Multiple cranial-nerve palsies - a diagnostic challenge

Article Abstract:

In the February 15, 1990 issue of 'The New England Journal of Medicine,' Manni et al. describe an apparently novel familial facial palsy, dubbed hyperostosis cranialis interna, which is caused by bone growth narrowing channels in the skull and restricting some cranial nerves. Other, similar, syndromes exist, but hyperostosis cranialis interna is unique in that the bone overgrowth seems to be limited to the skull. Although the diagnosis of palsies of this type may be confirmed by radiology, many cases of facial paralysis are distinctly challenging to the diagnostician. Possible causes include infections, including meningitis caused by bacteria, fungi, and viruses. Lyme disease often causes facial palsies, and herpes zoster virus may cause facial palsies, perhaps without the typical "shingles" rash. Facial palsies may be caused by sarcoidosis, or they may be the result of tumors. The multitude of factors which might contribute to the development of facial palsy is enormous. The evaluation of the patient should begin with computerized tomography (CT) or magnetic resonance imaging (MRI), cerebrospinal fluid examination including cultures and cytologic testing, sedimentation rates, blood counts, and routine blood chemistry. The diagnosis of syndromes with facial palsy does not permit shortcuts. (Consumer Summary produced by Reliance Medical Information, Inc.)

editorial

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Hyperostosis cranialis interna: a new hereditary syndrome with cranial-nerve entrapment

Article Abstract:

A young woman who had experienced occasional episodes of facial paralysis since the age of nine was seen at age 20, after three weeks of recurrent facial-nerve palsy. In addition to the facial palsy, neurologic examination revealed symmetric tearing, a reduced sense of smell, and bilateral taste impairment on the foremost portion of the tongue. When her skull was X-rayed, massive overgrowth of bone was revealed, and the auditory canals, optic foramina, and orbital fissures were severely narrowed. Her jaw and skeleton were otherwise normal. Her family had a history of similar facial palsies; X-rays and CAT scans revealed that the cranial abnormalities were similar also. The family pedigree showed a pattern of inheritance which is consistent with an autosomal dominant gene, but it is not yet possible to rule out the possibility of an X-linked recessive mode of inheritance. Further examination of the patient and relatives ruled out such disorders as craniotubular dysplasias and hyperostoses, sclerostenosis, and endosteal hyperostosis. No previous description matching this disorder has been found in the literature, and the name "hyperostosis cranialis interna" is suggested. (Consumer Summary produced by Reliance Medical Information, Inc.)

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Case 11-2007: A 59-year-old man with neck pain, weakness in the arms and cranial-nerve palsies

Article Abstract:

Treatment of 59 year old man admitted in hospital with symptoms of back and neck pain, cranial nerve palsy and weakness in right arm is studied. Patient was clinically diagnosed with Neuroborreliosis and infectious causes, possible inflammatory disorders causing the symptoms, with imaging tests and pathological study is discussed.

Usage, Case studies, Lyme disease, Diagnosis, Differential, Differential diagnosis, Clinical report

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