disease (MMND) has the characteristic features of onset in the young, atrophy and weakness of the limbs, multiple cranial nerve palsies particularly the seventh, ninth to twelfth and sensorineural hearing loss with unique geographic distribution to southern part of India. During a period of 28 years (1974-2001), 7 (13%) among 54 patients of MMND seen at a tertiary referral center at Bangalore, India, had the additional features of optic atrophy in all and cerebellar involvement in three of them. There were three males and four females, the mean age at onset was 11.7 years, with a mean duration of illness of 6.4 years. All except one patient were ambulant and independent in the activities of daily living. Family history of MMND was present in more than a quarter (28.6%) of patients. Compared to MMND, these patients had onset of illness at a younger age and family history was more frequently observed, however, these differences were not statistically significant. Bulbar palsy was an invariable feature, being present in all patients compared to 38.3% of MMND and the difference was statistically significant (p=0.003). This clinical profile may be considered to be a variant of Madras motor neuron disease (MMNDV).
Adolescent↗
Long-term follow-up of 44 patients with brachial monomelic amyotrophy.
OBJECTIVES: Monomelic amyotrophy of a single upper limb termed "brachial monomelic amyotrophy" (BMMA) is a benign lower motor neuron disorder in the young, with male preponderance, insidious onset of atrophy and weakness, electromyographic evidence of neurogenic pattern without conduction block, slow progression for 2-4 years followed by a stationary course. The aim of the study was to determine whether (i) atrophy and weakness in the affected limb progresses beyond 5 years; (ii) the illness spreads to the other limbs; and (iii) the disease progresses to amyotrophic lateral sclerosis. MATERIAL AND METHODS: Forty-four patients who had a duration of illness of 5 years or more at the last follow-up examination were included in the study. Assessment of symptom profile, neurologic deficit and disability was performed at variable intervals during the follow-up period. RESULTS: Progression of the disease was seen in 37 (84.1%) patients, up to 5 years in 35 (79.5%), 6 years in one and 8 years in another patient. In seven patients (15.9%) the atrophy was accidentally noticed and no further change in the neurologic deficit was observed thereafter. Subsequent to attaining a stationary course, none of the 44 subjects developed fresh symptoms or signs during a mean follow-up period of 9.7 years (range 2.5-23). The mean duration of illness at last follow-up was 12.8 years (range 5-26.5) and in 22 (50%) subjects the disease duration was more than 10 years. Seven patients (15.9%) at presentation had minimal involvement of contralateral upper limb with gross asymmetry and later one more patient developed similar features. Thus, in only a small proportion (18.2%) of patients the neurologic deficit had extended beyond the confines of one upper limb. None of the patients developed involvement of cranial nerves, lower limbs or pyramidal signs. CONCLUSIONS: Progression of the neurologic deficit in the affected limb was seen up to 5 years in the majority followed by a stationary phase with no evidence of fresh neurologic deficit during the follow-up period. Spread to the contralateral upper limb with minimal neurologic deficit was seen in less than a fifth of the patients, but involvement of lower limbs was not observed. BMMA did not evolve to amyotrophic lateral sclerosis. These observations underscore the benign and self limiting course of BMMA.
Adolescent↗
Epilepsy control program in India: a district model.
Based on data originating from neuroepidemiologic studies in India, epilepsy has been identified as a public health problem. The treatment gap varies from 50 to 70% among persons with epilepsy and is compounded further by limited availability of neurologists, often working in urban metropolises, thus restricting the availability of services in a predominantly rural agrarian country. Cost-effective, sustainable epilepsy care programs are urgently required in India. To bridge this wide gap, a district model was developed with the central focus on training the district medical officers in providing epilepsy care with sensitization of state health administrators and nodal neurologists to support and sustain the program. Workshops were conducted for all three groups during the period 1999-2001, with the involvement of 21 state health administrators, 148 district medical officers, and 28 neurologists. Preliminary evaluation indicates a growing interest, enthusiastic commitment, and willingness among health professionals to expand epilepsy care to the periphery. The program revealed that it is possible to reach people with epilepsy on the premise of early diagnosis and management, need-based referral network, and a rational planning for uninterrupted availability of antiepileptic drugs along with education of families. Policy-level initiatives are required further to transform this on a wider scale and to evaluate the efficacy and effectiveness of this approach.
Delivery of Health Care↗
Early or late appearance of "dropped head syndrome" in amyotrophic lateral sclerosis.
