Clinical Characteristics of Essential Tremor in South India: A Hospital-Based Cohort Study.

INTRODUCTION: Essential tremor (ET) is the most common adult movement disorder. Classic ET is characterized by action tremor of hands (95% cases), and tremor involving other regions is less common. Recent studies have revealed a few patients exhibiting nontremor features that include cognitive disorders, tandem gait abnormality, mood fluctuations, olfactory abnormality, hearing impairment, and sleep disorders. Very few studies on ET have so far been conducted in India, and the present study is a pioneering attempt to evaluate the clinical characteristics of patients diagnosed with ET. MATERIALS AND METHODS: A standardized assessment protocol was used to collect data. Diagnosis of ET was established using consensus criteria established by the Movement Disorder Society. Tremor Research Group Essential Tremor Rating Assessment Scale was used to evaluate tremor impact. The severity of hand tremor was assessed by Glass Scale, and cognitive function was assessed by Mini-Mental Status Examination. RESULTS: Out of the 45 patients enrolled, 73.3% were male and 26.6% were female, with a mean age of 44 ± 15 years. Postural tremor was observed in all, followed by intention tremor in 9 and rest tremor in 6 patients. Tremor of the hand was identified to be most predominant (100%). Voice tremor was observed in 15 (33.3%) patients and head tremor in 12 patients (26.6%) who were all females. Leg tremor was observed in 12 patients (26.6% of patients) and tongue tremor in 6 (13%) patients. Baseline asymmetry of tremor was observed in 60% of patients and positive family history in 35% of patients. The most common nontremor feature was tandem gait abnormality (40%). Moreover, most of the patients had Glass Scale II. CONCLUSION: Baseline asymmetry of tremor and male predominance were observed in the study. While hand tremor was the most common form of tremor, tandem gait abnormality was the most common nontremor feature as observed in patients with ET.

Hypocalcemic Recurrent Generalized Seizures with Bilateral Basal Ganglia and Frontal Calcification as the Initial Manifestation of Albright's Hereditary Osteodystrophy in a Child: A Pictorial and Video-graphic Representations.

Albright hereditary osteodystrophy (AHO) is a hereditary metabolic disorder that presents with seizure secondary to hypocalcaemia. A careful phenotypic assessment of patients presenting with seizure clues to the diagnosis of AHO. The characteristic features are short stature,obesity and brachydactyly.Pseudohypoparathyroidism (PHP) is observed in patients with AHO and is characterized by inability of the body to respond appropriately to parathormone, mainly characterized by hypocalcaemia, increased serum parathormone concentration, insensitivity to the biological activity of parathormone, and hyperphosphatemia. In this study, we report a 14-year-old boy with distinctive phenotype of AHO, oral manifestations, and signs of tetany with PHP presenting as recurrent generalized seizure.