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Table of Contents    
CASE REPORT
Year : 2020  |  Volume : 68  |  Issue : 3  |  Page : 677-680

Bruns Syndrome – An Unusual Presentation


1 Consultant Neurologist, MD Internal Medicine, DM Neurology, Institute of Neurosciences, West Bengal, India
2 Consultant Critical Care and Stroke Medicine, Institute of Neurosciences, West Bengal, India
3 Consultant Neurosurgeon, MCh Neurosurgery, Institute of Neurosciences, West Bengal, India
4 Consultant Intervention Radiologist, MD Radiodiagnosis, DM Neuroradiology, Institute of Neurosciences, West Bengal, India
5 Consultant Pathologist MDPathology, Institute of Neurosciences, Kolkata, West Bengal, India

Date of Web Publication6-Jul-2020

Correspondence Address:
Dr. Santosh Trivedi
Flat 708, Marvel Homes, Sector 61, Block B17, Noida- 201301, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.289006

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 » Abstract 


Bruns syndrome is characterized by attacks of sudden severe headache, vomiting, and vertigo precipitated due to abrupt movements of the head due to presence of mobile deformable intraventricular lesion causing episodic obstructive hydrocephalus. Proposed underlying mechanism is intermittent or positional CSF obstruction resulting from ball-valve mechanism. Most common etiologies are NCC and intraventricular tumors. Here we present an unusual case of Bruns syndrome that was initially MRI negative.


Keywords: Bruns syndrome, CSF obstruction, fourth ventricle, intraventricular tumors, NCC, obstructive hydrocephalus
Key Messages: Patients of Bruns syndrome in whom no etiology is found initially should be followed up over a period of time with repeat imaging. 3DCISS imaging at the level of fourth ventricle are helpful.


How to cite this article:
Trivedi S, Datta A, Trivedi S, Shobhana A, Pradhan DK, Bhattacharya K, Banerjee M. Bruns Syndrome – An Unusual Presentation. Neurol India 2020;68:677-80

How to cite this URL:
Trivedi S, Datta A, Trivedi S, Shobhana A, Pradhan DK, Bhattacharya K, Banerjee M. Bruns Syndrome – An Unusual Presentation. Neurol India [serial online] 2020 [cited 2020 Aug 13];68:677-80. Available from: http://www.neurologyindia.com/text.asp?2020/68/3/677/289006




Bruns syndrome is characterized by attacks of sudden severe headache, vomiting, and vertigo, triggered by abrupt head movements.[1] It results from presence of mobile deformable intraventricular lesion causing episodic hydrocephalus due to intermittent or positional CSF obstruction with elevation of intracranial pressure due to a ball-valve mechanism.[2] Though both intraventricular tumors [3] and neurocysticercosis (NCC) are known to cause Bruns syndrome, NCC remains the most common cause. Here we present an unusual case of Bruns syndrome which was initially MRI negative.


 » Case Report Top


A 42-year nondiabetic normotensive, hypothyroid female presented in early July 2019 with recurrent episodes of dizzy spells and episodic amnesia. The episode started acutely four days prior to admission when she had one episode of dizziness suddenly along with nausea while getting up from a chair lasting around few minutes. Next day morning again she had a similar shortlasting episode of head reeling on getting up from sleep associated with nausea and vomiting. The episodes were more pronounced on change of head posture on getting up from supine position. Subsequently patient could not recall many episode and events of the day like seeing off her mother, having lunch, etc., till late evening. The next day again she had an episode of transient amnesia and dizzy spells. No history of trauma, travel outside, or systemic illness. Routine physical and neurological examination was normal.

