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[Miller-Fisher syndrome: electrophysiological serial study of five patients]
J Calleja1, A García, C de Pablos
1Servicio de Neurofisiología Clínica, Hospital Universitario Marqués de Valdecilla, Santander, España.
Objective:
The object of the present study is to show the spectrum of neurophysiological findings during clinical course of Miller Fisher syndrome (MFS), avoiding the controversy over a combined central and peripheral pathology.
Patients And Methods:
We report five patients with a syndrome of ophthalmoplegia, ataxia and areflexia. A similar episode had been suffered previously by 2 of these patients, 14 and 13 years before. In one of them the second episode evolved to a typical Guillain-Barré syndrome (GBS). Motor and sensory conduction velocity of upper and lower limbs, F waves, blink-reflex and somatosensory evoked potentials were recorded in all cases. Needle electromyography in four and brainstem evoked potentials in three of them and the jaw-reflex in another one.
Results:
In all patients there was a markedly reduced amplitude of the distal sensory evoked response, and no signs of denervation in the EMG. The other results were variable both interindividually and intraindividually. The severity of abnormalities was also different between different patients. The clinical recovery was always accompanied by a improvement of neurophysiological parameters.
Conclusions:
The electrophysiological findings in MFS can be variable but they appear always related with the clinical symptomatology. We report for the first time a case in whom the jaw-reflex was abnormal whereas the blink-reflex was normal.
Insights
Neurophysiological findings in Miller Fisher syndrome (MFS) vary but correlate with clinical symptoms. Recovery in MFS patients shows improved neurophysiological parameters, highlighting the diagnostic utility of these tests.
Area of Science:
- Neurology
- Neurophysiology
Context:
- Miller Fisher syndrome (MFS) is a rare variant of Guillain-Barré syndrome.
- Characterized by ophthalmoplegia, ataxia, and areflexia.
- Distinguishing MFS from central nervous system disorders can be challenging.
Purpose:
- To elucidate the spectrum of neurophysiological findings in Miller Fisher syndrome.
- To correlate electrophysiological results with clinical presentation and recovery.
- To avoid controversy regarding central versus peripheral nervous system involvement.
Summary:
- Five MFS patients underwent comprehensive neurophysiological testing, including nerve conduction studies, F-waves, blink reflexes, and evoked potentials.
- All patients exhibited reduced distal sensory evoked response amplitudes; electromyography showed no denervation.
- Neurophysiological abnormalities varied but consistently improved with clinical recovery.
Impact:
- Demonstrates the variability yet clinical relevance of neurophysiological findings in MFS.
- Highlights the utility of electrodiagnostic studies in diagnosing and monitoring MFS.
- Reports the first case of abnormal jaw-reflex with a normal blink reflex in MFS.