Long term follow up of a hemimasticatory spasm

To describe the clinical and neurophysiological findings in a case of hemimasticatory spasm (HMS) followed during 14 years of evolution.

A woman suffered from very frequent paroxysmal episodes of painful involuntary occlusion of the jaw. Neurophysiological studies were performed at the 3, 12 and 14 years after the onset of symptoms. They included a needle electromyographic (EMG) evaluation of the main jaw closing and opening muscles, the jaw reflex (JR), the masseteric silent period (MSP) and the masseteric inhibitory reflex (MIR).

Clinical symptoms remained unchanged throughout the period of observation. Conventional EMG never disclosed neurogenic signs. Voluntary closure of the jaw systematically provoked an abnormal activity with muscle cramps characteristics, restricted to the left masseter muscle. Left JR response was normal in the first evaluation and became delayed and of reduced amplitude in the second. The MSP and MIR were abolished on the left side during the spasmodic episodes whereas they were strictly normal out of them. The MIR abnormalities showed the characteristic pattern of an efferent lesional type.

Hemimasticatory spasm probably is the consequence of an abnormal trigeminal hyperexcitability likely induced by the demyelinating lesion of its peripheral motor pathway. The main neurophysiological abnormalities may persist unmodified over a long course of the disease and allow the differential diagnosis of HMS from oromandibular dystonia and temporomandibular dysfunction (TMD).