Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Atypical skull base osteomyelitis suspected of spreading inflammation from the ear canal with unilateral multiple cranial neuropathy and cerebral infarctions

Yu Hashimoto, M.D.1) and Takahisa Tateishi, M.D., Ph.D.1)

1)Department of Neurology, Japan Community Healthcare Organization Kyushu Hospital

A 76-year-old man, who had undergone surgery for esophageal cancer in 2010, presented to our hospital in April 2017 complaining of prolonged slight fever, loss of appetite, and dysphagia. Initial evaluation revealed a paralyzed left vocal cord, slight muscle weakness of the extremities, left facial paralysis, hoarseness, left sternocleidomastoid and trapezius muscle weakness, tongue deviation to the left, and left hypacusia-suggesting a diagnosis of Garcin's syndrome. Laboratory tests revealed increased white blood cells and C-reactive protein. Cerebrospinal fluid (CSF) analysis showed mild pleocytosis (predominantly polymorphonuclear cells), elevated protein, and low CSF/plasma glucose ratio. CT showed mild clival erosion, with no evidence of carcinoma recurrence. Brain contrast-enhanced MRI showed abnormal clival marrow, enhanced soft tissue and dura matter from the clivus to the atlantoaxial joint, enhanced soft tissue around the left ear canal, multiple cerebral infarctions in the left watershed zones, and left internal carotid stenosis. There was excessive ear wax and inflammation of the left external acoustic meatus but no otorrhea or otalgia. On the basis of his overall presentation, he was diagnosed with atypical skull base osteomyelitis due to external otitis. He was treated with antibiotic treatment that included ceftazidime for the Pseudomonas aeruginosa detected on bacterial cultures. He did not respond to treatment and died approximately 4 months later. Skull base osteomyelitis is thus an important differential diagnosis candidate after finding unilateral, multiple cranial neuropathy, underscoring the importance of prompt treatment when suspected.
Full Text of this Article in Japanese PDF (797K)

(CLINICA NEUROL, 59: 205|210, 2019)
key words: skull base osteomyelitis, malignant external otitis, multiple cranial neuropathy, Garcin's syndrome, multiple cerebral infarctions

(Received: 11-Dec-18)