A 17‐year‐old boy presented with a 1‐week history of headache, confusion, and weakness. The headache was initially localized behind his left eye and was associated with emesis. He was given a prescription for sumatriptan and naproxen for presumed migraine. Four days later, he presented with blurry vision, left‐sided weakness, and word‐finding difficulty. He was somnolent but arousable. Strength was slightly decreased in left hand grip and left hip flexion. Chemistries, CBC with differential, and urine toxicology screen were unremarkable. CT of the head was negative. An MRI/MRA of the brain demonstrated increased cerebrospinal fluid (CSF) signal. CSF analysis revealed 360 nucleated cells/mm3 (94% lymphocytes), protein of 237 mg/dL, and glucose of 53 mg/dL CSF culture was negative. Tests for arbovirus, Rocky Mountain spotted fever, Bartonella, enteroviruses, HIV, Epstein–Barr virus, cytomegalovirus, and herpes simplex virus infection were negative. Anti‐nuclear antibody was weakly positive at 1:160 in a speckled pattern. Extractable nuclear antigen antibodies and double‐stranded DNA antibodies were negative. Based on these findings, a diagnosis of headache with neurologic deficits and CSF lymphocytosis (HaNDL) was made. No further treatment was necessary, and the patient was discharged with resolution of symptoms.
Headache with neurologic deficits is frequently encountered by hospitalists. Because an incorrect diagnosis can lead to significant morbidity and mortality, it is imperative that life‐threatening diagnoses be quickly established or ruled out. The differential diagnosis includes hemiplegic migraine, pseudotumor cerebri, meningitis, stroke, and structural brain lesions. This patient's symptoms and findings are consistent with HaNDL, which is a diagnosis of exclusion. First described by Bartleson in 1981, it is a rare condition with fewer than 100 patients reported in the 2 largest series. Patients with HaNDL experience variable neurologic deficits with headache and occasionally fever. Analysis of CSF demonstrates lymphocytic pleocytosis. Neuroimaging is typically normal. EEG may reveal focal slowing. The etiology of HaNDL is unknown, although viral meningoencephalitis and migraine are not likely causative factors. Because up to one quarter of patients have a viral prodrome, it is hypothesized that an infection causes an autoimmune response in the central nervous system with the resultant clinical picture. The disease course is self‐limited.
In a young patient with headache, transient neurological deficits, and lymphocytic pleocytosis with no infectious source, the diagnosis of HaNDL should be considered.
To cite this abstract:Bloomfield R, Edwards E. You Can't Handl the Truth: Another Cause of Headache with Neurologic Deficits. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 413. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/you-cant-handl-the-truth-another-cause-of-headache-with-neurologic-deficits/. Accessed April 4, 2020.