Neurosarcoidosis Presenting with Obstructive Hydrocephalus Successfully Treated with Endoscopic Third Ventriculostomy

Nakayasu, Hiromasa; Shirai, Toshihiro; Tanaka, Yūko; Sato, Tsukasa; Muro, Hiroyuki

doi:10.2169/internalmedicine.1259-18

Cited by 7 publications

(5 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This is supported by the outcome seen in case 1. One report described a patient recently diagnosed with pulmonary sarcoidosis who was found to have a contrast enhancing lesion in the cerebral aqueduct causing non-communicating hydrocephalus 5. This patient underwent endoscopic third ventriculostomy and NS was confirmed histologically.…”

Section: Discussionmentioning

confidence: 99%

Hydrocephalus as the first presenting symptom of neurosarcoidosis in two patients: a diagnosis more forthcoming in the context of systemic disease

McKeever¹,

Cox

Garnett

et al. 2019

BMJ Case Rep

View full text Add to dashboard Cite

Sarcoidosis is a multisystem disorder, characterised histologically by the presence of non-caseating epithelioid granulomas with exclusion of other granulomatous diseases. While the lungs and lymph nodes are affected in 90%, approximately 5% of patients have neurological involvement. The clinical manifestations of neurosarcoidosis (NS) are diverse, making diagnosis especially difficult in patients without known systemic disease. Hydrocephalus occurs in only 9% of patients with NS and although uncommon, is an important manifestation because it is associated with high morbidity and mortality. We report two cases of NS presenting with hydrocephalus, one as the first presentation of sarcoidosis and one in a patient with known multisystem sarcoidosis. The patient without systemic sarcoidosis posed the greater diagnostic challenge and followed a protracted course with multiple surgical interventions, progression of central nervous system inflammation and significant physical disability.

show abstract

Section: Discussionmentioning

confidence: 99%

Hydrocephalus as the first presenting symptom of neurosarcoidosis in two patients: a diagnosis more forthcoming in the context of systemic disease

McKeever¹,

Cox

Garnett

et al. 2019

BMJ Case Rep

View full text Add to dashboard Cite

show abstract

“…8 We identified four English case reports of neurosarcoidosis diagnosed by endoscopic biopsy of brain lesions. [4][5][6][7] In contrast to those cases, our patient exhibited local enhancing effects. Interestingly, endoscopy revealed numerous nodular lesions in the ventricles, which were not detected on contrast-enhanced MRI.…”

Section: Discussionmentioning

confidence: 57%

“…Contrast‐enhanced MRI can indicate active parenchymal or meningeal granulomas optimal for biopsy . We identified four English case reports of neurosarcoidosis diagnosed by endoscopic biopsy of brain lesions . In contrast to those cases, our patient exhibited local enhancing effects.…”

Section: Discussionmentioning

confidence: 79%

“…In neurosarcoidosis, hydrocephalus can occur in two forms: (a) obstruction of the CSF outflow tract by lesions (i.e., obstructive) and (b) involvement of tissues responsible for CSF absorption (i.e., communicating) . A few studies have reported successful treatment by ventricular shunting combined with immunotherapy .…”

Section: Discussionmentioning

confidence: 99%

“…7 A few studies have reported successful treatment by ventricular shunting combined with immunotherapy. [4][5][6][7] In our case, communicating hydrocephalus is suspected as the mechanism because obstructive lesions were not observed both on MRI and on endoscopy. Furthermore, her poor responsiveness to ventriculostomy is supportive for the diagnosis of communicating hydrocephalus.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Communicating hydrocephalus associated with neurosarcoidosis diagnosed by endoscopic biopsy

Maruno

Naito

Nakamoto

et al. 2019

Neurology & Clinical Neurosc

View full text Add to dashboard Cite

We present a case of communicating hydrocephalus associated with neurosarcoidosis in which the patient was diagnosed by endoscopic biopsy and successfully treated with corticosteroid therapy. The patient was a 53‐year‐old woman who presented with vertigo and nausea. Brain MRI revealed hydrocephalus and contrast‐enhancing areas only on the interthalamic adhesion. Sarcoidosis was suspected based on hilar lymphadenopathy and her past history of uveitis; however, there was no other organ involvement appropriate for biopsy. We performed endoscopic third ventriculocisternostomy and performed biopsy of nodular lesions of the interthalamic adhesion. Interestingly, endoscopy revealed numerous lesions on ventricular walls, which were not detected on contrast‐enhanced MRI. The histopathological examination revealed findings consistent with sarcoidosis. The patient was started on corticosteroid therapy, and her hydrocephalus improved conspicuously. Our case reinforces the notion that endoscopic biopsy is helpful in the definite diagnosis of neurosarcoidosis.

show abstract

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

2021

View full text Add to dashboard Cite

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.

show abstract

Neurosarcoidosis Presenting with Obstructive Hydrocephalus Successfully Treated with Endoscopic Third Ventriculostomy

Cited by 7 publications

References 10 publications

Hydrocephalus as the first presenting symptom of neurosarcoidosis in two patients: a diagnosis more forthcoming in the context of systemic disease

Hydrocephalus as the first presenting symptom of neurosarcoidosis in two patients: a diagnosis more forthcoming in the context of systemic disease

Communicating hydrocephalus associated with neurosarcoidosis diagnosed by endoscopic biopsy

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

Contact Info

Product

Resources

About