An Atypical Case of High-grade Glioma with Symmetric Cortical and Bilateral Deep Gray Matter Involvement
Jennifer Fokas1, KuangHua Guo1, Eyal Kimchi1, Karan Dixit1, Dan Tong Jia1
1Northwestern University
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 epilepticusand 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.
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