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Biomedical subjects

R Tandan

Publications and source records attributed to R Tandan.

At least 37 records · Page 2Linked to original sources
PubMed1992

Topical capsaicin in painful diabetic neuropathy. Effect on sensory function.

OBJECTIVE: To examine the effect of capsaicin on sensory function in painful diabetic neuropathy. RESEARCH DESIGN AND METHODS: We examined the effects of topical 0.075% capsaicin cream on thermal and vibration thresholds in 22 subjects with painful diabetic neuropathy who participated in a double-blind vehicle-controlled therapeutic trial. RESULTS: After 8 wk of use, there was no significant change in warm and vibration thresholds, but the cold threshold was significantly reduced by capsaicin and vehicle creams to an equal degree. In fewer subjects who used capsaicin cream in an open-label study, there was no significant effect on sensory thresholds after up to 32 wk of use. CONCLUSIONS: Although our results and those of others show no adverse effects of topical 0.075% capsaicin on human sensory function, even in subjects with preexisting neuropathic sensory impairment, the small number of subjects tested does not justify an inferential statement on safety. Further studies in more subjects are warranted to ensure the long-term safety of capsaicin for pain relief in humans.

Administration, Topical↗
PubMed1992

Topical capsaicin in painful diabetic neuropathy. Controlled study with long-term follow-up.

OBJECTIVE: We conducted an 8-wk controlled study with topical 0.075% capsaicin in subjects with chronic severe painful diabetic neuropathy who were unresponsive or intolerant to conventional therapy. Capsaicin is an alkaloid found in capsicum peppers and produces desensitization to noxious thermal, chemical, and mechanical stimuli when applied topically. RESEARCH DESIGN AND METHODS: In 22 randomly assigned subjects, either capsaicin or vehicle cream was applied to painful areas 4 times/day. Pain measurements were recorded at baseline and at 2-wk intervals for 8 wk. RESULTS: Capsaicin treatment was more beneficial than vehicle treatment in the overall clinical improvement of pain status, as measured by physician's global evaluation (P = 0.038) and by a categorical pain severity scale (P = 0.057). Decrease in mean pain intensity by a visual analogue scale was 16% in capsaicin-treated and 4.1% in vehicle-treated subjects. Mean pain relief on visual analogue scale was 44.6 and 23.2%, respectively. In a follow-up open-label study, approximately 50% of subjects reported improved pain control or were cured, and 25% each were unchanged or worse. A burning sensation at the application site was noted by some subjects but both its magnitude and duration decreased with time. CONCLUSIONS: Results from this preliminary study suggest that topical 0.075% capsaicin may be of value in subjects with diabetic neuropathy and intractable pain.

Administration, Topical↗
PubMed1991

Intracranial arteriovenous malformations in two brothers with myotonic dystrophy.

We describe two brothers with myotonic muscular dystrophy and intracranial arteriovenous malformations who developed acute vascular neurological syndromes. The simultaneous occurrence of these two disorders in brothers could either be due to chance association, or due to a common causative mechanism, possibly a membrane abnormality.

Adult↗
PubMed1990

Metastasizing thymoma and myasthenia gravis. Favorable response to glucocorticoids after failed chemotherapy and radiation therapy.

Myasthenia gravis (MG) occurs in up to 44% of patients with thymoma. Thirty-three percent of these neoplasms are invasive but extrathoracic disease is rare. Recently, we saw a patient with MG and recurrent, metastasizing mixed lymphoepithelial thymoma, whose disease was resistant to combination chemotherapy and radiotherapy but who responded dramatically to treatment with daily glucocorticoids. Thus, therapy with daily glucocorticoids should be considered in the treatment of invasive or metastatic thymoma associated with MG, including when conventional surgery, radiotherapy, and chemotherapy have failed.

Drug Administration Schedule↗
PubMed1990

Giant axonal neuropathy: studies with sulfhydryl donor compounds.

Giant axonal neuropathy (GAN) is a disorder characterized pathologically by distal neurofilament-filled bulbous swellings in axons, and widespread collection of intermediate filaments, including masses of vimentin filaments in cultured skin fibroblasts. A morphologically similar neurofibrillary disorder is produced by acrylamide and the toxic hexacarbons, agents which bind to thiol groups. We report, in GAN fibroblasts, inhibition of vimentin filament aggregation by dithiothreitol and penicillamine, sulfhydryl donor compounds which stabilize thiols. In addition, we describe clinical improvement in a GAN patient treated with penicillamine, despite earlier progressive disease. These findings support the hypothesis of disordered thiol metabolism in GAN, and open up avenues for further research.

