Intracranial Neurosarcoidosis

Kumpe, David A.; Rao, Chandrabhan; García, Julio Herrero; Heck, Albert F.

doi:10.1097/00004728-197906000-00004

Cited by 44 publications

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“…Cranial neuropathy is the most frequent manifestation but peripheral neuropathy, myopathy and parenchymatous disease of the CNS may also occur, singly or in combination. Most commonly granulomatous meningitis, either diffusely or as a more circumscribed process at the skull base, causes obstruction of outlet from the fourth ventricle (7)(8)(9)(10). Both communicating and obstructive forms have been described.…”

Section: Discussionmentioning

confidence: 99%

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Sarcoidosis with hydrocephalus: report of a case successfully treated with a ventriculo-peritoneal shunt and methylprednisolone pulse therapy

Lundh

Wikkelsø

1987

Acta Neurologica Scandinavica

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We describe a female patient with systemic sarcoidosis and hydrocephalus. She was initially satisfyingly treated with peroral steroids. When she later deteriorated, the treatment had to be changed due to side-effects. High dose intravenous methylprednisolone pulse therapy was tried with some, but insufficient, improvement. Therefore, a ventriculoperitoneal shunt operation was performed with immediate improvement. After that she was treated with intravenous methylprednisolone and her condition has remained proper and stable for 5 years. The possibility that pulse therapy might have contributed to the favourable course in our patient is discussed.

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Section: Discussionmentioning

confidence: 99%

“…Both communicating and obstructive forms have been described. Most commonly granulomatous meningitis, either diffusely or as a more circumscribed process at the skull base, causes obstruction of outlet from the fourth ventricle (7)(8)(9)(10).…”

Section: Discussionmentioning

confidence: 99%

Sarcoidosis with hydrocephalus: report of a case successfully treated with a ventriculo-peritoneal shunt and methylprednisolone pulse therapy

Lundh

Wikkelsø

1987

Acta Neurologica Scandinavica

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“…Many publications have emphasized the variety of syndromes caused by sarcoidosis of the CNS. The predilection of this granulomatous process for the leptomeninges at the cranial base accounts for the predominance of cases with neuroendocrine dysfunction due to involvement of hypothalamus, pituitary stalk, and hypophysis (Stuart et al, 1978); with cranial nerve palsies (Colover, 1948); with hydrocephalus (Kumpe et al, 1979); and with neuropsychiatric manifestations (Gilmore et al, 1980). A meningoencephalomyelitic process may cause various neurologic deficits and/or elevated intracranial pressure, spinal cord syndromes (Day & Sypert, 1977), spinal nerve and nerve root disturbances, and large, tumor-like intracranial granulomatous masses (Delaney, 1977).…”

Section: Discussionmentioning

confidence: 99%

Pathobiology of Neurosarcoidosis and Clinicopathologic Correlation

Manz

1983

Can. j. neurol. sci.

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SUMMARY:In a retrospective study of 4,294 consecutive postmortem cases, there were 15 with histologically proved sarcoidosis, including 4 with CNS involvement. Whereas in previously reported autopsy series the prevalence of neurosarcoidosis was 15%, it is 27% in this review. Five cases with incidental, clinically inapparent sarcoidosis died at a mean age of 54.6 years; those six with systemic sarcoidosis, not involving the CNS, died at a mean age of 47.0 years; while those four with a variety of neurologic manifestations in addition to systemic sarcoidosis died at a mean age of 38.2 years. Thus, it appears that at one end of the spectrum of morbidity, sarcoidosis may cause no or only trivial symptoms and permit prolonged survival. At the other extreme, sarcoidosis may affect a younger population more severely. CNS involvement occurs relatively early in the course and is rather rapidly progressive, accounting for the poor prognosis, despite appropriate medical and neurosurgical management. In its turn, neurosarcoidosis may present with a variety of signs and symptoms, depending on the site of involvement along the craniospinal axis. This feature is illustrated by the manifestations of compression myelopathy, hydrocephalus with dementia, hydrocephalus with seizures and ataxia, and anosmia, blindness, seizures, and diabetes insipidus. In addition, one patient developed a Nocardia brain abscess as a complication of the altered immune system in sarcoidosis.

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“…Literatur-Nummer 1,6,7,16,17 (2 x), 24,25,37,47,87,89,96,105,106,125 (2 x), 141,142 (2 x), 148, 165 (5 x), 169,171,178,191,196,198,215 7,17 (2 x), 18,24,37 (2 x), 47,68, 70, 71 (2 x), 93,102,125 (2 x), 165 (7 x), 176,188,196,198,200,218,228 10,12,20,22,27,54,66,68,71,96,102,130,137,146,156 (8 x) 72,74,88,89,92,93,98,103,106,116,135,141,156,197,209 (3 x), 214,215,229 (3 x ),236 (2 x) 24,55,71,82,111,124,127,138,150,200,215 3,6 (2 x), 7, 9,10,11,12,13,15,18,20,22,24 (7 x 170,…”

Section: Mende R M a Suchenwirthmentioning

confidence: 99%

Neurosarkoidose

Mende

Suchenwirth²

1990

Fortschr Neurol Psychiatr

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Observation of neurosarcoidosis in a thirty-year-old black female characterised predominantly by bilateral facial nerve paralysis gave rise to a review of literature since 1978 and also to a comparison with an early study in 1963. As described 1963 the clinical picture is characterized by increased protein content of the CSF (33.8%), facial nerve paresis (25.5%), pleocytosis (23%), diabetes insipidus (21%), hemiparesis (17.2%), organic psychosis (16.9%), papilloedema (15.5%), ataxia (13%), convulsive seizures (12.5%), optic atrophy (12.5%), loss of hearing (12.2%), nystagmus (8.6%) and numerous other symptoms more rarely found. This corresponds to the symptoms of chronic basal meningitis with an infiltration in the neighbouring structures of brain and less frequently the spinal cord. In only 58.7% of the cases (presumably at the onset of sarcoidosis) was the bronchial tract (or the lungs) affected, in 11.5% the skin or the eyes. Although the clinical picture is clear enough the etiology has yet to be determined. Evidence of a pathogen or a pathogenic agent (analogous to berylliosis) has never been established to date. On the other hand there are some indications of a disturbance in the immune system, perhaps of a particular genetic foundation since sarcoidosis strikes black patients with conspicuous frequency. There exist more cases in one family. Exceeding expected random distribution, many patients have the HLA-Factor B 8 (on the chromosome 6) and DR 3. The Kveim-Test was in 71 cases positive, in 12 cases negative. The possibilities of carrying out studies of CSF - analogous to the studies of bronchial lavage - in the most cases of neurosarcoidosis have not been exhausted as to determine the activity of the T-lymphocytes, the interleucines, the angiotensin-converting enzyme while the Gallium 67 scintigraphy and other methods to determine the non-specific activity of the inflammation. The efficiency of the treatment with corticosteroids (Prednison or Triamcinolonacetonid) depends of the phase of the inflammatory process. 12% of the registered cases died.

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Intracranial Neurosarcoidosis

Cited by 44 publications

References 0 publications

Sarcoidosis with hydrocephalus: report of a case successfully treated with a ventriculo-peritoneal shunt and methylprednisolone pulse therapy

Sarcoidosis with hydrocephalus: report of a case successfully treated with a ventriculo-peritoneal shunt and methylprednisolone pulse therapy

Pathobiology of Neurosarcoidosis and Clinicopathologic Correlation

Neurosarkoidose

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