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Published on: April 23, 2021
Miller-Fisher syndrome associated with unilateral cerebral white matter lesions
1Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, PR China.
Abstract:
Miller-Fisher syndrome (MFS) is characterized by classical triad of ophthalmoplegia, ataxia and areflexia. The involvement of cerebral white matter in MFS is very rare. We report a typical MFS patient whose brain MRI showed unilateral and extensive involvement in cerebral white matter. We also found mild pleocytosis and raised protein concentration in cerebrospinal fluid. Deficits resolved completely after treatment with intravenous immunoglobulins. Subsequent brain MRI shows cavity formation in involved white matter.
Insights
Miller-Fisher syndrome (MFS), a rare neurological disorder, typically presents with ophthalmoplegia, ataxia, and areflexia. This case highlights MFS with unusual cerebral white matter involvement, successfully treated with immunoglobulins.
Area of Science:
- Neurology
- Neuroimmunology
- Radiology
Background:
- Miller-Fisher syndrome (MFS) is a rare variant of Guillain-Barré syndrome.
- Classical MFS is defined by ophthalmoplegia, ataxia, and areflexia.
- Cerebral white matter involvement in MFS is exceptionally uncommon.
Observation:
- A patient with typical MFS presented with unilateral and extensive cerebral white matter involvement on MRI.
- Cerebrospinal fluid analysis revealed mild pleocytosis and elevated protein levels.
Findings:
- Intravenous immunoglobulin (IVIg) therapy led to complete resolution of neurological deficits.
- Follow-up MRI demonstrated cavity formation in the previously affected white matter regions.
Implications:
- This case expands the understanding of MFS's potential central nervous system manifestations.
- Cerebral white matter involvement in MFS, though rare, should be considered in differential diagnoses.
- MRI findings of white matter lesions and subsequent cavity formation offer insights into MFS pathophysiology.

