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Wildervanck's syndrome presenting as hemiparesthesia
N A Johnson1, M J McClure, E Protzko
1Department of Family and Community Medicine, Dwight D. Eisenhower Army Medical Center, Fort Gordon, GA 30905, USA.
Military Medicine
|April 1, 1995
Summary
Wildervanck's syndrome, a rare condition, typically presents with Duane's retraction syndrome, Klippel-Feil anomaly, and hearing loss. This case uniquely includes hemiparesthesia, a symptom not previously reported in this syndrome.
Area of Science:
- Neurology
- Genetics
- Ophthalmology
Background:
- Wildervanck's syndrome is a rare congenital disorder characterized by the triad of Duane's retraction syndrome, Klippel-Feil anomaly, and sensorineural hearing loss.
- This syndrome results from developmental abnormalities affecting the brainstem, cervical spine, and inner ear.
- While the classic triad is well-documented, atypical presentations are increasingly being reported.
Observation:
- A 41-year-old male presented with acute left hemiparesthesia, a tingling numbness affecting his face, torso, and upper extremity.
- His medical history included lifelong horizontal diplopia, neck stiffness, and recent-onset left-sided hearing loss.
- Physical examination confirmed Duane's retraction syndrome, Klippel-Feil anomaly (congenital C2-C3 fusion), and sensorineural hearing loss.
Findings:
- The patient was diagnosed with Wildervanck's syndrome based on the presence of the classic triad.
- Despite a comprehensive workup, no clear objective cause was identified for the episodic hemiparesthesia.
- This represents the first reported case of hemiparesthesia associated with Wildervanck's syndrome.
Implications:
- This case expands the known clinical spectrum of Wildervanck's syndrome.
- It highlights the importance of considering rare neurological presentations in patients with this syndrome.
- Further research may elucidate the underlying mechanisms connecting hemiparesthesia to the pathophysiology of Wildervanck's syndrome.