Bruns Syndrome – An Unusual Presentation
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.289006
Source of Support: None, Conflict of Interest: None
Keywords: Bruns syndrome, CSF obstruction, fourth ventricle, intraventricular tumors, NCC, obstructive hydrocephalus
Bruns syndrome is characterized by attacks of sudden severe headache, vomiting, and vertigo, triggered by abrupt head movements. 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. Though both intraventricular tumors  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.
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.
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.
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.
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.
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.
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., 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.
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. 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. 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. 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.
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Conflicts of interest
There are no conflicts of interest.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]