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[Pachymeningitis with autonomic dysfunctions: a case report].

H Miura¹, M Itoh, H Shimamura

¹Department of 5th Internal Medicine, Tokyo Medical University, 3-20-1 Chuo, Ami, Inashiki, Ibaraki 300-0395, Japan.

No to Shinkei = Brain and Nerve

|October 2, 2001

Summary

This summary is machine-generated.

This study details a patient with cranial nerve palsies and autonomic dysfunction, likely due to inflammation affecting the nervous system. Corticosteroid treatment led to significant clinical improvement, suggesting an inflammatory cause.

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Area of Science:

Neurology
Immunology
Autonomic Nervous System Research

Background:

A 67-year-old man presented with progressive neurological deficits including hearing loss, cranial nerve palsies (IX, X, XII), and severe orthostatic hypotension.
Initial otological treatments were ineffective, necessitating further neurological investigation.

Observation:

Neurological examination revealed cranial nerve palsies and significant orthostatic hypotension.
Cerebrospinal fluid analysis showed pleocytosis and elevated protein, with negative bacterial and tuberculous cultures.
Cranial MRI demonstrated diffuse dural thickening, and autonomic function tests indicated denervation hypersensitivity in cardiovascular and pupillary systems.

Findings:

The patient exhibited post-ganglionic afferent fiber lesions in both cardiovascular and pupillary systems.
Autonomic dysfunctions were characterized by hypersensitivity to noradrenaline and epinephrine.
Corticosteroid therapy resulted in dramatic improvement of neurological and autonomic symptoms.

Implications:

These findings suggest an inflammatory process affecting the autonomic nervous system, potentially involving circulation disorders or inflammatory cell infiltration.
Corticosteroid treatment appears effective for this specific presentation of autonomic dysfunction and cranial nerve palsies.
This case highlights the importance of considering inflammatory etiologies in complex neurological and autonomic presentations.