Question

Case study

An 8-year-old boy was presented for difficulty in walking and muscle weakness with difficulty in climbing stairs, running and particularly in vigorous physical activities, he also had decreased strength and endurance. His parents did not have consanguineous marriage, one brother and two sisters were all healthy; no other family members were similarly affected. On examination, he had difficulty jumping onto the examination table, a Gower sign, proximal weakness of pectoral and pelvic girdle muscles, a waddling gait tight hell cords, and apparently enlarged calf muscles. His serum creatine kinase and aldolase levels were 50 fold higher than normal, motor nerve conduction studies revealed low amplitude compound muscle action potentials throughout, with normal conduction velocities.

Question: How would you differentiate this disease from Lambert Eaton Syndrome, which also produce muscle weakness?

Answer 1

Answer: Lambert Eaton syndrome is an autoimmune disease. In this disease the immune system itself start engulfing and weakening the muscles, which leads to the body weakness along with the bone. The symptoms includes difficulty in talking an breathing, fatigue and muscles weakness which is followed by dry mouth. In the case of severity respiratory system gets more weak and leads to the breathing problems.

But in the above case the patient is suffering from muscles weakness but it is not Lambert Eaton syndrome, as nerve co-ordination problem along with the high serum etc are the problem which shows over all weakness of the child.