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Biomedical subjects

A B Taly

Publications and source records attributed to A B Taly.

At least 37 records · Page 2Linked to original sources
PubMed2004

Is low serum tocopherol in Wilson's disease a significant symptom?

BACKGROUND: Free radical mediated injury is increasingly recognized in many metabolic diseases including Wilson's disease (WD). Use of antioxidants as an adjunctive therapy in WD may have therapeutic significance. AIM: The aim of the study was to correlate serum levels of tocopherols with serum copper and ceruloplasmin and clinical status of these patients. METHODS: Serum levels of tocopherol of were measured spectrophotometrically using the Emmerie-Engel reaction in 34 patients from a large cohort of WD being followed up at a tertiary care center. RESULTS: Majority of patients were male (M/F=23:11). The mean serum copper was 43.6+/-26.2 microg/dl (range=10-121 microg/dl) and serum ceruloplasmin was 5.6+/-5.5 mg/dl (range=0-30 mg/dl). The mean serum tocopherol level was 0.68+/-0.18 mg/dl (range=0.23-1.14 mg/dl) and compared to the control (1.07+/-0.17 mg/dl), nearly 59% of patients had decreased levels (p<0.001). No significant correlation was noted between low serum tocopherol levels and serum copper levels, Mini Mental Status Examination (MMSE) scores and CHU staging. However, serum tocopherol levels were lower in patients with relatively short duration of treatment (7.8 years vs. 12.4 years). CONCLUSION: Decreased levels of serum tocopherol were detected in 59% of patients compared to controls. However, low tocopherol levels did not correlate with clinical status or biochemical parameters of WD, except for relatively shorter duration of treatment. Further studies, especially in newly diagnosed patients, need to be done to validate the role of low tocopherol levels in Wilson's disease.

Adolescent↗
PubMed2004

HIV-1 clade-C-associated "ALS"-like disorder: first report from India.

A patient of ALS-like disorder in an HIV-1 clade-C-infected heterosexual male is being reported. A 37-year-old gentleman presented with subacute, progressive asymmetrical onset of weakness and wasting of upper limbs associated with brisk muscle stretch reflexes and without any sensory or sphincter involvement. While nerve conduction tests were normal, the EMG of proximal and distal limb muscles on both sides revealed evidence of denervation and reinnervation. Routine blood and urine tests and investigations for underlying causes of motor neuron disease were noncontributory. He was HIV-1, subtype clade C seropositive. A diagnosis of HIV-related anterior horn cell disease was considered and zidovudine, lamivudine and nevirapine were started. After 1 month, there was a subjective improvement of 10% and objective improvement in strength of muscles of proximal upper limb on both sides by one grade power on MRC scale. Reports of amyotrophic lateral sclerosis (ALS)-like illness in HIV are sparse. The reversibility of "ALS"-like features in this subgroup of patients might offer an insight into the pathogenesis of amyotrophic lateral sclerosis. This is a first report of ALS-like illness caused by subtype C of HIV-1 strain.

Adult↗
PubMed2004

Successful pregnancies and abortions in symptomatic and asymptomatic Wilson's disease.

BACKGROUND: There are only a few reports regarding the fertility and outcome of pregnancy in Wilson's disease (WD) and none from India. The authors in this study discuss various aspects of fertility in 16 women with WD. METHODS: Retrospective analysis of data from a large cohort of WD, being followed at a tertiary care center. RESULTS: Sixteen patients had conceived on 59 occasions with 30 successful pregnancies, 24 spontaneous abortions, 2 medical terminations of pregnancy and 3 still births. Diagnosis of WD was established after conception in 10 presymptomatic patients while six patients were already on treatment. Among these 16 patients, 9 had history of spontaneous abortions and 12 had successful pregnancies. None of the clinical features of WD changed during pregnancy, with or without treatment. All the 30 babies were full-term and delivered healthy. CONCLUSION: Recurrent abortions are common especially in women with untreated Wilson's disease. However, successful pregnancies and uneventful full-term delivery may occur in mothers of WD on treatment and in undiagnosed, undetected presymptomatic patients. Pregnancy does not seem to have adverse effect on the clinical course of Wilson's disease. Teratogenecity was not seen in the present series with low-dose penicillamine and zinc sulphate.

Abortion, Induced↗
PubMed2004

Tangier disease--a diagnostic challenge in countries endemic for leprosy.