BACKGROUND: "Dropped head syndrome" caused by neck extensor weakness has been reported in a variety of neuromuscular disorders. Previously published reports include isolated cases with amyotrophic lateral sclerosis (ALS). In this report, nine patients with ALS and dropped head syndrome seen during a 20 year period are described. PATIENTS AND INVESTIGATIONS: Between 1981 and 2000, 683 patients with ALS were diagnosed, based on El Escorial criteria. Nine of these had profound neck extensor weakness observed as an early feature, or developing during the later stages of the disease. The protocol for evaluation included detailed clinical history, neurological examination, electromyography, and nerve conduction studies. Investigations were undertaken to exclude malignancy, lymphoproliferative disorders, thyroid dysfunction, and collagen vascular disease. RESULTS: The incidence of dropped head syndrome was 1.3%. The mean (SD) age of the affected patients was 53.3 (10.3) years (range 33 to 65), with an equal distribution of cases in the fourth to seventh decades. In six patients, head drop was an early feature (mean interval from onset of illness 11.6 months (range 3 to 24)); in three it was late (between three and eight years after onset). In five patients, mild neck flexor weakness was present in addition to severe extensor weakness. In all nine patients there were diffuse upper and lower motor neurone signs. None of the patients had difficulty in breathing but all had difficulty in swallowing and social embarrassment, both of which could be corrected by simple measures. CONCLUSIONS: Dropped head syndrome is an important clinical sign and usually occurs as an early feature within the first one to two years after the onset of ALS. The cause of dropped head syndrome in these nine cases could be easily established as ALS by the presence of generalised signs.
Adult↗
Cyclophosphamide attenuates the degenerative changes induced by CSF from patients with amyotrophic lateral sclerosis in the neonatal rat spinal cord.
Our earlier studies have shown that cerebrospinal fluid (CSF) of patients with amyotrophic lateral sclerosis (ALS), when intrathecally injected into the neonatal rats, produces an aberrant phosphorylation of neurofilaments (NF) in the ventral horn neurons and reactive astrogliosis in the spinal cord. We wanted to investigate the effect of cyclophosphamide in the spinal cords of neonatal rats exposed to ALS-CSF. A single dose (5 microg in 5 microl saline) of cyclophosphamide was injected, 24 h after the administration of CSF samples from ALS and non-ALS neurological patients into the spinal subarachnoid space of 3-day-old rat pups. Rats were sacrificed after a period of 24 h, and stained with antibodies against the phosphorylated NF (SMI-31 antibody) and glial fibrillary acidic protein (GFAP). Cyclophosphamide treatment resulted in a 50% decrease in the number of SMI-31 stained neuronal soma in ventral horns of spinal cords of ALS-CSF exposed rats. This was accompanied by a decrease in the number of GFAP immunoreactive astrocytes. Furthermore, lactate dehydrogenase (LDH) activity was also decreased significantly, following cyclophosphamide treatment. These results suggest that cyclophosphamide could exert a neuroprotective effect against the neurotoxic action of factor(s) present in the ALS-CSF.
Amyotrophic Lateral Sclerosis↗
Sympathetic skin response in monomelic amyotrophy.
OBJECTIVES: Monomelic amyotrophy (MMA) a variant of motor neuron disease, has the characteristic features of wasting and weakness usually confined to a single upper or lower limb occurring predominantly in young males and a benign outcome. Symptoms of increased sweating, coldness and cyanosis have been observed in a few patients. The objective was to evaluate the involvement of the sympathetic nervous system in MMA by measuring sympathetic skin response. METHODS: Electromyography, motor and sensory nerve conduction studies were done in all the four limbs of 9 patients with atrophy of one upper limb. Stimulation at Erb's point, and above and below elbow was done to look for evidence of conduction block. The sympathetic skin response (SSR) was recorded in all the limbs of these patients. Wasting and weakness of right upper limb in 7 patients and left upper limb in 2 patients was seen. The mean age was 28.3+/-10.1 years. Twenty-five age matched (24.8+/-4.8 years) healthy subjects served as controls. RESULTS: The mean SSR latency in the affected upper limbs of 9 patients was prolonged compared to the 25 control subjects (1.51+/-0.07 s vs 1.42+/-0.19 s, P=0.03). The mean value of SSR latency in 18 upper limbs of the 9 patients which included atrophied and unatrophied limbs was also prolonged compared to the controls (1.50+/-0.08 s vs 1.42+/-0.19 s, P=0.05). There was no significant difference of the mean latency of SSR between the atrophied upper limbs and the clinically normal upper limbs (1.51+/-0.07 s vs 1.49+/-0.09 s, P=0.51). The mean SSR latency in the lower limbs of the patients (2.09+/-0.09 s) did not significantly differ from the control subjects (1.97+/-0.28 s, P=0.09). Motor and sensory nerve conduction was normal and there was no evidence of conduction block. CONCLUSION: In MMA the sympathetic nervous system is involved in the atrophic upper limb and also in the clinically unaffected upper limb but not in the lower limbs.
