Supranuclear Eye Movement Disorders in Fisher's Syndrome of Ophthalmoplegia, Ataxia, and Areflexia

Correlation of 1A afferent conduction with the ataxia of fisher syndrome

“…When abnormal, the electrophysiologic findings are identical to those seen in GBS.'.' 1,15918,20,28 These include sporadic evidence of denervation, abnormalities of motor and sensory conduction (including F responses), or absent soleus H reflexes. These findings suggest that Fisher syndrome and GBS share a common pathophysiology.…”

“…Many of the eye movement abnormalities suggest a brain stem lesion, which might also account for the ataxia. 1,4,7,20 One recent electrophysiologic study has led to the claim that the combination of impaired joint sensation with intact muscle proprioception or the reverse is responsible for the ataxia. *' A strong case for the hypothesis that peripheral nerve lesions alone are responsible for the ataxia should include: (a) normal pathologic studies of the cerebellum and brain stem with demyelination of the peripheral nervous system in a patient with Fisher syndrome; (b) identification, by means of clinical electrophysiologic testing, of an abnormal population of peripheral nerve fibers responsible for the ataxia; and (c) demonstration that the degree of abnormality of this nerve fiber population correlates with the degree of clinical ataxia while all other electrophysiologic parameters remain normal and unchanged.…”

Bickerstaff brainstem encephalitis and Fisher syndrome: anti-GQ1b antibody syndrome

“…Further arguments for a central cause in FS were made in which the eye movements were described in detail in an FS patient attributing this to a supranuclear cause 11. In the editorial of the same issue, Ropper disagreed with the authors' reasoning, suggesting that a peripheral cause could just as easily, if not more likely, give rise to the observations described 12.…”

NOSOLOGICAL ENTITIES?: Bickerstaff's encephalitis and the Miller Fisher syndrome

“…Even the absence of tendon reflexes has been ascribed to interruption of the tegmentum midbrain spinal cord pathways resulting in altered α-motor neuron function 7. Observations on a patient with Miller Fisher syndrome who developed a supranuclear gaze palsy were cited as strong evidence of a central cause for the syndrome 8. In an editorial in the same volume of the Archives of Neurology Ropper9 hotly debated that conclusion, pointing out that some physical signs normally considered to indicate CNS involvement could be mimicked by peripheral nerve disease—for example, preservation of Bell's phenomenon, and supranuclear gaze palsy.…”