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Abstract Details

Chronic Myelomonocytic Leukemia (CMML) Presenting with Numb Chin Syndrome and Intractable Headache
Neuro-oncology
P6 - Poster Session 6 (8:00 AM-9:00 AM)
5-029

To report a case of chronic myelomonocytic leukemia (CMML) presenting with numb chin syndrome and intractable headache.

Numb chin syndrome is a rare condition characterized by altered sensation in the distribution of the mandibular branch of the trigeminal nerve. The condition has been associated with different solid organ and hematological malignancies, likely due to leptomeningeal seeding of neoplastic cells. To our knowledge, numb chin syndrome has not been reported with CMML.
We present the case of a 70-year-old male with a history of clonal cytopenia of unknown significance (CCUS) who presented with a six-week history of intractable left temporal headache with associated jaw claudication and bilateral chin numbness. Laboratory evaluation revealed elevated C-reactive protein (203 mg/L) and erythrocyte sedimentation rate (36 mm/hour). Cerebrospinal fluid evaluation demonstrated normal cell count, protein, glucose, cytology, and flow cytometry. Given the patient’s presenting symptoms and elevated inflammatory markers, the patient was treated with steroids for possible giant cell arteritis (GCA) and a diagnostic work-up for malignancy was concurrently pursued.

Computed tomography of the chest, abdomen, and pelvis revealed the presence of diffuse osseous sclerotic lesions of the pelvis and adenopathy of the mediastinum and retroperitoneum. Ultimately, bone marrow biopsy revealed findings consistent with CMML. Biopsy of the left temporal artery demonstrated perivascular and epineural mononuclear cell inflammation with atypical morphological features. Likewise, skin biopsy of the chin revealed cutaneous monocytic infiltrate. The temporal artery and skin biopsies were felt to be consistent with involvement by CMML. The patient was initiated on chemotherapy with decitabine.

Numb chin syndrome may be a presenting feature of CMML and involvement of the temporal artery may mimic features of GCA.  Its presence should warrant investigation for an underlying malignancy.

Authors/Disclosures
Saori L. Haigo, MD, PhD
PRESENTER
Dr. Haigo has nothing to disclose.
Rumyar V. Ardakani, MD Dr. Ardakani has nothing to disclose.
Zenggang Pan No disclosure on file
Daniel Pollyea No disclosure on file
Daniel Pastula, MD, MHS, FAAN (University of Colorado, Department of Neurology) Dr. Pastula has nothing to disclose.
Amanda L. Piquet, MD, FAAN (University of Colorado) The institution of Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Genentech/Roche. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Kyverna . The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech/Roche. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. The institution of Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Sands Anderson PC. Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Joe Jones Law Firm. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Cortez & Associates. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Falk Waas. The institution of Dr. Piquet has received research support from Rocky Mountain MS Center. The institution of Dr. Piquet has received research support from Roche/Genentech. The institution of Dr. Piquet has received research support from NYU. The institution of Dr. Piquet has received research support from Anokion. The institution of Dr. Piquet has received research support from UCB . The institution of Dr. Piquet has received research support from Foundation for Sarcoidosis. The institution of Dr. Piquet has received research support from Kyverna . Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as a Litigative Consultant with US-Dept HHS/DICP. Dr. Piquet has a non-compensated relationship as a Medical Advisory Board Member with Autoimmune Encephalitis Alliance (AEA) that is relevant to AAN interests or activities. Dr. Piquet has a non-compensated relationship as a Medical Advisory Board Member with Stiff Person Syndrome Research Foundation (SPSRF) that is relevant to AAN interests or activities.