MRI Findings and Evoked Potentials in Patients with Myotonic Dystrophy versus Facioscapulohumeral Dystrophy

Abstract

Italian

Evoked potentials recordings have been applied to many neurological disorders, localizing the lesions in the central nervous system (CNS) pathways. Facioscapulohumeral muscular dystrophy (FSHD) is a slowly progressive degenerative disease involving the muscles of the face and shoulders. On the contrary, myotonic distrophy (MD), the most frequent and severe myotonic disease, is caracterized by myotonia (delay of relaxation after voluntary contraction), muscular atrophy and dystrophic changes in non-muscular tissues.

In the present investigation, patients with clinically and electromyographically verified FSHD and MD were examined using somatosensory evoked potentials (SEP) and brainstem auditory evoked responses (BAEP). The main purpose of the investigation was to determine whether impaired central conduction in patients with MD or FSHD could be associated to specific cerebral abnormalities detected by MRI.

Nine patients with FSHD (7 males, 2 females) 22 to 43 y.o. (mean age 35.11 +/– 9.32 y.o.) and ten patients with MD (8 males, 2 females), aged 24 to 48 (mean age 36.27 +/– 11.80 y.o.), were examined.

Our results suggest that patients with FSHD may present subclinical involvement of the WM on MRI examinations and of the afferent sensory and auditory system at SEP/BAEP analysis irrespective of age, duration or clinical severity of the disease.

Keywords

facioscapulohumeral dystrophy myotonic dystrophy neurophysiology MRI

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