A case of Tangier disease (TD) is reported from India. The patient had presented with indolent mononeuritis multiplex and trophic ulcers of 16 years duration mimicking Hansen's disease. He received antileprosy treatment for one and a half years. Nerve conduction studies revealed features of demyelinating neuropathy. Biopsies of the sural nerve and skin showed striking vacuolation of Schwann cells and myelin sheaths, and foamy vacuolated fibroblasts, respectively, and no evidence of Hansen's disease. Low levels of apolipoprotein A1 (ApoA1) and cholesterol in the serum and undetectable levels of high density lipoprotein (HDL) and low density lipoprotein (LDL) cholesterol in the blood confirmed the diagnosis of TD. This is the first reported case of TD from a tropical country-India. An attempt to establish a correct diagnosis should be made by demonstrating the histopathological and lipoprotein abnormality to avoid long term medications that are chosen empirically and are unnecessary. The importance of recognising this disease in a country where Hansen's disease is highly endemic cannot be overemphasised.

Adult↗
PubMed2004

Wilson's disease: diagnostic errors and clinical implications.

BACKGROUND: Therapeutic outcome of Wilson's disease (WD) significantly depends upon its early recognition. However, because of its rarity in community and protean manifestations, the diagnosis and treatment are often delayed. AIM: To ascertain diagnostic errors at initial evaluation in these patients. METHODS: Analysis of medical records of 307 patients of WD registered over 30 years was done regarding presenting manifestations, initial diagnostic omissions, and interval between onset of symptoms to diagnosis and treatment. RESULTS: Of the 307 patients of WD diagnostic errors by referring doctors from different specialties of health care were detected in 192 patients. These were diverse and multiple and included schizophrenia, juvenile polyarthritis, rheumatic chorea, nephrotic syndrome, metachromatic leucodystrophy, congenital myopathies, subacute sclerosing panencephalitis, neurodegenerative disease among others. The mean (SD) delay was two (three) years (range: 0.08-30 years). Some of the interventions before establishment of correct diagnosis were electroconvulsive therapy, thalamotomy, antipsychotics, and surgical correction for bony deformity. While 98 patients were referred with correct diagnosis, only 16 were given specific treatment. CONCLUSION: Awareness among health professionals about varied presenting features of WD and high index of suspicion may have prognostic implications.

Adult↗
PubMed2004

Cardiac involvement in Wilson's disease--an electrocardiographic observation.

BACKGROUND: Wilson's disease is known for its protean manifestations; however electrocardiographic abnormalities have not received much attention. AIM: To evaluate the various electrocardiographic (ECG) changes in patients with Wilson's disease. METHOD: The resting ECGs of 50 patients with Wilson's disease were systematically analyzed independently by three observers after excluding other causes that could induce ECG abnormalities. RESULT: Fifteen patients had at least one abnormality in the ECG. Sinus tachycardia was seen in eight and sinus bradycardia in six. Other abnormalities included: bifid P wave (1), ST elevation (2), ST depression (2), T inversion (4), ventricular premature contraction - VPC (1) and prominent U waves (1). QRS axis, PR interval, QRS complex, R/S amplitude ratio and QT interval were normal in all. Ventricular premature beats were not recorded in any. None had features of ventricular hypertrophy. There was no statistically significant difference in ECG abnormalities with reference to age, gender, duration of illness or treatment, serum copper or ceruloplasmin, and severity of neurological impairment. CONCLUSION: ECG abnormalities are not uncommon in Wilson's disease and are presumably related to an underlying cardiomyopathy due to deposition of copper in heart.

Adolescent↗
PubMed2003

Penile urethral obstruction in a subject with spinal cord injury.

STUDY DESIGN: Single case report. OBJECTIVE: To report a case of urethral obstruction because of calculus in a subject with spinal cord injury (SCI). SETTING: Bangalore, India. CASE REPORT: A 25-year-old man sustained complete SCI at C(6) level following a road traffic accident. After 14 months, while on self-intermittent catheterization he noticed difficulty in introducing catheter and acute retention of urine. X-ray examination revealed a calculus in the penile urethra at the level of the glans penis. CONCLUSION: Impaction of calculi in penile urethra, although rare, can manifest with acute painless urinary retention in patients with SCI.

Accidents, Traffic↗
PubMed2003

Low prevalence of progressive multifocal leukoencephalopathy in India and Africa: is there a biological explanation?

Infection with human immunodeficiency virus (HIV) clade C virus is the most common form of HIV infection in the world. It largely infects populations in Africa and Asia and not much is known about the neurological complications associated with the virus. Cases of progressive multifocal leukoencephalopathy (PML) have been rarely reported in the literature in the acquired immunodeficiency syndrome (AIDS) or non-AIDS populations from these regions. In this article, the authors present three recently diagnosed patients with AIDS and PML from one neurological center in India, review the diagnostic challenges faced, and speculate on the possible biological reasons, including viral strain differences as well as HIV and JC virus interactions, that may account for the low incidence.

