Abstract
We report the case of a young girl who suffered bilateral upper extremity paralysis after minor head trauma. Her clinical picture, as well as radiographic evidence of a fracture at C1, led to the diagnosis of Bell's cruciate paralysis, caused by a small lesion in the rostral part of the pyramidal decussation. Magnetic resonance images showed, in addition to a Chiari Type I malformation, an abnormality in the medulla. All previous cases of cruciate paralysis lack both pathological and radiographic supporting evidence.
Abstract
Introduction Chiari type I malformations can present in different ways, but the most frequent symptom is an occipitocervical headache. Hearing loss as the main presenting symptom is rare.
Case A young woman with progressive left-sided unilateral hearing loss was diagnosed with a Chiari type I malformation. She underwent a suboccipital craniectomy with C1 laminectomy and duraplasty. The hearing loss had resolved postoperatively with normalization of the audiometry.
Conclusion Chiari type I malformation can present solely with hearing loss. Improvement after surgical decompression is possible. This phenomenon is not emphasized well enough within the neurologic community. In this report, we present a summary of the pathophysiology and management in Chiari type I malformations.
The association of neurofibromatosis type 1 (NF1) with Chiari malformations of the cerebellum and brain stem has been reported on only two previous occasions.1,2 The pathogenesis of both conditions has remained unclear, although the Chiari type I malformation is most likely due to hypoplasia of the posterior fossa with subsequent extension of the cerebellum through the foramen magnum.3 NF1 is also associated with a variety of cerebral dysplasias.4 We present a patient with both of these dysplastic lesions whose Chiari malformation was asymptomatic.
Prevalence of Chiari type I malformation on cervical magnetic resonance imaging: a retrospective study