[Quantitative evaluation of the effect of 3,4-diaminopyridine in a patient with Lambert-Eaton myasthenic syndrome...

We reported a 72-year-old woman with Lambert-Eaton myasthenic syndrome. The chief complaint was weakness and atrophy of the thigh muscles, which prevented her from climbing stairs even with a handrail. Sensory and autonomic function was normal without amblygeustia. There was no malignancy found, and her serum anti-V/Q type voltage-gated calcium channel antibody was negative. Administration of 3,4-diaminopyridine (DAP), known to accelerate acetylcholine release, was very effective and she became able to climb stairs without a handrail. For evaluation of the therapeutic effect of DAP, the initial compound muscle action potential (ICMAP) on evoked electromyogram has been recommended because it provides highly sensitive and reproducible results. Unfortunately this method is usually applied to several particular distal muscles for technical reasons. In the present case, evaluation of the quadriceps femoris muscle was most important because it was most responsible for her disability. We attempted to measure the angular velocity and the angular acceleration on knee extension movement using dynamic dynamometry. The angular velocity improved from 124 to 162 deg/sec and the angular acceleration from 220 to 390 deg/sec2. The results were more sensitive and more relevant to her demonstrable ADL improvement than grasping power increase and ICMAP improvement recorded at the distal muscles.