[Pachymeningitis with autonomic dysfunctions: a case report]

A 67-year-old man temporarily complained of hearing loss and earache in the left side. Afterwards, hearing impairment in the right, pharyngalgia, dysphagia, and ipsilateral facial weakness occurred, however, otological treatments did not completely improve these symptoms except facial weakness. On admission neurological examination revealed right cranial nerve palsies(IX, X, XII), and severe orthostatic hypotension was noted one month after admission. Cerebrospinal fluid revealed pleocytosis(16/mm3), and increased protein level(91 mg/dl), but bacterial, and tuberculotic cultures were negative. Cranial and neck magnetic resonance imaging with gadolinium administration showed diffuse dural thickening. Autonomic dysfunctions were found in cardiovascular and pupillary systems. Plasma noradrenaline level was normal in supine position but noradrenaline infusion test showed denervation hypersensitivity. Additionally pupils showed hypersensitivity to 1.25% epinephrine. These results suggest that lesions were post-ganglionic afferent fibers in both cardiovascular and pupillary systems. Administration of corticosteroid hormone resulted in dramatic improvement of his clinical symptoms including autonomic dysfunctions. Circulation disorder or infiltration of the inflammatory cells to the autonomic nervous system may be the cause of these dysfunctions in this patient.