Practice patterns of neurology in India: fewer hands, more work.

BACKGROUND: India is a populous country housing over a billion people. Neurology as a specialty is being practiced in India for over 50 years but the number of physicians devoted to fulltime neurology is limited. This fact coupled with the privatized healthcare system and limited infrastructure has led to situations different from the more developed healthcare systems. AIM: To study the practice patterns of neurology in India. SETTING AND DESIGN: Questionnaire-based study. MATERIALS AND METHODS: Questionnaire was sent to 250 members of the Indian Academy of Neurology [sample size approximately 25%] using random number table. The responses were tabulated and analyzed. RESULTS AND CONCLUSIONS: The neurology group is small and hence is exposed to a large workload. The average number of patients seen daily by Indian neurologists is three to four times those seen by the United States and United Kingdom neurologists. Neurologists based at district places are more likely to see direct patients; whereas metropolitan neurologists see more referrals. Investigative facilities are available to neurologists but affordability is a concern. Clinical work leaves less time for academic and research activities, which the consultants are keen to participate in. In the privatized health system of India, emergency work constitutes a difficult area to cope with. The concept of single specialty group practice is welcomed by the majority with the idea of streamlining their work and life. These factors highlight an urgent need for increasing the neurology work force and argue for further involvement of primary physicians and internists in neurological care in India.

Focal neurological manifestations in Legionellosis.

Legionnaires' disease is an atypical pneumonia with protean multisystem manifestations. Neurological involvement in legionellosis is rare and tends to be among the presenting manifestations. We report a previously healthy young lady who developed focal sensory deficits and cerebellar dysfunction after clinical recovery from Legionella pneumonia. The care is unusual for the delayed appearance of striking focal sensory abnormalities and cerebellar dysfunction.

Myasthenia gravis.

Myasthenia gravis is the prototype neuromuscular disease with immunological pathogenesis. The recognition and interpretation of the symptoms should be stressed as the diagnosis is initially achieved on clinical ground. Tests in the areas of immunology, electrophysiology and imaging further help the diagnosis, management and prognosis of the condition. The recent knowledge of immunology seems to point to variations in the immune abnormalities, but it remains to be seen whether the differences have clinical relevance. With the availability of intensive care units, the management of acute events in the myasthenic patients has improved considerably and the morbidity is reduced. Long term remissions are achievable in majority of patients, with supervised use of immunosuppression. In the modern times, the grave connotations of the name myasthenia gravis may be only rarely justified.

Autoimmune myasthenia gravis in two brothers.

Two brothers with immune-mediated myasthenia gravis are presented for the rarity. The clinical presentation was dissimilar. Both had acetylcholine receptor antibody positivity and one had thymoma. Both responded to immunomodulation and thymectomy. Relevant literature is reviewed.

Idiopathic hypertrophic cervical pachymeningitis: a case report with 5 year follow up.

Idiopathic hypertrophic pachymeningitis is an extremely rare entity. It usually affects cranial meninges. The spinal form is further uncommon and presents as a chronic progressive disease. We describe a 42 year old female with isolated idiopathic hypertrophic cervical pachymeningitis who had a relapsing remitting course under observation for five years. Laminectomy and immunosuppressive therapy produced temporary and partial relief. The long term course and relevant literature is reviewed.

Sarcoglycanopathies: a report of 25 cases.

Twenty five patients with sarcoglycanopathies were studied prospectively. 21 of them had mild phenotype. Muscle involvement was more pronounced in adductor and flexor groups of muscles of the limbs, hip adductor muscles being the weakest. The selective and differential weakness between weak hip adductors and stronger hip abductors resulted in the hip abduction sign in 64% of cases. Distal muscle involvement in lower limbs was seen in 92% of cases, but was mild and late in the course of the disease. 44% patients had winging of scapulae. Immunocytochemistry showed multiple sarcoglycan deficiencies in 84% patients. Primary beta and delta sarcoglycanopathy was seen in the remaining 16% cases. Secondary dystrophin reduction was seen in 44% patients and correlated with beta sarcoglycan deficiency but not with functional disability.

Dissimilar Deletion In DMD Gene Of Two Brothers : A Case Study.

Two brothers who are affected by DMD were studied with respect to CPK assay, muscle biopsy and deletion analysis of their DNA. The severity of phenotype in these two brothers is comparable. Onset of the disease in both the cases was at 4 years of age. CPK values obtained at the age of 10 years for the elder brother and 6years for the younger one were 2550 IU and 1650 IU respectively. Muscle biopsy of the patients indicated dystrophy. PCR analysis of the patient's DNA was carried out for deletion detection in DMD gene. The deletion pattern observed for these two cases was found to be quite different from each other. In case of the elder brother, deletion was found to be expanding exons 8-13 whereas, for the younger brother exons 8-13 as well as exons 43-45 were found to be deleted.

A Report On Higher Frequency Of DMD Gene Deletion In The Indian Subcontinent.

Duchenne Muscular Dystrophy (DMD) gene analysis for 25 unrelated patients from Western India using PCR screening for 14 exons and the promoter region was carried out. Intragenic deletions were detected in 18 patients and most of them were located at the 3' hot spot region of the gene indicating that this part of the gene is more deletion prone in the Indian population from Western India as well. The frequency of deletions observed in the present study is 72%.

Vasculitic neuropathy: profile of twenty patients.

Twenty patients with vasculitic neuropathy were analyzed. Sixteen of the twenty presented with classic mononeuritis multiplex but four had distal, symmetrical, sensorimotor polyneuropathy. Though vasculitic neuropathy is classically associated with Collagen vascular syndromes like, polyarteritis nodosa, rheumatoid arthritis and systemic lupus erythematosis, only 13/20 of our patients had definitive Collagen vascular disease. A large proportion (7/20) had vasculitic neuropathy as the only clinical feature.

Capsular involvement and sensorimotor stroke with posterior cerebral artery territory infarction.

The traditional concept of a clear demarcation between the vascular supply of the internal capsule and the thalamus cannot be regarded as absolute. Two cases of sensorimotor stroke due to posterior cerebral artery territory infarction are described, with CT evidence of internal capsular involvement.