Chronic Myelomonocytic Leukemia (CMML) Presenting with Numb Chin Syndrome and Intractable Headache
Saori Haigo1, Rumyar Ardakani1, Zenggang Pan2, Daniel Pollyea3, Daniel Pastula1, Amanda Piquet1
1Department of Neurology, 2Department of Pathology, 3Department of Medicine, Division of Hematology, University of Colorado Anschutz Medical Campus
Objective:
To report a case of chronic myelomonocytic leukemia (CMML) presenting with numb chin syndrome and intractable headache.
Background:
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.
Design/Methods:
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.
Results:
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.
Conclusions:
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.