Clinical and laboratory features and response to treatment in patients presenting with vitamin B12 deficiency-related neurological syndromes.

AIMS AND OBJECTIVES: To study the clinical and laboratory features of patients admitted with vitamin B12 deficiency-related (B12def) neurological syndromes. SETTINGS AND DESIGN: A hospital-based retrospective and prospective study conducted at a referral teaching hospital. MATERIALS AND METHODS: Consecutive patients admitted with vitamin B12 deficiency-related neurological disorders during a three-year period from June 2000 to May 2003 were included. Data regarding clinical and laboratory features were obtained. Follow-up was done at least six months following treatment with parenteral vitamin B12. Chi-square test was used for statistical analysis. RESULTS: A total of 63 patients (52 males) with a mean age of 46.2 years were studied. The mean duration of symptoms at presentation was 10.3 months. Myeloneuropathy (54%) was the commonest neurological manifestation, followed by myeloneuropathy with cognitive dysfunction (34%), and peripheral neuropathy (9%). Neuropsychiatric manifestations and dementia were observed in 38% and 19% of patients respectively. All the patients had megaloblastic changes in the bone marrow smear. Eleven (17.5%) patients had both hemoglobin and the mean corpuscular volume (MCV) within the normal range. Follow-up after at least six months of therapy with parenteral B12 showed improvement in 54% patients. CONCLUSIONS: A high index of suspicion of B12def is required in patients presenting with myelopathy, cognitive decline, or neuropathy. A normal hemoglobin or MCV does not exclude B12def; therefore, other tests such as bone marrow smear and serum vitamin B12 assay are essential, as the condition is often reversible with treatment.

Recent experience with Rett syndrome at a tertiary care center.

Rett syndrome (RS), a neurological developmental disorder, is one of the commonest causes of cognitive impairment in girls and women. These patients are often initially misdiagnosed as idiopathic mental retardation, cerebral palsy, or autism. Despite several reports from the West, there are very few reports from the Indian population. We present four female children with RS and emphasize the importance of early diagnosis.

Autosomal recessive limb girdle myasthenia in two sisters.

Limb girdle myasthenic syndromes are rare genetic disorders described under the broad heterogeneous group known as congenital myasthenic syndromes and present with mixed features of myasthenia and myopathy. The familial limb girdle myasthenia has been described as one with selective weakness of pectoral and pelvic girdles, showing a positive response to edrophonium chloride. A report of two sisters affected by this disorder is presented.

SSPE - The continuing challenge: A study based on serological evidence from a teritary care centre in India.

PURPOSE: To assess the prevalence of subacute sclerosing panencephalitis (SSPE). METHODS: In the period June 96 to December' 98 an analysis for measles virus (MV) antibody was carried out on 103 serum-CSF pairs received from patients clinically suspected of SSPE. Measles antibody was detected in an indirect immunofluorescent assay (IIF) test. RESULTS: Antibody to measles was detectable in 49 (48%) of the serum-CSF pairs tested, a diagnostic criterion for SSPE. Antibody titers ranged from 20 to 1280 in serum and neat to 32 in CSF. The serum: CSF ratio ranged from 5:1 to 80:1. Of the 49 patients diagnosed to have SSPE, 36 were males and 13 females, and the age of the patients at the time of diagnosis of SSPE ranged from 5 to 26 years. Ten of the SSPE patients gave a history of measles vaccination. CONCLUSIONS: Inadequate vaccine coverage and quality of vaccine used continue to have an impact on occurrence of SSPE.

Subacute sclerosing panencephalitis : CT and MR imaging in a rapidly progressive case.

We report the findings on CT and MR imaging in a patient with rapidly progressive subacute sclerosing panencephalitis (SSPE), which correlated with the clinical progression of the disease. In view of the rapid neurological deterioration and CSF pleocytosis, a brain biopsy was done and this confirmed the diagnosis.

Outcomes in the Guillain Barre syndrome: the role of steroids.

Guillain-Barre Syndrome (GBS) is one of the commonest demyelinating diseases of the peripheral nervous system. This retrospective cohort study reports the outcomes of 97 patients in a large teaching hospital in South India. Fifty patients were treated with steroids and 47 had no steroids. Twenty one of the 41 evaluable patients in the steroid group had functional improvement at discharge as compared to 20 of 42 evaluable patients in the non-steroid group. Six patients in each group had worsening of their weakness. Steroids did not show any significant beneficial effect in either improving the disability scores at discharge (steroid-0.42 vs. non-steroid-0.29) or in reducing the duration of ICU stay (4 vs. 8 median days). A higher proportion of patients on steroids developed complications (p = 0.02). The median duration of hospital stay was 16 days in the steroid group as opposed to 14 days in the group not treated with steroids. The mortality was 6 in the steroid treated group and 5 in the non-steroid group. Steroids have no significant benefit on the outcome of GBS.

Delayed effects of exposure to organophosphorus compounds.

In a group of 34 industrial workers, chronically exposed to organophosphorus (OP) compounds, serum pseudocholinesterase activity was depressed significantly in the exposed group as compared to the control group. There was a significantly higher incidence of peripheral neuropathy among the workers exposed to OP compounds, as compared to the control group. Mild to profound sensorineural hearing deficits were detected in both the exposed and control groups. As the pre-exposure hearing status of the workers was not known and since many other factors can also cause pathological changes in the cochlear nerve, a definite conclusion about the ototoxic nature of the OP compounds could not be drawn.

Resolution of cryptococcal meningitis and associated granuloma lung with antifungal therapy: report of a case.

A 49-year-old diabetic male had been unsuccessfully treated with antitubercular therapy for a granulomatous lesion of the left lung detected elsewhere nine months ago. He presented with evidence of meningitis which was found to be due to infection with Cryptococcus neoformans. Both the meningeal and lung lesions resolved after 6 weeks of combined therapy with amphotericin B and flucytosine. The report of the case along with a brief review of the relevant literature is presented.