Rare, acquired nerve disease considered to be a
variant of Guillain-Barré syndrome. It is characterized by abnormal
muscle coordination, ophthalmoplegia, and tendon areflexia. As with
Guillain-Barré syndrome, the symptoms may be preceded by a viral
illness. Other clinical features include generalized muscle weakness and
respiratory failure. The prognosis is good, with recovery beginning within 2
to 4 weeks and almost completed within 6 months. Residual neurologic
deficits may occur, and relapses occur in less than 3% of individuals
affected. The majority of individuals with Miller Fisher syndrome have a
unique antibody that characterizes the disorder.
First described in 1956 by Charles Miller Fisher,
a Canadian neurologist.
Rare; 223 cases reported in world literature;
male-to-female ratio is 2:1.
Not genetically inherited. Probably a viral
Precipitating factors of the Miller Fisher syndrome are an upper respiratory
tract infection in the majority of cases but also include infection, surgery,
vaccination, or insect bite. Diplopia and ataxia are the first signs of
development of the syndrome. The exact nature of the clinical entity is
unclear, but the following three interpretations have been suggested: (1) the
Miller Fisher syndrome is a variant of the Guillain-Barré syndrome; (2)
the Miller Fisher syndrome is a brainstem encephalitis without involvement of
peripheral nerves; and (3) areflexia is caused by a lesion of the
mesencephalon and the upper pontine reticular formation.
Ophthalmoplegia, unilateral or bilateral ptosis, and
cerebellar ataxia with lower limb areflexia are required for diagnosis.
Other cranial nerves may be involved in the
process, causing facial palsy, dysarthria, or dysphagia. The course of the
disease has a duration between 3 weeks and 18 months with spontaneous
remission. Rarely is the cerebellar ataxia or areflexia severe enough to
cause major problems; the major impediment to these patients is their
vision. Steroids, plasmapheresis, and immunoglobulin therapy have all been
attempted to reduce the course of this illness.
History and physical examination
should reveal the extent of problems caused by the syndrome. Autonomic
neuropathy and its consequent problems have not been reported in these
patients. Cranial nerve impairment must be carefully documented before
anesthesia. Eye examination by the anesthetist is worthwhile in order to
have a baseline against which to gauge recovery.
No specific measures must be undertaken
in the anesthetic care of these individuals. Patients being treated with
high-dose corticosteroids will require appropriate steroid therapy
No specific indications or
Guillain-Barré Syndrome: Acquired neurologic
disorder preceded by a viral infection and leading to the destruction of
posterior horn of the spinal cord neural tissue. It is clinically manifested
with muscle weakness, areflexia, and numbness or tingling in the arms, legs,
face, and other parts of the body. It may progress to complete paralysis.
Difficulties in breathing and swallowing can occur, and intermittent
positive-pressure ventilation can be required. It can ...