MRI brain showed small acute infarct in left inferior frontal gyrus with normal angiogram of brain and neck vessel [Figure 1], but her MRI finding did not correlate with her amnesia and dizziness. Her 24-hour Holter, ECG, echocardiography also did not reveal any abnormality to suggest a possible vascular origin. However, EEG showed generalized sharp waves suggesting possible epileptic origin of her amnesia [Figure 2]. CSF fluid analysis revealed 95 cells (mostly lymphocytes), protein 95 mg/dl, glucose 65 mg/dl and negative for ZN, fungal and gram stain. ADA was 6 U/L. Possibility of infective vs autoimmune encephalitis etiology was thought of and patient was started on injection acyclovir, injection ceftriaxone, and injection dexamethasone and CSF HSV DNA PCR and autoimmune profile was sent. However, after seven days HSV DNA PCR turned out to be negative and acyclovir was stopped. ANA hep2, ANF profile, cANCA, pANCA sent as a part of her autoimmune workup which could explain her small infarcts and clinical features were also negative. Patient improved with no further episodes of amnesia or dizziness and was discharged.
Figure 1: Imaging done at first admission. T2 weighted axial images at level of lateral ventricles (a) and fourth ventricle (b) show mild ventriculomegaly, without any transependymal seepage. No other lesion was seen, except (c) diffusion weighted imaging showing a focus of diffusion restriction in the left high convexity in parasagittal location

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Figure 2: EEG showing generalized sharp waves

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Four weeks later she came back with transient black outs and fall related to change in posture and new onset global headache for a few days. A repeat MRI brain revealed large intraventricular cystic lesion in the 4th ventricle with possible eccentric scolex and tetra-ventricular hydrocephalus [Figure 3]. Repeat CSF study was performed and revealed 18 cells (mostly lymphocytes), glucose 75 mg/dl, protein 46 mg/dl, negative for all stains and atypical cells, ADA 5 u/l. CSF for cysticercus IgG was sent and was found to be negative. Neurosurgery opinion was taken and she underwent midline suboccipital craniotomy and excision of the cyst. Histopathology report confirmed it to be NCC [Figure 4]. Post-operatively patient was given albendazole under cover of intravenous high-dose steroids followed by switch to oral form. In postoperative follow-up at 2 weeks patient is better with no headache and vertigo.
Figure 3: Repeat MRI Imaging done at 2nd admission. (a) T2 weighted axial image at the level of (a) lateral ventricles shows increase in ventriculomegaly mild transependymal seepage along frontal horns. (b) T2WI at the level of 4th ventricle shows cystic dilatation of the ventricle with transependymal seepage. A suspicious eccentric nodule was noted in along the posterior wall of the ventricle. Hence, it was decided to perform CISS-3D sequence at this level.(c and d) A cyst with well-defined margin (arrowheads) was noted in the 4th ventricle. An eccentric nodule (arrow) was noted in the posterior wall of the cyst, suggestive of scolex

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Figure 4:

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 » Discussion Top


In a study by Torres et al., from a group of 285 patients with neurocysticercosis, only seven clinically presented with Bruns syndrome due to NCC in third ventricle, diagnosed on MRI and underwent endoscopic third ventricular cyst extraction. All seven patients were asymptomatic during follow-up period.[2]

In another case series of 18 patients, all patients with intraventricular NCC presented with hydrocephalus and/or meningitis. The most valuable diagnostic tests were MRI brain and enzyme linked immunotransfer blot (EITB) of serum and/or cerebrospinal fluid. Treatment included albendazole and steroid therapy in all cases, and when necessary urgent or emergency ventriculostomy and/or surgical removal of the obstructing cyst.[4]

Intraventricular NCC in 4th ventricle presenting with hydrocephalus has also been reported in a case series of 13 patients all of which were treated by endoscopic excision.[5]

In our case, initially patient presented with features of classical Bruns syndrome but associated with it was also history of transient amnesia possibly epileptic as evidenced by EEG findings which have not been reported. Another unusual feature was initial MRI negative finding. Most of the reported case series and reviews and reports have confirmed diagnosis of Bruns syndrome due to intraventricular lesion evidenced in MRI brain imaging.

In a review by Campbell et al.,[6] for extraparenchymal lesions MRI is the preferred diagnostic test. On MRI, viable cysticerci are small and round, with thin walls and fluid isodense to CSF. T1 sequences may reveal a dense nodule within the cyst (”hole-in-dot”). Gadolinium-enhanced MRI may aid in detecting ependymitis, a relative contraindication to neuroendoscopy. However, Gd-MRI is not proven to characterize subarachnoid or intraventricular NCC, as these cysts have similar signal intensity to CSF.[7]

Newer MRI acquisition techniques including fast imaging employing steady-state acquisition (FIESTA), three-dimensional constructive interference in steady-state (3D-CISS) have demonstrated the best sensitivity in detecting intraventricular NCC.[8] FIESTA is more sensitive in demonstrating the cysticercus membrane in subarachnoid NCC.

