Four cases of Kennedy's disease in three families earlier diagnosed as amyotrophic lateral sclerosis

ABSTRACT

Kennedys disease (KD) is a rare, slowly progressive neurodegenerative disorder of motor neurons in the spinal cord and brain stem. Most of the cases of KD in clinical practice are misdiagnosed. The knowledge of the initial presentation, the range of age within which the disease would manifest and the clinical course of the disease would be very helpful to better manage and anticipate the outcome of such cases. This report highlights the typical earliest presentation of KD and the clearcut clinical picture of KD that differentiates it from other motor neuron diseases of grave scenario and prognosis We report clinical details of 4 male patients with KD seen in our center. Diagnosis of these four patients were based on their clinical picture the time they were first seen. Common features in their history and presentation were the onset of prolonged and intermittent muscle cramps followed by weakness and atrophy of the muscles involved. All of them developed gynecomastia. Three of them have concomitant diabetes, and one has thyroid problem. All of them were initially diagnosed as Amyotrophic lateral Sclerosis.