Adolescent↗
PubMed1990

Focal neuropathy preceding chronic inflammatory demyelinating polyradiculoneuropathy by several years.

We report three patients who exhibited an unusual clinical course of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in which mononeuropathic limb weakness developed 2, 11 and 23 years, respectively, before the development of generalized polyradiculoneuropathy. The eventual diagnosis remained uncertain until other causes of neuropathy were excluded, and the clinical disorder progressed to involve the other limbs. Focal or regional variants of CIDP suggest that the pathologic, and perhaps the immunologic, abnormalities can be localized and selective for prolonged periods of time. Although this clinical variant seems to account for a small number of CIDP cases, its recognition may aid in making an early diagnosis.

Adult↗
PubMed1990

Chronic segmental spinal muscular atrophy of upper extremities in identical twins.

We present the 1st report of chronic segmental spinal muscular atrophy confined to the upper extremities in identical male twins. This occurrence in identical twins, together with reports of siblings and parent-child pairs of a disorder phenotypically similar to the more common sporadic form in the literature, suggests a genetic etiology in some cases.

Aged↗
PubMed1990

Benign autosomal dominant syndrome of neuronal Charcot-Marie-Tooth disease, ptosis, parkinsonism, and dementia.

We present a kindred with a previously undescribed combination of neuronal Charcot-Marie-Tooth disease, ptosis, parkinsonism, and mild dementia. The propositus, a 72-year-old man, had pes cavus, peripheral neuropathy, ptosis, parkinsonism, hyperreflexia, orthostatic hypotension, central hypoventilation, and mild dementia. Peripheral electrophysiologic studies showed features of an axonal neuropathy. The electroencephalogram showed intermittent 2 to 4 Hz activity symmetrically in the hemispheres. Several family members in 3 generations had pes cavus, neuropathy, ptosis, parkinsonism, and dementia although not all of the features were consistently present. Survival past the 7th decade was common. Autopsy in 2 affected members revealed the neuropathy to be axonal in type and showed mild to moderate loss of anterior horn cells in the spinal cord and pigmentary loss with gliosis in the substantia nigra. This is a unique, benign, autosomal dominant syndrome which shows complete penetrance, variable expression, and both central and peripheral nervous system involvement.

Adult↗
PubMed1989

Quantitative measures of neurological function in chronic neuromuscular diseases and ataxia.

Interexaminer and intraexaminer reliability were determined for 30 quantitative measures of neurological function, including sensory threshold, tendon reflexes, maximum isometric strength, and timed tests and coded ratings of functional ability, in patients with Charcot-Marie-Tooth disease (n = 30), idiopathic polyneuropathy (n = 16) and spinocerebellar degeneration (n = 30). Five of 6 sensory and reflex measures had interexaminer reliability greater than 0.80 when the neurologists' examinations were 1 h apart; 2 of 7 achieved this level when the examinations were separated by 1 month. Interexaminer reliability between physical therapists was greater than 0.80 for 19 of 20 measures of strength and functional ability. Intraexaminer reliability coefficients greater than 0.80 were found for 13 of 24 sensory and reflex measures, 28 of 30 isometric strength measures, 24 of 30 timed tests and 5 of 6 coded ratings of functional ability. Electro-oculography, oral diadochokinetic syllable rates, hand and foot accelerometry, and tracings of sine and square waves were examined as quantitative indices of ataxia for patients with spinocerebellar degeneration. Of 11 measures of ataxia, only the oral diadochokinetic syllable rate test had acceptable reliability.

Activities of Daily Living↗
PubMed1989

Adult-onset autosomal recessive neurogenic scapuloperoneal syndrome.

We report a brother and sister who showed weakness, atrophy and fasciculations starting in adulthood, predominantly affecting the muscles of the shoulder girdles and distal lower extremities. Mild ptosis and facial weakness were present in both. Electrophysiological studies and muscle histology supported a neurogenic basis for the autosomal recessive scapuloperoneal syndrome in this sibship.

Adult↗
PubMed1989

DNA repair deficiency for alkylation damage in cells from Alzheimer's disease patients.

