Clinical characteristics and outcome of hydrocephalus in neurosarcoidosis: a retrospective cohort study and review of the literature

Hydrocephalus is reported in approximately one-tenth of neurosarcoidosis patients. However, data on clinical characteristics and outcome are lacking. In this retrospective study, we present 11 patients with neurosarcoidosis and hydrocephalus on neuroimaging. Median age was 52 years and seven were fe...

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Vydané v:Journal of neurology Ročník 269; číslo 5; s. 2727 - 2733
Hlavní autori: ten Dam, Leroy, van de Beek, Diederik, Brouwer, Matthijs C.
Médium: Journal Article
Jazyk:English
Vydavateľské údaje: Berlin/Heidelberg Springer Berlin Heidelberg 01.05.2022
Springer Nature B.V
Predmet:
Acuity
Azathioprine
Central Nervous System Diseases - complications
Central Nervous System Diseases - diagnostic imaging
Central Nervous System Diseases - therapy
Cerebrospinal fluid
Cohort analysis
Decompression
Female
Gait
Glucocorticoids
Humans
Hydrocephalus
Hydrocephalus - diagnostic imaging
Hydrocephalus - etiology
Hydrocephalus - surgery
Immunosuppressive agents
Infliximab
Literature reviews
Male
Medicine
Medicine & Public Health
Meta-analysis
Methotrexate
Middle Aged
Monoclonal antibodies
Nervous system diseases
Neuroimaging
Neurology
Neuroradiology
Neurosciences
Neurosurgery
Original Communication
Patients
Remission
Retrospective Studies
Sarcoidosis
Sarcoidosis - complications
Sarcoidosis - diagnostic imaging
Sarcoidosis - therapy
Systematic Reviews as Topic
Treatment Outcome
Tumor necrosis factor-α
Ventricle
Ventricles (cerebral)
Vertigo
Auto-immune disease
Cerebrospinal fluid
Hydrocephalus
Neurosarcoidosis
ISSN:0340-5354, 1432-1459, 1432-1459
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Shrnutí:Hydrocephalus is reported in approximately one-tenth of neurosarcoidosis patients. However, data on clinical characteristics and outcome are lacking. In this retrospective study, we present 11 patients with neurosarcoidosis and hydrocephalus on neuroimaging. Median age was 52 years and seven were female (64%). Presenting symptoms consisted of headache in 8 out of 11 (73%), vertigo in 5 (46%), gait abnormalities in 4 (36%), diplopia in 2 (18%) and decreased visual acuity in 1 (9%). Cranial imaging showed obstructive hydrocephalus in 10 (91%) and non-obstructive hydrocephalus in 1 (9%) out of 11, obstruction occurred at the level of the fourth ventricle in 6 out of 10 (60%). Treatment consisted of glucocorticoids in all the patients with additional methotrexate or azathioprine in 6 (55%) and infliximab in 1 (9%) patient. Neurosurgical intervention was performed in 10 out of 11 (91%) patients. Treatment led to remission, improvement or stabilization of disease in 9 out of 10 (90%) of patients. One patient died due to cerebral herniation despite neurosurgical decompression and CSF shunting. Median modified Rankin scale score at last follow-up was 2 (range 0–6). A systematic review and meta-analysis of studies on hydrocephalus due to neurosarcoidosis identified 36 patients that compared to our patients had a lower median age at onset and a higher mortality. Acute obstructive hydrocephalus due to neurosarcoidosis is a potentially fatal medical emergency requiring neurosurgical intervention and initiation of immunosuppressive therapy. If patients survive the initial phase, the outcome is generally favorable.
Bibliografia:ObjectType-Article-1
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ISSN:0340-5354
1432-1459
1432-1459
DOI:10.1007/s00415-021-10882-2