Adult↗
Disabilities in children with Duchenne muscular dystrophy: a profile.
Proper assessment of disabilities is essential for rehabilitation of patients with Duchenne muscular dystrophy. The aim of this study was to identify and quantify the disabilities in children with Duchenne muscular dystrophy and correlate them with impairment. Thirty-one patients with Duchenne muscular dystrophy of age four years and above were studied. The motor functions were evaluated using total motor score, upper and lower extremity function grades and timed function tests. Disability was quantified with Barthel index. The mean scores of motor scales were: total motor score -52 +/- 7.8, total functional grade -4.4 +/- 1.9 and timed function score -12.5 +/- 5.8. Barthel index scores ranged from 45-95 with a mean of 70.8 +/- 12.7. Motor scales correlated with each other and with Barthel index. Thirty children had disabilities in multiple spheres of life, which were significantly influenced by the motor power. Barthel index was useful in identifying and quantifying specific areas of disabilities in these children. Evaluation of disabilities using specific measures may be crucial for planning comprehensive management.
Activities of Daily Living↗
Pathology of Madras type of motor neuron disease (MMND)--a histological and immunohistochemical study.
A neuropathological report of Madras type of motor neuron disease (MMND) is presented and the differences from other forms of MND are discussed. An 18-year-old girl presented with nerve deafness and slowly progressive bulbo-spinal muscular atrophy, characteristic of MMND. Post-mortem examination of the spinal cord showed a severe loss of anterior horn cells, prominent dilatation of vessels, diffuse, but sparse sprinkling of microglial cells and lymphocytes, and demyelination and sclerosis of the ventrolateral columns. Neuronal depletion and marked gliosis was noted in the cochlear nucleus on both sides, while other bulbar motor nuclei were also involved. The cochlear nerve showed demyelination and axonal loss. Trigeminal and vestibular ganglia revealed features of ganglionitis. The possibility of an inflammatory aetiology for MMND needs to be considered.
Adolescent↗
HIV antibody profiles in serum and CSF of patients with neurological disease can serve as predictors of outcome.
The serum and CSF antibody profiles were investigated in 100 patients with HIV in relation to the type and severity of neurological disease. Among them, 87 were positive for anti-HIV antibodies in the CSF. In 30 of 87 patients detailed analysis by western blot could be performed. In 20 of 30 the profiles were dissimilar, with more of bands being found in the serum than in the CSF. The correlation of western blot profiles to the clinical outcome indicated that the number of anti-HIV antibody bands as well as the index in the CSF of fatal cases were significantly less compared with non-fatal cases (p=0.019 and p=0.039 respectively).
Adolescent↗
Profile of neurologic disorders associated with HIV/AIDS from Bangalore, south India (1989-96).
One hundred patients (95 males, 5 females, mean age at presentation 31.6 +/- 9.4 yr) with various neurological disorders associated with HIV infection during 1989-1996 were evaluated at NIMHANS, Bangalore. Eighty patients belonged to group I associated with opportunistic neuroinfections and 20 to group II--non infectious neurological disorders. Cryptococcal meningitis either alone (n = 31) or associated with tuberculous meningitis (n = 6) was the most common (46.3%) followed by neurotuberculosis either alone (n = 24) or with cerebral toxoplasmosis (n = 4) accounting for 35 per cent. Other opportunistic neuroinfections included cerebral toxoplasmosis, herpes zoster, fulminant pyogenic meningitis and neurosyphilis. Clinical characteristics, diagnostic clues, their laboratory and radiological profiles and problems encountered in diagnosis and management of these opportunistic infections are highlighted. In group II (19 males and one female; mean age of 32.6 +/- 9.4 yr), two patients had cortical dementia, three acute brain stem involvement, two epilepsy and one had features suggestive of progressive multifocal leukoencephalopathy. Two patients of group I during follow up developed cortical dementia. Six had peripheral nervous system involvement similar to Guillain-Barre syndrome. Sixty six patients (63 of group I and 3 of group II) progressed to AIDS, 33 patients from group I and one patient from group II succumbed to the disease. With the rapid increase in the incidence of HIV/AIDS and an increase in the neurological manifestations of HIV/AIDS it is important to recognise the magnitude of the problem for health planning in India.
AIDS-Related Opportunistic Infections↗
Reactive astrogliosis in neonatal rat spinal cord after exposure to cerebrospinal fluid from patients with amyotrophic lateral sclerosis.