In our patient initial MRI only showed mild ventriculomegaly. In her second presentation, however, MRI T2 weighted sequences showed cystic dilatation of the 4th ventricle with transependymal seepage. CISS-3D imaging showed cyst with well-defined margin and eccentric nodule was noted in the posterior wall of the cyst, suggestive of scolex.

The sensitivity and specificity of EITB are close to 100% for IVNCC.[9] EITB carries a greater sensitivity on serum samples as compared to CSF samples. Therefore, there is essentially no advantage to using CSF for EITB assays. In our patient also CSF EITB was negative.

Removal of the cysticerci by minimally invasive neuroendoscopy over other surgical approaches has been recommended.[10] In our patient, the cyst was removed by suboccipital craniotomy followed by high-dose steroid and albendazole therapy with follow-up improvement in her symptoms.[11]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Bruns L. Neuropathologische demonstrationen. Neurol Centralbl 1902;21:561-7.  Back to cited text no. 1
    
2.
Torres-Corzo J, Rodriguez-della Vecchia R, Rangel-Castilla L. Bruns syndrome caused by intraventricular neurocysticercosis treated using flexible endoscopy. J Neurosurg 2006;104:746-8.  Back to cited text no. 2
    
3.
Krasnianski M, Muller T, Stock K, Zierz S. Bruns syndrome caused by intraventricular tumor. Eur J Med Res 2008;13:179-81.  Back to cited text no. 3
    
4.
Cuetter AC, Andrews RJ. Intraventricular neurocysticercosis: 18 consecutive patients and review of the literature. Neurosurg Focus 2002;12:E5.  Back to cited text no. 4
    
5.
Suri A, Goel RK, Ahmad FU, Vellimana AK, Sharma BS, Mahapatra AK. Transventricular, transaqueductal scope-in-scope endoscopic excision of fourth ventricular neurocysticercosis: A series of 13 cases and a review. J Neurosurg Pediatr2008;1:35-9.  Back to cited text no. 5
    
6.
Campbell BR, Reynoso D, White AC Jr. Intraventricular neurocysticercosis and Bruns' syndrome: A review. J Rare Dis Res Treat 2017;2:1-5.  Back to cited text no. 6
    
7.
Lucato LT, Guedes MS, Sato JR, Bacheschi LA, Machado LR, Leite CC. The role of conventional MR imaging sequences in the evaluation of neurocysticercosis: Impact on characterization of the scolex and lesion burden. Am J Neuroradiol 2007;28:1501-4.  Back to cited text no. 7
    
8.
Mont'Alverne Filho FE, Machado LR, Lucato LT, Leite CC. The role of 3D volumetric MR sequences in diagnosing intraventricular neurocysticercosis: Preliminary results. Arg Neuro Psiquiatr 2001;69:74-8.  Back to cited text no. 8
    
9.
Rodriguez S, Dorny P, Tsang VC, Pretell EJ, Brandt J, Lescano AG, et al.; Cysticercosis Working Group in Peru. Detection of Taenia solium antigens and anti-T. solium antibodies in paired serum and cerebrospinal fluid samples from patients with intraparenchymal or extraparenchymal neurocysticercosis. J Infect Dis 2009;199:1345-52.  Back to cited text no. 9
    
10.
White AC Jr, Coyle CM, Rajshekhar V, Singh G, Hauser WA, Mohanty A, et al. Diagnosis and treatment of neurocysticercosis: 2017 Clinical practice guidelines by the infectious diseases society of America (IDSA) and the American society of tropical medicine and hygiene (ASTMH). Am J Trop Med Hyg 2018;98:945-66.  Back to cited text no. 10
    
11.
Gunawat PV, Patil SS, Karmarkar VS, Deopujari CE. Intraventricular hemorrhage--unusual presentation of central neurocytoma. Neurol India 2014;62:691-3.  Back to cited text no. 11
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