Abstract Details

Title An Atypical Case of High-grade Glioma with Symmetric Cortical and Bilateral Deep Gray Matter Involvement

Topic Neurohospitalist

Presentation(s) P7 - Poster Session 7 (5:00 PM-6:00 PM)

Poster/Presentation
Number 3-007

Objective To report an atypical case of rapidly progressive dementia in a patient found to have a high-grade glioma.

Background Midline cortical and deep gray matter structures can be specifically affected by several pathologies. They are highly metabolic regions susceptible to toxic metabolic insults such as B1 deficiency, mitochondrial disorders, and heavy metal poisoning. Infectious causes such as West Nile virus, Powassan virus, or Creutzfeldt Jakob disease also have a predisposition for deep gray matter. Additionally, autoimmune or paraneoplastic encephalitis commonly target these structures. Neoplasms involving bilateral deep gray matter and bilateral thalami, however, are rare and have poor prognosis.

Design/Methods Case report.

Results A healthy 66-year-old presented with 3 months of memory loss. MRI brain demonstrated symmetric bilateral FLAIR hyperintensities of the thalami, paramedian frontal, and mesial temporal lobes without contrast enhancement. CSF was non-inflammatory, including negative RT-QUIC, arbovirus serologies, and autoimmune encephalopathy panel. The patient was empirically treated for autoimmune encephalitis with methylprednisolone and plasma exchange with mild improvement in cognition. While tapering corticosteroids, the patient presented in status epilepticus and new obstructive hydrocephalus requiring extra-ventricular drain placement. Repeat MRI showed new diffusion restriction in the corpus callosum without enhancement, and MR spectroscopy demonstrated a low NAA peak, suggesting neuronal damage. Repeat LP remained non-inflammatory. Given her atypical course, brain biopsy was pursued and revealed IDH-wild type, MGMT-unmethylated high grade glioma.

Conclusions Neurocognitive changes and limbic involvement are common in viral and autoimmune encephalitides but can also be seen in older patients with brain tumors. Development of hydrocephalus raised suspicion for mass effect from a malignancy. Gliomas infiltrating the corpus callosum can have bilateral involvement, however the atypical imaging features in this case include symmetric thalamic and limbic involvement without contrast enhancement. In uncertain cases, brain biopsy is necessary to establish a diagnosis and guide treatment.

Authors/Disclosures
Jennifer A. Fokas, MD (Northwestern Neurology) PRESENTER	Dr. Fokas has nothing to disclose.
KuangHua Guo, MD, PhD	Dr. Guo has nothing to disclose.
Eyal Y. Kimchi, MD, PhD (Northwestern University)	The institution of Dr. Kimchi has received research support from NIH. The institution of an immediate family member of Dr. Kimchi has received research support from NIH.
Karan S. Dixit, MD (Northwestern University)	Dr. Dixit has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Servier.
Dan Tong Jia, MD	Dr. Jia has nothing to disclose.

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