Previous studies have proposed the presence of circulating toxic factor(s) in the cerebrospinal fluid (CSF) of patients with amyotrophic lateral sclerosis (ALS). In the present study we show that there is an increased number of astrocytes intensely immunoreactive for glial fibrillary acidic protein (GFAP) in the gray matter of the spinal cords of neonatal rats exposed to ALS CSF. There is also increased expression of GFAP in the astrocytes of the white matter of neonatal rat spinal cords exposed to ALS CSF. Western blot analysis also confirmed the increased expression of GFAP. Accordingly, our study provides for the first time a clear evidence for the pathological response of glia to the circulating toxic factor(s) in the CSF of ALS patients.
Amyotrophic Lateral Sclerosis↗
Correlation of clinical profile of myotonic dystrophy with CTG repeats in the myotonin protein kinase gene.
The molecular genetic analyses (PCR and Southern hybridization) of Indian patients with myotonic dystrophy (DM) were carried out to determine the degree of repeat expansion and an attempt was made to correlate the repeat number with disease severity. A scoring system based on the salient clinical features was devised to objectively assess the disease severity. The repeat expansion was seen in 11 of 12 patients examined and showed an inverse correlation with the age of onset confirming the phenomenon of anticipation. This was further established in the two pedigrees studied, clearly demonstrating both clinical and genetic anticipation. The clinical severity score, however, did not correlate well with the repeat number. Nonetheless, such molecular genetic analyses may have immense value as a screening procedure to identify premutations as well as in prenatal diagnoses.
Adolescent↗
Temporary amelioration of symptoms with intravenous cyclophosphamide in amyotrophic lateral sclerosis.
Based on the evidence that autoimmunity may play a role in the pathogenesis of amyotrophic lateral sclerosis (ALS), a variety of immunomodulating agents have been used in the treatment. In an uncontrolled trial we treated 44 patients of ALS with intravenous cyclophosphamide (IVCP) at a total dose of 1.5 g/m2 given over a period of 8 to 10 days. The patients were evaluated using neurological score which included bulbar, motor and daily activity scores before and following treatment. Twenty three patients showed a significant improvement (P=<0.001) in the composite and the individual scores. The improvement persisted only for 2 to 3 months. Amongst them the severely (7) and moderately (16) affected (score less than or more than 150) showed almost a similar response to treatment. A comparison of the improved group of 23 patients with the unimproved group of 21 patients did not reveal any significant factors which influenced the response to IVCP. However, there was a suggestion that patients below the age of 60 years and a duration of illness less than 12 months may respond to the drug. In conclusion, treatment with intravenous cyclophosphamide resulted in mild and temporary improvement in clinical status of the patients with amyotrophic lateral sclerosis. This may be considered as an alternative method of treatment in developing countries where newer drugs are not available and affordable.
Adult↗
Variable histomorphology of muscle in congenital muscular dystrophy.
Congenital muscular dystrophy (CMD) is a relatively uncommon disease with a controversial nosological status. That collagen synthesis could be the primary abnormality has been suggested earlier (Fidzianska et al., 1982). Amongst eighteen cases of CMD diagnosed during the past twelve years, muscle biopsy in three cases revealed prominence of myofibre necrosis and phagocytosis, and serum CPK was markedly elevated suggesting a rapidly progressive form. In twelve cases, marked increase in endomysial collagen, pronounced fallout of myofibres and significant fibre diameter variation was seen. This was associated with myonecrosis and regenerative activity of mild degree resembling the classical form of CMD. In the remaining three cases, polyfocal, polyphasic necrosis was noticed. Fibre splitting was more frequently observed, better delineated in the enzyme histochemical preparations, affecting both fibre types, while endomysial fibrofatty tissue was only moderately increased. The histomorphology in the latter group resembled that of limb girdle dystrophy. Ultrastructural findings in all the eighteen cases correlated well with light microscopic observations. lmmunohistochemical studies done on three of the eighteen cases showed normal localization of dystrophin protein. Such variable histomorphology, revealing a spectrum of myopathic features, suggests that the primary change in CMD is likely to be in the myofibre rather than in collagen synthesis.
Biopsy↗
Co-existence of cerebral cysticercosis with Japanese encephalitis: a prognostic modulator.
In this study, we investigated the frequency of co-existence of cerebral cysticercosis (CC) in Japanese encephalitis (JE) cases with special emphasis on its role in predicting the final clinical outcome. Amongst the 163 confirmed cases of JE, 37.42% (61/163) had co-existent CC. This was confirmed by antibody detection in the CSF of 45 cases, CT scan of the brain in 6 cases and at autopsy in 3 cases. In 2 cases confirmation was possible by CT scan as well as at autopsy, in 4, CSF antibody levels and CT scan were suggestive of CC while in 1, CSF antibodies and autopsy were suggestive of CC The co-occurrence of Cysticercus cellulosae in the brain emerged as a prognosticator of poor outcome in JE cases (P < 0.03).
Adolescent↗