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Rapidly Progressive Quadriplegia and Encephalopathy.
DonRaphael Wynn1, Donald McCorquodale1, Angela Peters1
1Department of Neurology, University of Utah, Salt Lake City.
A rare neurological disorder caused rapid motor weakness and encephalopathy in a 77-year-old woman. Despite extensive testing, the condition progressed, leading to death, highlighting diagnostic challenges.
Area of Science:
- Neurocritical Care
- Neurology
- Pathology
Background:
- A 77-year-old woman presented with rapidly progressive motor weakness and encephalopathy.
- Clinical examination revealed altered consciousness, myoclonus, and orofacial dyskinesias.
Observation:
- Initial imaging was unremarkable; repeat studies showed cauda equina, trigeminal nerve, and pachymeningeal enhancement.
- Cerebrospinal fluid analysis indicated mild pleocytosis and elevated protein.
- Electrodiagnostic testing revealed a rapidly progressive sensory motor axonopathy.
Findings:
- Electroencephalogram showed progression from generalized slowing to periodic lateralized epileptiform discharges.
- A diagnostic biopsy was performed due to critical historical details.
- The patient's condition deteriorated, becoming unresponsive and ultimately fatal.
Implications:
- This case underscores the diagnostic complexity of rare neurological conditions presenting with rapid deterioration.
- It highlights the importance of serial investigations and diagnostic biopsies in neurocritical care.
- The case facilitates discussion on differential diagnoses and pathological findings of a rare neurological entity.
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