Recurrent Cranial Nerve Palsies Research Articles

An unusual case of hypopituitarism and recurrent cranial nerve palsies

Key words: Intracerebral vasculitis, magnetic resonance angiogram DOI: 10.4038/cmj.v50i2.1578 Ceylon Medical Journal Vol.50(2) 2005 pp86-88

Calvarial doughnut lesions and osteoporosis: A new three‐generation family and review

Familial calvarial doughnut lesions (CDLs; OMIM 126550) is a rare autosomal dominant low bone density disorder characterized by distinctive X-ray translucencies of the skull, multiple fractures, elevated serum alkaline phosphatase, and dental caries. Only three families comprising 22 cases and 29 sporadic cases with the disorder have been reported. We describe a three-generation family consisting of three cases with clinical, radiological, biochemical, and histological findings consistent with this condition. All affected family members presented with childhood onset primary osteoporosis and typical CDLs or hyperostosis of the skull. In addition, the youngest family member was diagnosed with congenital glaucoma and her paternal grandmother with chronic congestive glaucoma. Glaucoma has not been previously described in this disorder. Adult patients also had recurrent cranial nerve palsies. No pathogenic mutations in the genes encoding low density lipoprotein receptor-related protein 5 (LRP5) or type I collagen (COL1A1 or COL1A2) were identified, suggesting that the disorder is caused by another dominant, yet unidentified gene. The literature is reviewed.

Primary Sjögren′s syndrome manifesting as multiple cranial neuropathies: MRI findings

We report a case of primary Sjögren's syndrome presenting with multiple cranial nerve palsies and radiological evidence of cranial pachymeningitis and hypophysitis. A 47-year-old woman developed right sensory neural hearing loss followed, 2 months later, by right facial palsy. Cranial magnetic resonance imaging showed features of pachymeningitis and pituitary gland infiltration. The diagnosis of primary Sjögren's syndrome was confirmed by demonstrating positive SS-A and SS-B antibodies and histological evidence of lymphocytic infiltration of the sublabial salivary gland. During the 2-year follow-up, the patient had transient VIth, IXth, Xth, and XIIth cranial nerve palsies. Sjögren's syndrome should be considered in the differential diagnosis of patients presenting with multiple recurrent cranial nerve palsies, even if prominent sicca symptoms are absent.

Recurrent cranial nerve palsies, midbrain infarction and hydrocephalus due to megadolichobasilar artery

A 67-year-old man presented four recurrent, alternating facial palsies, two right abducens palsies and eventually a right extrinsic third nerve palsy due to brain-stem infarction in a 37-year time-span. Neuroradiological examinations showed hydrocephalus and an elongated, tortuous, ectasic basilar artery. This patient presented in his lifetime the whole clinical spectrum of the dolichoectasic basilar artery complications.

Recurrent cranial nerve palsies of dysglobulinaemic origin

The authors report 5 cases of recurrent cranial nerve palsy in which a dysglobulinaemic condition appeared to be the single aetiological factor. In all the 5 patients dysglobulinaemia manifested itself clinically only by neurological symptoms. In 4 of the 5 cases the protein disorder was represented by cryoglobulinaemia. The physiopathological mechanisms by means of which multiple recurrent cranial nerve palsies may result from dysglobulinaemia are reviewed.

MELKERSSON'S SYNDROME. MULTIPLE RECURRENCE OF BELL'S PALSY AND EPISODIC FACIAL EDEMA.

RECURRENT cranial-nerve palsies have been described in such disorders as diabetes mellitus, migraine, demyelinating diseases, aneurysms and myasthenia gravis. A lesser known entity is recurrent par...