Objective:

Explore diagnostic clues in a case of secondary headache mimicking trigeminal autonomic cephalalgia, ultimately diagnosed as secondary to B-cell lymphoproliferative disease. 

Background:

Trigeminal autonomic cephalalgias (TACs) are characterized by unilateral, severe headaches with ipsilateral cranial autonomic features. Though most are primary, secondary causes must be considered in patients with atypical features, abnormal exams, or poor response to therapies. 

Design/Methods:

NA

Results:

A 47-year-old previously healthy male developed new-onset headaches and upper respiratory symptoms after a week of travel to New Mexico, where he spent time in straw bale homes and participated in banjo camp. One week after return, he presented to an emergency department for headaches. Head CT/CTA noted mildly prominent nonspecific lymph nodes. One month into symptoms, he presented outpatient to neurologist describing severe, non-positional, stabbing, left-sided headache with conjunctival injection, congestion, and lacrimation lasting hours with minimal relief between episodes. TAC was considered, however both indomethacin and sumatriptan were ineffective. Notably, serial courses of prednisone provided excellent transient relief. Two months into his course, headaches worsened in duration and severity prompting him to seek care in our emergency department. A secondary cause was suspected, with fungal (given travel history) and neoplastic causes of greatest concern after fundus photography demonstrated bilateral papilledema.  He was admitted to Neurology, and CSF analysis revealed a clonal B-cell population consistent with chronic lymphocytic leukemia.  He was transferred to Oncology for staging and initiation of Rituximab.  

Conclusions:

Secondary causes of TAC should be considered in patients with red flag features or exam findings. Atypical symptoms in this patient’s case included travel history, rapid escalation of symptoms, exquisite symptomatic response to steroids, and poor response to standard therapies. Atypical signs included papilledema and persistent lymphadenopathy. Secondary headaches can mimic primary forms but may be associated with underlying malignancy, infection, or inflammatory disease; recognition and targeted diagnostic workup are critical.