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Clinical and Electrodiagnostic Findings

James W. Albers, MD, PhD; Simon Zimnowodzki, MD; Claude M. Lowrey, MD; Barry Miller, MD

Arch Neurol. 1983;40(6):351-353.

Abstract
• Differentiation of juvenile progressive bulbar palsy from bulbar myasthenia gravis is difficult. Characteristics of both may include ocular involvement, fluctuant course, abnormal fatigability, and normal acetylcholine receptor (AChR) antibody titers. Electrodiagnostic evaluation may demonstrate moment-to-moment variability in motor unit action potential amplitude, fibrillation potentials, and decremental motor evoked responses. Increased jitter with blocking may be the most prominent electrodiagnostic abnormality in either disorder, even in asymptomatic extremity muscles. Complete paralysis of facial muscles with electrical silence on needle electromyography, low-amplitude facial evoked responses without a decrement to repetitive stimulation, increased jitter and fiber density in asymptomatic extremity muscles, and normal AChR antibody levels suggested juvenile progressive bulbar palsy in two patients initially thought to have bulbar myasthenia. Early differentiation of these disorders is important because of therapeutic, genetic, and prognostic implications.

Author Affiliations
From the Departments of Neurology (Drs Albers and Zimnowodzki) and Physical Medicine and Rehabilitation (Dr Albers), University of Michigan Medical Center, Ann Arbor; and the Departments of Neurology (Dr Lowrey) and Physical Medicine (Dr Miller), St Joseph Mercy Hospital, Ann Arbor.

Footnotes
Accepted for publication Oct 15, 1982.

Read before the Annual Meeting of the American Association of Electromyography and Electrodiagnosis, St Paul, Oct 8, 1982.

Reprint requests to Department of Neurology, University of Michigan Medical Center, B4952, Clinical Faculty Office Bldg, Ann Arbor, MI 48109 (Dr Albers).

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