Ultrasound and EMG-NCV study (electromyography and nerve conduction velocity) correlation in diagnosis of nerve pathologies.

PURPOSE: Nerve disorders are commonly encountered in clinical practice. Ultrasonography (USG) is a useful modality in the evaluation of most of the peripheral and superficial pathologies amenable to penetration by ultrasound. The primary objective is to study the USG findings of various peripheral nerve pathologies and to correlate them with electrophysiological (EMG-NCV) findings. METHOD: 42 patients referred with suspicion of peripheral nervous system affection were evaluated with USG along with EMG-NCV. After reviewing detailed anatomy of the region, the affected nerve was visualized along the major neurovascular bundle or at a known anatomical landmark with a high-frequency (9-20 MHz) linear/hockey stick transducer. RESULTS: The USG parameters, namely loss of fibrillary pattern, hypoechogenicity and nerve thickening, showed significant p value (p < 0.05) on the tests of significance, suggesting these parameters are significant predictors of nerve affection/pathology on USG. Each ultrasound parameter was correlated individually with SNAP and CMAP. The results revealed positive correlation of echogenicity (r = 0.210, p = 0.05), fibrillary pattern (r = 0.209, p = 0.05) and thickening (r = 0.387, p < 0.05) with sensory nerve action potential (SNAP) and compound muscle action potential (CMAP). CONCLUSION: USG can be used as corroborative investigation to strengthen the findings of EMG-NCV. This combination represents a powerful tool in enabling appropriate planning for treatment, preventing unnecessary intervention and thus improving overall outcomes in patients with peripheral neuropathy.

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.

A study of clinical and laboratory features of 14 Indian patients with dysferlinopathy.

AIM: The aim of the study was to analyze the clinical and laboratory characteristics of Indian patients having dysferlinopathy. METHODS AND MATERIAL: Patients with limb girdle muscular dystrophy (LGMD) were prospectively studied. History, examination, and laboratory evaluation, including creatine kinase, electrophysiology, and muscle biopsy with immunocytochemistry, was carried out. RESULTS AND CONCLUSIONS: Fourteen patients (14.58% of patients with LGMD) had dysferlin deficiency. The mean age at onset was 19.9 years. Nine patients had distal presentation and in the remaining 5 patients, it was proximal. Asymmetry of muscle weakness was seen in 6 patients. Three patients experienced initial calf pains with transient hypertrophy. Gastrocnemius and tibialis anterior muscles were almost similarly affected. The brunt of proximal weakness was on iliopsoas, hip adductors, hamstrings, and quadriceps muscles. Upper limbs were mildly affected. Biceps lump was seen in 4 patients. The phenotype was mild and ambulation was maintained in all patients, many years into the illness.

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.

Hip abduction sign: a new clinical sign in sarcoglycanopathies.

A new clinical sign in sarcoglycanopathies is described. Patients with sarcoglycanopathies showed a phase of abduction of the thighs while rising from the ground: the hip abduction sign. The sign results from selective weakness of hip adductors and comparative preservation of hip abductors. This sign had a sensitivity of 76.2%, specificity of 98%, and was most conspicuous between 3 and 10 years of disease duration. The hip abduction sign is an important clinical indicator of sarcoglycanopathies.