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Brain & Heart Case of rhombencephalitis and hydrocephalus

with sarcoidosis. These manifestations cause vivid or Bickerstaff brainstem encephalitis emerges as another
psychosis, including auditory, visual hallucinations, and possibility, but the pathogenesis of these conditions does
delusions. Consistent with these standard presentations, not align with the disease process of hydrocephalus;
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our patient manifested delusion and aggressive behavior in therefore, these conditions were not considered. After close
the initial presentation, and subsequently delirium. possibilities such as tuberculosis or CNS infections were

The diagnosis and treatment of neurosarcoidosis can ruled out, sarcoidosis was regarded as a plausible diagnosis,
be very challenging for several reasons. It affects clinically on the basis of the multisystemic involvement of the
5 – 10% of sarcoidosis patients, but according to autopsy disease process, and the quick, progressive, and sustainable
examinations, up to 25% of deceased individuals are improvement with steroid. Sarcoidosis is a disease with
affected by sarcoidosis, representing a clear sign of under- an unknown origin, which provides the rationale for the
diagnosis of this pathological condition. Hence, physicians lack of definite evidence in support of diagnosis. Hence,
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need to exercise caution while investigating cases featuring patients with undetermined pathological condition
consistent characteristics with sarcoidosis to avoid mis- featuring attributes as described above should be indicated
diagnosis and the ensuing catastrophic deterioration an aptly-designed treatment regimen, through which
of the condition. To facilitate a definite diagnosis of treatment response can be used to justify the exclusion of
neurosarcoidosis, nervous system biopsy is preferred implausible conditions, leading up to a definite diagnosis.
for investigation, but it is not always practical due to on
the site of disease. Thus, the general diagnostic process 4. Conclusion
for a possible neurosarcoidosis involves confirming a Our study portrays the need for a detailed evaluation of
neuroinflammatory basis of a pathological condition under the etiology of hydrocephalus in a young patient besides
investigation and evaluating the response to treatment performing ventriculoperitoneal shunt for symptomatic
after rigorous exclusion of implausible causes. 5
improvement. We need to conduct a more specific
Primary CNS lymphoma (PCNSL) stands as another and elaborate investigation, driven by a strong clinical
possible diagnosis in this case. It is an uncommon type of suspicion, and rule out rare possibilities: primary CNS
extra-nodal non-Hodgkin lymphoma, which originates lymphoma and sarcoidosis are one of them. This approach
in a type of cell not normally present in the CNS. PCNSL will help us start proper treatment regimen as early as
is known to regress completely with corticosteroids but possible and save valuable time as “time is brain”.
may recur later and cause fatal outcomes, as described
in the current case. Brain parenchyma, spinal cord, Acknowledgments
leptomeninges, and eyes are affected in PCNSL, giving
rise to highly variable presentations and even psychiatric None.
symptoms (also described in the current case) in up Funding
to 43% of cases. In fact, hydrocephalus may be the sole
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manifestation of PCNSL. CSF cytology approach, which None.
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was employed in this case, is not very sensitive in detecting
PCNSL (with a rate of 2–32%), and flow cytometry has Conflict of interest
been reported to have higher sensitivity. To enhance The author declares no competing interest.
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sensitivity (up to 85%) in this respect, several CSF
markers, such as CSF lactate dehydrogenase isozyme 5 Author contributions
and β2-microglobulin, can be assayed. Few other markers This is single-authored article.
may be utilized to increase specificity (up to 95%) like
proteomics and microRNA analysis. However, brain/ Ethics approval and consent to participate
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leptomeningeal/vitreous biopsy remains the current gold-
standard diagnostic approach for PCNSL. On a separate The patient gave consent to participate in the study.
note, CNS infections represent another etiological facet of
PCNSL that should not be neglected, as evidenced by the Consent for publication
increased uptake of thallium 201 in single-photon emission Patient gave consent to release their data and images in this
CT scan that is strongly indicative of the disorder. 7 paper.
In this case, a definite diagnosis of the disease cannot be Availability of data
reached due to the suspended investigative works caused
by patient’s loss to follow-up. Autoimmune encephalitis Not applicable.

Volume 2 Issue 2 (2024) 4 doi: 10.36922/bh.2133