A Rare Case of Gliomatosis Cerebri Uncovered After Vertigo: Diagnostic Challenges in Diffuse Gliomas

Introduction

• Gliomatosis cerebri is a rare and aggressive glial tumor characterized by diffuse infiltration of at least three cerebral lobes without forming a well-defined mass.^1,2
• The disease may present in two morphologic forms: a classical type characterized by diffuse infiltration without a discrete mass, and a secondary type exhibiting both diffuse involvement and a focal tumor component.⁶
• The condition poses a diagnostic challenge due to its nonspecific presentation, which may include cognitive or behavioral changes, seizures, and progressive neurological deficits depending on the regions affected.⁷
• Imaging typically reveals widespread T2/FLAIR hyperintensity with minimal enhancement or mass effect, reflecting diffuse tumor infiltration rather than localized expansion.⁴
• The diffuse pattern makes complete surgical resection nearly impossible, leaving radiotherapy and chemotherapy as primary treatments, often with limited effect.^3,5,8
• Prognosis is poor, with 26-52% surviving less than a year from symptom onset.⁷

Case Description

History of Presenting Illness

54-year-old female presented with 3 days of dizziness most consistent with vertigo and intermittent headache. The dizziness was worse with turning her head in certain directions and with laying down which caused her to feel like she was spinning in circles and nauseous.

Past Medical History 

• PMH: Hashimoto's thyroiditis
• PSH: Cesarean Section, hysterectomy with left oophorectomy due to fibroids (2015) and laparoscopic cholecystectomy
• Allergies: NKDA
• Medications: meloxicam 15 MG PO daily, metronidazole 0.75% vaginal gel 37.5 mg into the vagina nightly and synthroid 88 PO daily
• Social History: 4 alcoholic beverages a week (2 glasses of wine, 2 cans of beer per week). No tobacco/illicit drug use.

Physical Exam

• General: A&0x3, comfortable, no acute distress, speech clear and fluent, affect/mood normal
• Heart: RRR, no murmurs/rubs/gallops
• Lungs: Clear to auscultation bilaterally
• Neuro: CN II-XII: PERRLA, EOM intact with some slight nystagmus on R end gaze, visual fields and acuity intact, facial sensation intact, face symmetrical with normal eye closure and smile, hearing normal to rubbing fingers, shoulder shrug intact
  • DH maneuver with L ear down provokes no symptoms or nystagmus. DH maneuver with R ear down provokes intense spinning vertigo and rotatory nystagmus
  • Coordination: FNF, HKS intact
  • Gait: tentative but stable and independent.
• Vitals: BP 139/85 mmHg, HR 90 bpm, T 96.7 °F, RR 17 breaths/min, SpO2 98% on RA, BMI 29.48 kg/m^
• Laboratory Analysis
  • WBC 9.4 x 10³/µL, Hgb 12.9 g/dL, Hct 39.3%, PLT 257 x 10³/µL
  • TSH 2.41 mIU/L, ESR 22 mm/hr, RF <10 IU/mL, C-Reactive
Protein <0.3 mg/dL, ANA positive, SSA <1.0, SSB <1.0
  • CSF Analysis: Culture negative, Total Protein 33 mg/dL, Glucose 61 mg/dL
  • Meningitis/encephalitis PCR panel: Not detected

Differential Diagnosis

• Neoplasm:
  • Glioma
  • Metastatic tumor
  • CNS lymphoma
• Infection:
  • Brain abscess
  • Subacute viral encephalitis
  • Progressive Multifocal Leukoencephalopathy
  • Tuberculosis
• Autoimmune/Inflammatory process
  • Tumefactive multiple sclerosis
  • Acute disseminated encephalomyelitis (ADEM)
• Recent/acute infarct

Hospital Course

• Physical examination revealed a normal physical and neuro exam and Epley maneuver was positive. Despite BPPV diagnosis, further testing was ordered as this was her first vertigo episode.
• CT scan revealed a focal area of edema in the right frontal lobe suspicious for an neoplasm or abscess.
• Pt was admitted and MRI revealed extensive T2/FLAIR hyperintensity in the right frontal lobe, peri-insular regions, corpus callosum, and cingulate gyri.
• Due to MRI findings, further testing was performed. LP was performed to reveal no abnormalities within the CSF fluid. All infectious and inflammatory testing that was drawn resulted with normal findings.
• Brain biopsy was performed and the suspicious area within the right frontal lobe was noted to have extremely friable tissue. Frozen section was sent to pathology showing proliferation of glial cells but with no definitive diagnosis.
• Permanent specimens were sent for further cytology with later resulted as glioma, mild pleomorphism, no mitotic figures or necrosis, IDH wildtype.

Diagnostics

• CT Head: Vasogenic edema right frontal lobe compatible with underlying neoplasm or abscess.
• MRI w/wo contrast: Extensive T2 FLAIR signal hyperintensity within the right frontal lobe, bilateral peri-insular region (right worse than left), along the corpus callosum and cingulate gyri bilaterally.
• Brain Biopsy: Area of lesion noted to have extremely friable tissue. Frozen section sent to pathology showing proliferation of glial cells. Permanent specimens later resulted as glioma, mild pleomorphism, no mitotic figures or necrosis, IDH wildtype.