Page 84 - BH-2-2

Brain & Heart Case of rhombencephalitis and hydrocephalus

treatment to rule out certain possible etiologies and to (neuromyelitis optica) and anti-myelin oligodendrocyte
save him from plunging into worse deterioration. He was glycoprotein antibodies; negative results from human
eventually diagnosed with “possible neurosarcoidosis.” immunodeficiency virus serology, venereal disease
research laboratory test and TB GeneXpert; and non-
2. Case presentation contributory findings from CSF oligo clonal band, central
A 49-year-old man, suffering from diabetes, hypertension, nervous system (CNS) autoimmune, and paraneoplastic
and moderate obstructive sleep apnea, was diagnosed with encephalitis panel tests.
normal pressure hydrocephalus based on the presentation Given that he acquired pachymeningitis and
of the classic triad of cognitive decline, imbalance, rhombencephalitis with recent detection of hydrocephalus
and incontinence lasting for over 6 months. Fundus while presented with hypercalcemia, the physicians
examination and routine cerebrospinal fluid (CSF) study considered neurosarcoidosis a plausible diagnosis, after
revealed normal results. He showed improvement in ruling out active infection as the principal cause of his
the “Timed Up and Go Test” post-therapeutic drainage. condition. To validate the diagnosis, a brain biopsy was
Magnetic resonance imaging (MRI) of his brain revealed recommended but declined by the patient and his family. As
features of hydrocephalus with periventricular oozing. an alternative, a positron emission tomography-computed
Hence, he was indicated a ventricular-peritoneal shunt, tomography scan of the whole body was performed,
which led to improved balance to some extent and yielding negative findings that rule out the involvement of
regaining of his ability to walk independently. a systemic factor. Evidence of peripheral nodule was not
One month after the treatment, he presented to us available since biopsy was not conducted.
with exacerbated acute-onset imbalance and speech The patient also suffered from a recent proteinuria,
impairment. He declared no history of fever or trauma which was unveiled as a part of the evaluation of
from the treatment leading up to the most recent medical raised creatinine level (very high 24-h urinary
consultation. The patient had stable vitals but was grappling protein: 2106.5 mg/day). Hence, a renal biopsy was
with confusion and dysarthria. He had quadriparesis (MRC recommended but again declined by his family.
grading was 3/5 of all four limbs) and ataxia (both axial and While experiencing gradual deterioration, the patient
appendicular) but showed no signs of meningitis. Routine was given methylprednisolone (1 g for 5 days) followed
blood investigations include assessment of biochemical by oral prednisolone (60 mg orally) and mycophenolate
parameters, unveiled hypercalcemia (calcium level of mofetil (500 mg orally) twice a day, after active infection was
11.8 mg/dL), high creatinine level of 1.4 mg/dL, and high ruled out as the etiology. His response to the medications
erythrocyte sedimentation rate of 68 mm/h. Brain MRI was remarkable, leading to a resolution of encephalopathy,
revealed T2 hyperintensities in the thalamus, midbrain, evidenced by a radiological resolution after a 5-day course
pons, and medulla, and diffusion-weighted imaging showed of methylprednisolone (Figure 2). However, he developed
facilitated diffusion, with post-contrast enhancement of psychosis afterward, which was initially perceived to have
the lesion in FLAIR along with pachymeningitis (Figure 1). been caused by steroid. A detailed psychiatric evaluation
The MRI of the spine did not yield any significant, relevant was conducted on the patient based on the information;
findings. Based on these findings, a preliminary diagnosis we could glean from his wife, who recounted that the
of rhombencephalitis was made, and the exact etiology was patient had been experiencing a delusion of persecution
investigated afterward. over the previous 1 year and used to have violent
A new session of CSF study revealed only increased behavior. A follow-up examination 7 days after the clinical
protein level (122 mg/dL), providing no evidence of an consultation revealed that his cognitive function, ataxia,
active infection, based on a polymerase chain reaction and quadriparesis all improved (MRC power improved to
analysis for Listeria and the comprehensive infective panel. 4+/5 in all four limbs) and he regained the ability to walk
There was no history or findings suggestive of vasculitis, independently albeit with mild ataxia. His psychiatric
Bechet’s disease, or histiocytosis. Radiologic findings symptoms also alleviated to a considerable extent over time.
from repeat brain MRI did not consistently align with After 4 months, the patient was readmitted with a
features of either neuromyelitis optica spectrum disorder lower respiratory tract infection and sepsis. Treatment
or other demyelinating disorders. In addition, an armada comprising steroid and mycophenolate mofetil was
of investigations performed did not seem contribute discontinued by the attending physicians. His condition
to the indicative findings that could lead to a definite improved following the administration of anti-infection
diagnosis: negative vasculitis markers; normal serum treatment but he became subjected to rapid deterioration
level angiotensin-converting enzyme; negative anti-NMO afterward, marked by declining senses and unstable vital

Volume 2 Issue 2 (2024) 2 doi: 10.36922/bh.2133