Relief is often sought by increasing the intake of ergot or serotonin agonist preparations or even opioids, often to an alarming degree, but with only temporary relief, serving at times to perpetuate the condition. In the diagnosis of such persistent cases, the possibility should be considered that migraine has been complicated by this type of overuse of symptomatic medications with subsequent (“rebound”) worsening of headache. This cycle may produce a transformation of previously intermittent migraine into a low grade continuous headache with superimposed migrainous exacerbations. Narcotic addiction is another worry. In cases of status migrainosus, it is our practice to discontinue narcotic medications, and administer several of the following: intravenous hydration, metoclopramide, rapidly acting nonsteroidal anti-inflammatory drugs, magnesium, corticosteroids, or dihydroergotamine intravenous infusion in selected patients (see further on for details of treatment). In all likelihood, the patient has already been treated unsuccessfully with several medications and furthermore, the widely used serotonin agonist (“triptan”) medications are less likely to be helpful at this later stage of migraine.
An intriguing problem arises in the patient with migraine who is found to have a slight lymphocytic pleocytosis in the spinal fluid. Most of these cases in our experience have proved to be simply instances of aseptic meningitis that have precipitated migraine in susceptible individuals. In others, a few cells are found in the spinal fluid during an attack of migraine without obvious explanation; probably a minor cellular reaction of 3 to 10 white blood cells (WBCs)/mL may be innocuous if there is no fever or meningismus.
Bartleson, Swanson, and Whisnant under the title “A migrainous syndrome with cerebrospinal fluid pleocytosis.” A subsequent series by Berg and Williams introduced the acronym HaNDL (headache with neurological deficits and CSF lymphocytosis). The most extensive report was by Gomez-Aranda and colleagues, who described 50 adolescents and young adults, predominantly males, who developed multiple widely separated episodes of transient neurologic deficits lasting hours, accompanied by migraine-like headaches, sometimes with slight fever but no stiff neck. One-quarter of this group had a history of past migraine and a similar number had a viral-like illness within 3 weeks of the neurologic problem. The CSF contained from 10 to 760 lymphocytes per cubic millimeter, and the total protein was elevated. The transient neurologic deficits were mainly sensorimotor, often involving the hand and lips, and aphasia; only 6 patients had visual symptoms. The patients were asymptomatic between attacks and in none did the entire illness persist beyond 7 weeks. We have observed several cases, all in otherwise healthy middle-aged men and we found corticosteroids to be helpful.
It is important to exclude an immune reaction to nonsteroidal anti-inflammatory medications or to intravenous immunoglobulin, which are among the agents that cause otherwise unexplained aseptic meningitis and headache but generally not with migraine features.
The causation and pathophysiology of the HaNDL syndrome and its relation to migraine are obscure but may relate to a hypothesized neurogenic inflammation basis of migraine discussed further on. The distinction between this syndrome and the recurrent aseptic meningitis of
Mollaret and other chronic meningitic syndromes as well as cerebral vasospasm or vasculitis is difficult (see “Chronic Persistent and Recurrent Meningitis” in Chap. 31).