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Susac syndrome presenting with acute hemibody paraesthesia
Philip Campbell1, Diego Kaski2, Tabish A Saifee2,3
1Department of Clinical and Movement Neuroscience, UCL Queen Square Institute of Neurology, Royal Free Hospital, London, UK.
JRSM Cardiovascular Disease
|May 2, 2019
Summary
Susac syndrome, a rare neurological disorder, presents with varied symptoms, often delaying diagnosis. Early recognition is crucial for timely treatment, especially when stroke-like symptoms appear.
Area of Science:
- Neurology
- Ophthalmology
- Otorhinolaryngology
Background:
- Susac syndrome is a rare autoimmune condition affecting the brain, retina, and inner ear.
- It is characterized by encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss.
- The classic triad of symptoms is infrequently present at onset, leading to diagnostic delays.
Observation:
- A 47-year-old male presented with acute hemisensory loss, an atypical initial symptom.
- The patient was diagnosed with Susac syndrome.
- This case highlights diagnostic challenges in Susac syndrome.
Findings:
- Early diagnosis of Susac syndrome can be difficult due to non-specific or incomplete symptom presentation.
- Hemisensory loss can be an initial presenting symptom of Susac syndrome.
- Misdiagnosis can lead to delayed treatment and potentially worse outcomes.
Implications:
- Increased awareness of Susac syndrome's varied presentations is needed among clinicians.
- Prompt diagnosis and treatment are vital for managing Susac syndrome effectively.
- This case underscores the importance of considering rare diseases in differential diagnoses, especially in acute neurological presentations.
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