Acquired Immunodeficiency Syndrome↗
PubMed2003

Hemiplegia: an initial manifestation of Japanese encephalitis.

A 7-year-old boy from an area endemic to Japanese encephalitis (JE) manifested with acute febrile illness, left hemiplegia and preserved consciousness during the prodromal phase of illness. The child developed features of encephalitis 48 hours after the onset of hemiplegia. IgM MAC ELISA for JE virus revealed high titers in the serum and cerebrospinal fluid suggestive of JE. MRI of the brain showed asymmetrical bilateral thalamic hyperintense lesions on T2 weighted image, considered diagnostic of JE. Hemiplegia during the prodromal phase or as an initial symptom of JE is rather unusual.

Child↗
PubMed2003

Megalencephalic leukoencephalopathy with subcortical cysts: MRI and proton spectroscopic features.

Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare, recently described disease. It is characterized clinically by a large head, mild neurological symptoms and a remarkably slow course of functional deterioration. The MRI is characterized by 'swollen white matter' and large symmetrical cystic changes in the cerebral hemispheres. MLC should be considered in the differential diagnosis of children with megalencephaly and leukoencephalopathy. We report a child with this disease and discuss the MRI and MRS features.

Basal Ganglia↗
PubMed2002

Pneumatic splints: fabrication and use in neurorehabilitation.

Asymmetrical tone and weakness in antagonistic muscles often result in misalignment of joint, contractures and deformities. Traditional static splints used to prevent these complications are expensive, have to be custom made and cannot be used when deformities are marked. Authors describe fabrication and use of pneumatic splints, which are economic, safe and easy to apply. These splints have wide application in the management of common medical problems in neurological rehabilitation like hypotension, edema, pain, spasticity and early deformities.

Air↗
PubMed2002

MRI in subacute combined degeneration of spinal cord: a case report and review of literature.

A 56 year old man presented with acute onset posterior column and lateral spinothalamic tract dysfunction over a period of 15 days. MRI showed diffuse hyperintensity on T2WI involving the posterior columns. A diagnosis of subacute combined degeneration (SCD) of the spinal cord was considered and confirmed by laboratory findings. The patient showed complete recovery on B12 therapy. MRI lesion also compeletely resolved.

Humans↗
PubMed2001

Afferent pathways of sympathetic skin response in spinal cord: a clinical and electrophysiological study.

BACKGROUND: Sympathetic skin response (SSR) recording is an established test of sudomotor autonomic functions. However, knowledge of its pathways in spinal cord is putative. OBJECTIVE: This study involved subjects with isolated spinal cord lesions to evaluate the afferent pathways of SSR. METHODS: Clinical examination was done according to standard neurological classification of spinal cord injury. Electrophysiological evaluation included: (1) conventional nerve conduction studies to exclude peripheral nerve lesions, (2) scalp somato-sensory-evoked potentials (SEP) with posterior tibial nerve (PTN) stimulation and (3) SSR recording from palm by stimulating supra orbital nerve (SON) at forehead, and PTN at ankle. Subjects with absent SSR in palm to SON stimulation were excluded. In such patients, the afferent tracts were considered abnormal when SSR was absent in palm on stimulation of PTN. RESULTS: Among 37 subjects (age-28.1+/-12.8 years), the afferent tracts of SSR were affected in 13. Sparing of afferent SSR tracts correlated with preservation of bladder sensations (P<0.01). There was no correlation between SSR and SEP. CONCLUSIONS: Spinal cord lesions frequently involve afferent tracts of SSR. Spinal afferents of SSR are closely related with tracts of bladder sensations and are different from pathways for SEP.

Adolescent↗
PubMed2001

Pressure ulcers: an unusual complication of indwelling urethral catheter.

INTRODUCTION: Pressure ulcers are common among patients with spinal cord disorders (SCD) and occur due to unrelieved pressure on soft tissues. CASE REPORTS: Two ladies with paraplegia following acute transverse myelitis developed pressure ulcers over medial aspects of thighs due to indwelling urethral catheter. Absence of sensation, weakness of both legs and lack of knowledge about catheter care contributed to ulcer formation. CONCLUSION: Indwelling urethral catheter may unusually result in pressure ulcers over the thighs in patients with SCD. Among health professionals involved in the care of these subjects awareness is essential for preventing this complication.

Adult↗