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Cushing's syndrome II

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A syndrome of multiple tumours of the spinal nerve roots and auditory nerves.

Description

A syndrome of multiple tumours of the spinal nerve roots and auditory nerves, usually associated with Recklinghausen's disease (neurofibromatosis generalisata), nervous system abnormalities, and occasional multiple gliomas. Onset of acoustic neuroma in majority of cases between third and sixth decades, onset of glioma before age 20. The tumours have the features of meningioma and neurilemmona. The condition is characterized by tinnitus, deafness, ipsilateral paralysis of the 6th and 7th cranial nerves (disturbance or loss of labyrinthine function), hyperaesthesia of the face, headache, vomiting, vertigo, diminished corneal reflex, dimness of vision; and paresis of the facial and external rectus muscles. Bilateral forms are hereditary with autosomal dominant transmission. Prevalent in females (3:1).

The association of multiple tumours of the spinal nerve roots and auditory nerves was first described by the Scottish physician John H. Wishart (1781-1834) in 1822.

Cushing's syndromes
There is disagreement among authors as to what number should be ascribed to the three Cushing syndromes that are usually referred to as Cushing I, II, or III. The numbering used here seems to be the most common.

Bibliography

  • H. W. Cushing:
    Tumors of the nervus acusticus and the syndrome of the cerebello-pontine angle.
    Saunders. Philadelphia, 1917.

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