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Localization of the pathological process in Miller Fisher syndrome
F Barontini1, S Di Lollo, S Maurri
1III Clinica Neurologica, Università di Firenze.
Abstract:
A 64 year old woman died at the third attack of MFS. Histological examination demonstrated segmental demyelination and axonal swelling of the peripheral nerves studied, oculomotor included. In the C.N.S. only mild chromatolytic changes and rare pyknosis of the nerve cells in the midbrain were found without signs of primary inflammation. We reviewed the findings in all the 4 anatomoclinical cases of MFS and in 2 cases of GBS with ophthalmoplegia or ataxia. With one exception, they appear to be concordant with those of our case. As the histological examination showed CNS involvement consequent upon peripheral nerve impairment, we are bound to change our opinion on the nosological position of MFS. Any small CT enhancements in the brain in MFS may be due, as in some cases of demyelinating polyneuropathy, to focal rupture of the blood-brain barrier.
Insights
A 64-year-old woman
Area of Science:
- Neurology
- Pathology
Background:
- Miller Fisher Syndrome (MFS) is a rare variant of Guillain-Barré Syndrome (GBS).
- Oculomotor nerve involvement is a key feature of MFS.
Observation:
- Histological examination revealed segmental demyelination and axonal swelling in peripheral nerves, including the oculomotor nerve.
- Central nervous system (CNS) showed mild chromatolytic changes and nerve cell pyknosis in the midbrain, without primary inflammation.
Findings:
- The study reviewed four anatomoclinical MFS cases and two GBS cases with ophthalmoplegia/ataxia, finding concordance with the current case.
- Histological findings suggest CNS involvement secondary to peripheral nerve damage in MFS.
Implications:
- The findings necessitate a reevaluation of the nosological classification of MFS.
- Cerebral CT enhancements in MFS may result from blood-brain barrier disruption, similar to demyelinating polyneuropathies.