Case 1: Vertigo and Episodes of Slurred Speech in a 5-year-old Girl (2017)

A 5-year-old girl presents with symptoms of vertigo and intermittent episodes of slurred speech for 1 day. This morning she had a “popping” headache localized bilaterally to the sides and the back of the head. Her headache spontaneously resolved within 20 minutes, although she later experienced “a spinning sensation” and her knees buckled, causing her to fall backward. There is no history of ataxia, visual changes, weakness, or fatigue before the fall. Similarly, there is no history of altered mental status or evidence of injury afterward.Six months earlier, she had been diagnosed as having localization-related epilepsy. It was likely that her epilepsy was of genetic origin and considered benign because of normal brain magnetic resonance imaging (MRI) findings, electrographic abnormalities noted on video electroencephalography (EEG), and semiology that was noted during 3 witnessed episodes of tonic-clonic movements. Her epilepsy was initially well controlled with levetiracetam and oxcarbazepine therapy. In the preceding months, she would experience episodes of “spacing out” during which she would see colors in the air or smell things, “freeze up” (ie, have behavioral arrest), and then be unable to recall what happened.Her birth, developmental, and immunization history is noncontributory. Her mother has a history of depression, anxiety, and migraines with aura. Evaluation in the emergency department reveals episodic dysarthria without aphasia. She is alert and oriented, with normal vital signs and neurologic examination findings, including cranial nerves, cerebellar function, and deep tendon reflexes.Laboratory evaluation includes a complete blood cell count, serum electrolytes, urinalysis, and toxicology screen. She is hospitalized for further evaluation.Given the history of localization-related epilepsy in this child, the primary question was whether this presentation was related to the diagnosis of epilepsy or whether it was a new and unrelated condition. A subsequent normal 24-hour video EEG (on antiepileptics) and a negative urine toxicology screen helped to exclude nonconvulsive status epilepticus (a state of continuous or repetitive seizures without convulsions) and toxic ingestions, respectively. (1) More serious neurologic conditions, including arteriovenous malformations, intracranial neoplasms, stroke, and intracranial hemorrhage, were ruled out by a normal brain MRI (with and without contrast) and the fully reversible nature of the patient’s symptoms. Other disorders, such as speech and language disorders (ie, autism spectrum disorder, acquired aphasia), central auditory processing disorder, infection (ie, tick paralysis), head trauma, and abuse, were not considered, given the acute nature of the symptoms and the lack of a supporting clinical history.Given the maternal history of migraines and the episodic nature of her symptoms, migraine with brainstem aura (MBA) was considered after the MRI and EEG results were obtained. Although normal EEG findings do not rule out seizure disorder, the EEG obtained during a high yield period (within 24 hours of symptom onset) along with hyperventilation/photic stimulation was unremarkable.Migraines are surprisingly common in children, and approximately 14% to 30% are associated with auras (ie, visual, sensory, speech, language, motor, brainstem, and retinal). However, MBA is a rare diagnosis. It is usually suspected when a patient presents with episodic attacks of vertigo, dysarthria, diplopia, ataxia, and confusion and, specifically, when more typical migraine auras are also present. (2) Because there are no specific diagnostic tests for MBA, uncovering MBA as the underlying etiology of a patient’s presentation requires a high index of suspicion and an understanding of the pathogenesis of migraine disorders.More commonly, MBA is seen in females aged 7 to 20 years, with the migraine episodes becoming more typical as the patient ages. (3) The most characteristic feature of MBA is the absence of weakness, setting it apart from familial hemiplegic migraine with basilar-type symptoms, in which, commonly, visual disturbances with unilateral motor and sensory symptoms occur. Specific emphasis is placed on the paroxysmal and alarming nature of the symptoms, abrupt and complete resolution, lack of motor weakness, and a family history of migraines. Prolonged weakness can follow multiple attacks of hemiplegic migraine but rarely follow MBA. Migraine with brainstem aura should be diagnosed only when the aura symptoms arise from the brain stem and there is no evidence of motor weakness. (4)(5) The auras usually consist of combinations of vertigo (61%), dysarthria (53%), tinnitus (45%), diplopia (45%), bilateral visual symptoms (40%), bilateral paresthesia (24%), decreased level of consciousness (24%), or hypoacusis (21%). (6) The International Classification of Headache Disorders includes specific diagnostic criteria for MBA. (4) It emphasizes that the presence of an aura with motor symptoms excludes MBA and points to the diagnosis of hemiplegic migraine.Conditions that cause symptoms and signs referable to the brain stem or posterior circulation, such as transient ischemic attacks, strokes, basilar aneurysm, subarachnoid hemorrhage, episodic ataxia, Ménière disease, vestibular neuritis, posterior fossa tumors, toxic-metabolic encephalopathy, or psychogenic attacks, must be kept in mind as possible differential diagnoses. Therefore, it is vital to obtain a detailed history, to observe the patient for 24 hours to rule out these conditions, and to confirm the episodic nature of the aura symptoms.Data on prognosis and therapy for MBA are limited. Acute therapy for attacks is no different than that for a typical migraine with aura (ie, antiemetics such as phenothiazine and other nonvasoconstricting abortive agents, such as nonsteroidal anti-inflammatory drugs). However, limited data have shown that patients with MBA may be at risk for cerebral ischemia when exposed to vasoconstricting agents, such as β-blockers, serotonin agonists commonly referred to as “triptans,” or ergotamine derivatives. (7) We recommend using acute nonvasoconstricting agents as needed and preventive therapy on a case-by-case basis, given the limited literature providing specific criteria for treatment, mainly if the episodes are recurrently debilitating. We suggest using sustained-release verapamil or topiramate for this purpose. (8)(9)Patients and families should be counseled on the relatively benign nature of this disorder and how the presentation may change to a more typical migraine as the patient ages. The frequency of episodes may decrease as well.For our patient, no changes were made to her seizure medication regimen and no limitations were placed on her activities. (7)(10)