Abstract

Abstract Acute Myeloid Leukemia (AML) involvement in central nervous system (CNS) is poorly understood in pathophysiology, with reported incidence rates ranging from 1-33% of patients with relapsed AML. CNS involvement is uncommon in the early stages of AML. FLT3-ITD, chromosome 11 abnormalities, and inv(16) cytogenetics are known to have more potential to invade the CNS. A 28-year-old woman with recently diagnosed AML (FLT D835 mutation with deletion in the long arm of chromosome 11) and migraines was admitted for HIDAC consolidation #3. On day two, she started experiencing headaches in the bifrontal and bitemporal regions associated with photophobia, phonophobia, and nausea. Treatment for status migrainous provided little relief. MRI brain showed progression of Chiari malformation (CM) with tonsillar herniation from 16 to 19 mm, which precluded a lumbar puncture. On day 4, she was noted to have grade III papilledema and bilateral cranial nerve IV palsy with non-enhancing patchy increased FLAIR signal in dorsal pons, bilateral cerebral and middle cerebellar peduncles, without diffuse restriction on imaging. The patient underwent sub-occipital craniotomy and C1 laminectomy for decompression for chiari. On day 5, she developed cranial nerve palsies involving nerves VII, IV, and XII. Lumbar puncture (LP) performed after decompression demonstrated elevated opening pressure and 98% abnormal myeloid blast population, confirming CNS involvement of AML. 4 days before starting intrathecal chemotherapy, the patient was found to be in asystole (unclear etiology). This case highlights the range of differential diagnoses for headaches in a patient with multiple comorbidities, including obesity, migraine, Chiari malformation with tonsillar herniation, and cancer. It underscores the necessity for proactive screening of CNS invasion in AML patients with high-risk mutations and neurological symptoms. Moreover, it emphasizes the need to consider CNS involvement in AML patients with headaches, even if standard imaging does not show typical signs of leptomeningeal spread.

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