Contributed by Osorio Lopes Abath Neto, MD, PhD, and Scott Kulich, MD, PhD
Contributed by Osorio Lopes Abath Neto, MD, PhD, and Scott Kulich, MD, PhD
CLINICAL HISTORY
A woman in her 60's, with a past medical history of non-alcoholic cirrhosis, obesity, hypertension, hyperlipidemia, thyroid carcinoma, and poorly controlled type 2 diabetes mellitus presented to the emergency room with headache, nausea, and vomiting. A head computed tomography scan and laboratory studies were normal, and the patient was discharged with migraine medications. The patient returned to the emergency department 4 days following her initial discharge due to multiple falls associated with episodes of loss of consciousness triggered by supination, as well as hallucinations. On examination a hematoma involving her forehead, multiple rib fractures, a right knee laceration and a right patellar fracture we noted, and an EKG showed first degree AV block. Additional workup included blood work which revealed leukocytosis, which prompted blood cultures, in addition to elevated ammonia levels. Due to the patient's altered mental status and intermittent loss of consciousness, an EEG was performed and read as negative. A psychiatry consultation suggested delirium, the cause of which remained unclear. A MRI of the head was ordered but the patient refused. On the morning of the third day after admission, the patient had a cardiac arrest and resuscitation efforts were unsuccessful. An autopsy was requested in an attempt to identify the underlying cause of death.
GROSS AND MICROSCOPIC FINDINGS
The brain was grossly unremarkable except for mildly hazy leptomeninges throughout the outer surface of the cerebrum and cerebellum and mild atherosclerosis in the circle of Willis.
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Figure 1. Mild haziness in the leptomeninges, more pronounced over the convexities of the frontal and parietal lobes. Leptomeninges are artifactually avulsed overlying the medial aspects.
H&E stained sections demonstrated a generalized thickening of the leptomeninges with a mild to focally moderate increase in cellularity. The increased cellularity was composed of mononuclear inflammatory cells admixed with smaller variably sized (roughly 5 to 15 microns) refractile, round to oval blue structures that extended deep into sulci and expanded Virchow-Robin spaces. Additional studies demonstrated that these structures were highlighted by PAS, methenamine silver and mucicarmine stains. Of note, similar structures were also identified within the airspaces of the lungs.
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Figure 2. Leptomeninges diffusely infiltrated by round to oval structures. A: cerebellar section, 4X; B: mid-frontal section, 4X; C: mid-frontal section, 20X.
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Figure 3. Leptomeningeal infiltration in a mid-frontal section. A: H&E, 20X; B: Grocott methenamine silver, 20X; C: PAS, 20X; D: mucicarmine, 20X.