We have shown that fibroblasts, lymphocytes and lymphoblasts from patients with Alzheimer's disease (AD) are deficient in the repair of DNA damage induced by the alkylating agents methylmethane sulfonate (MMS) and N-methyl-N'-nitro-N-nitrosoguanidine (MNNG). Unscheduled DNA synthesis and alkaline elution studies of DNA repair using human skin fibroblasts obtained from patients coming to autopsy have shown that the cells from autopsy confirmed cases of AD have lower levels of DNA repair synthesis after exposure to varying concentrations of either MMS or MNNG. Lymphoblasts derived from individuals with dominantly inherited AD have also been used to study DNA repair. Alkaline elution analysis of DNA repair after exposure to 200 uM MMS or 6 uM MNNG indicates that there is significantly less repair in the lymphoblasts from AD patients. When healthy control cell lines repaired and cell lines from AD patients were exposed to MMS or MNNG respectively significantly less repair occurred in the AD cells. After studying five at risk individuals results indicate that cell lines from two of these people have low levels of DNA repair and three lines have normal repair. These findings support the hypothesis of a DNA repair deficiency in familial AD. Monocytes from healthy control subjects and putative AD patients were analyzed for mutant frequency and DNA repair capacity. Results of unscheduled DNA synthesis experiments using monocytes from 7 healthy controls and 9 presumed AD patients indicates that there is a decreased ability of AD cells to repair MMS and MNNG induced DNA damage.

Alkylating Agents↗
PubMed1988

Continuous motor unit activity confined to the upper extremities.

We report a patient with clinical vermiform motor activity, muscle cramps, delayed relaxation of grip, and continuous motor unit discharges who developed mild symmetric symptoms in the upper extremities following a viral infection. Treatment with carbamazepine produced considerable symptomatic improvement. Needle electromyography (EMG) in several muscles showed irregular grouped spontaneous discharges composed of potentials resembling normal motor units. The discharges occurred asynchronously in distal muscles with identical and different peripheral nerve innervation but were not seen in more proximal muscles innervated by the same nerves. Our EMG findings indicate an origin of this spontaneous activity in the terminal branched motor nerves.

Arm↗
PubMed1988

Double-blind controlled trials of Cronassial in chronic neuromuscular diseases and ataxia.

We report three 12-month, double-blind, three-phase studies comparing the effect of placebo and 40 mg and 100 mg IM daily of purified bovine brain gangliosides (Cronassial) in chronic neuromuscular diseases. Thirty patients with Charcot-Marie-Tooth disease, 16 with idiopathic polyneuropathy, and 30 with spinocerebellar degeneration had neuromuscular function measured monthly by quantitative testing of motor and sensory function, coordination, and electrophysiologic factors. Analysis of these studies, and of longer term (up to 2 years) open studies of 100 mg daily of Cronassial in 67 patients failed to show therapeutic efficacy of Cronassial. Statistical power calculations indicated that five of the 37 measures had greater than a 70% chance of detecting a 20% difference in the rate of progression of the active-drug and placebo groups. A number of measures significantly improved during prolonged placebo treatment, suggesting that the placebo effect has a strong influence on "objective" measures of neuromuscular function.

Adult↗
PubMed1988

Early-onset benign autosomal dominant limb-girdle myopathy with contractures (Bethlem myopathy).

We report a large French-Canadian kindred with 33 affected members in six generations showing early-onset autosomal dominant limb-girdle myopathy and contractures. This myopathy is unique because of its benign course, with many members only minimally impaired even in old age. Examination of affected members revealed mild to moderate proximal weakness and wasting. Contractures were observed at the elbows and ankles in all, while in some they were more widespread. Serum CK was either normal or slightly raised, and electrodiagnostic studies suggested a primary myopathy. Muscle biopsy revealed nonspecific features of a myopathy without fiber necrosis or regeneration. Cardiac involvement was absent clinically in all patients and at autopsy in two affected individuals. The similarities between four previously reported families and our own establishes this myopathy as a distinct clinicogenetic entity, for which we propose the name "Bethlem myopathy."

Adolescent↗
PubMed1987

Alzheimer's disease cells exhibit defective repair of alkylating agent-induced DNA damage.

The most common cause of senile and presenile dementia is Alzheimer's disease, a disorder with an undetermined cause. A number of studies have indicated that neurons from patients with Alzheimer's disease have decreased ribonucleic acid levels and reduced protein synthesis. Recent studies using lymphoblasts from patients with Alzheimer's disease have indicated that these cells are more sensitive to deoxyribonucleic acid (DNA)-alkylating agents. We have used cell survival, unscheduled DNA synthesis, and alkaline elution to assess the capacity for DNA repair in skin fibroblasts from normal control subjects, control subjects with central nervous system disease, and patients with Alzheimer's disease. Our results indicate that the Alzheimer's disease cells, unlike normal cells, fail to repair methylmethane sulfonate-induced DNA damage. Both normal and Alzheimer's disease cells are able to ameliorate the effects of ultraviolet light. These results indicate that a specific pathway for DNA repair is affected in Alzheimer's disease. The repair defect may be related to the cause of the disease or may be the cause of the disease.

Adolescent↗