A Case of Intermittent Ataxia Associated with Migraine Headaches Teaching Case Report

Practice Teaching Case Report Box 1: Possible diagnoses in a A case of intermittent ataxia associated with migraine patient with ataxia Primary intermittent ataxia headaches syndromes (often not progressive) With headaches The case: A previously healthy 42-year- or dysarthria and did not display any • Episodic ataxia type 2 old woman had presented to our neu- myokymia about the eyes, lips or fin- • Basilar migraines rology service 1 year earlier with left- gers. She recalled experiencing similar • Episodic ataxia with paroxysmal sided temporal headaches that she mild attacks since the age of 25 that in- choreoathetosis and spasticity said typically lasted from a few hours volved clumsiness and occasional falls • Familial hemiplegic migraines to all day. They were associated with and that lasted from a few hours to a Without headaches* nausea but not with vomiting. Begin- day. These attacks were not associated • Episodic ataxia type 1 ning at age 13 years, these headaches with diplopia, hearing loss, weakness, • Periodic vestibulocerebellar had occurred at a frequency of once a choreiform movements, abnormal pos- ataxia month to once every 2–3 months. The turing, fatigable weakness, cognitive • Spinocerebellar ataxia type 6 woman did not experience visual deficits, seizures, stiffness or myotonia. Secondary intermittent ataxias* auras, such as scintillation photopsias The attacks were not related to head (often progressive) (flashes of light), migrating scotomata position or change of posture. Her mi- • Hartnup’s disease (patches of blurred or absent vision) graine headaches occurred both with • Pyruvate decarboxylase deficiency or fortification spectra (wavy linear and without episodes of ataxia. Simi- • Leigh’s disease “zig-zag” patterns that resemble the larly, ataxic episodes occurred without • Hereditary hyperammonemias battlements of a medieval fort). She migraine headaches. An electroenceph- had no history of vertigo or seizures. alogram appeared normal, as were the Primary ataxia syndromes* (sometimes progressive) The headaches tended to occur results of routine blood tests, including • Spinocerebellar ataxia around the time of her menses. She blood count, electrolyte levels and rated the pain at 3 on a scale of 1–10 serum glucose level. The patient pro- • Multiple system atrophy (10 being most severe), but she noted vided a detailed family history, describ- • Celiac disease that on occasion the headaches could ing intermittent clumsiness in her fa- • Antiphospholipid syndrome be severe enough to wake her from ther and migraine headaches in 2 of 4 • Paroxysmal psychosis sleep and merit a pain rating of 8 or 9. siblings. • Seizures Clinical examination was unremark- A provisional diagnosis of episodic • Drugs (e.g., anticonvulsants) able during each of her visits. Ten ataxia type 2 was made in view of the • Toxins (e.g., alcohol) years earlier, atypical migraine had patient’s history of migraine, episodes “Pseudoataxia”* been diagnosed and treated sympto- of ataxia with normal examination be- • Paroxysmal kinesigenic matically; no migraine prophylaxis tween episodes and a strong family dyskinesias 1,2 had been given in view of her infre- history. The patient responded to ac- *Headaches may occur incidentally. quent and mild headaches. Magnetic etazolamide, 250 mg 3 times a day, resonance imaging scans of the brain but experienced relapses of ataxia were unremarkable. The diagnosis of once or twice a year when she did not atypical migraine was not altered at take her medication. With amelio- 19p13, which codes for the main trans- this time. ration of her ataxic episodes, she membrane component of the neuronal One year after her initial visit to our became bothered by her migraine calcium channel.2 neurology service, the patient was ad- headaches, which responded to pro- mitted to hospital because of unsteadi- phylaxis with amitriptyline and in- ness of gait, gaze-evoked nystagmus domethacin for analgesia. Sumatrip- Ataxia can be progressive, stable or and truncal ataxia (see video of typical tan was efficacious for more severe episodic and can occur with or with- 1 episode, available online at www.cmaj migraine headaches. out headaches (Box 1). Our patient 0 0 0 .ca/cgi/content/full/177/6/565/DC1). She The patient underwent genetic had migraine headaches and episodes 7 0 . j a did not report experiencing headaches counselling and testing. None of the of ataxia, with normal clinical findings m c / at the time. Her condition resolved known mutations that cause episodic between attacks. Her ataxic episodes 3 0 5 1 spontaneously after about 18 hours, ataxia was detected in exons 23, 26, 28, and migraines may have been distinct . 0 1 : and findings on clinical examination 29, 32 and 35 of the calcium-channel but coincidental clinical entities or I O D were normal. She had no hearing loss gene (CACNA1A) on chromosome manifestations of a single disease. A CMAJ • September 11, 2007 • 177(6) 565 © 2007 Canadian Medical Association or its licensors Practice 5 5 6 6 6 6 Table 1: Clinical characteristics of the key forms of episodic ataxia with and without headaches Episodic ataxia with paroxysmal Periodic Episodic ataxia Episodic ataxia choreoathetosis and Basilar type Familial hemiplegic vestibulocerebellar Spinocerebellar Characteristic type 11,2 type 21,2 spasticity1 migraine3 migraine1,2 ataxia1 ataxia type 64 Onset Childhood Childhood to second First to second Mostly seen in First to second decade Third to sixth decade Third to sixth decade decade decade young adults Type and Attacks of midline Attacks of midline Occasional attacks Cerebellar ataxia Truncal ataxia and Short episodes Progressive cerebellar duration of cerebellar disturbances cerebellar lasting minutes during migraine nystagmus sometimes increase in frequency dysfunction, with gait ataxia lasting seconds to disturbances lasting attack occur and may eventually ataxia and dysarthria minutes hours become constant Type and None Migraine Occasional Migraine with Migraine None None duration of headaches auras originating headache from brain stem CMAJ • September 11, 2007 • 177(6) Triggers Emotional (startle) and Emotional and Physical exertion, Usual migraine Usual migraine triggers Change in head None. Ataxia is physical stress physical stress sleep deprivation, triggers position, fatigue, progressive emotional stress, environments where alcohol objects are moving past (e.g., moving car or escalator) Other Interictal neuromyotonia Myokymia Choreoathetosis, Dysarthria, Transient hemiplegia during Defective smooth Pure cerebellar neurologic and myokymia uncommon. Subtle, dystonia, vertigo, tinnitus, migraine aura. Hemianopia pursuit, gaze-evoked syndrome. Vibratory manifestations prominent. May be slowly progressive paresthesia, diplopia, visual or dysphasia possible. nystagmus, vertigo, and proprioceptive associated with interictal cerebellar diplopia symptoms. Last Occasional abnormal eye diplopia, oscillopsia sensory loss and epilepsy, infantile signs, especially minutes to hours movements due to and tinnitus impaired eye contractions and nystagmus vestibulocerebellar movements. Sensory or neuromyotonia. Ictal dysfunction sensorimotor axonal dysarthria in some cases neuropathy possible Neuroradiology Not applicable Cerebellar (vermis) Not applicable Not applicable Cerebellar (vermis) atrophy Cerebellar (vermis) Pure cerebellar atrophy may be seen occasionally seen on MRI. atrophy possible atrophy on MRI Normal diffusion weighted images and evidence of hyperperfusion seen on SPECT and perfusion MRI, which suggests that hemiplegia is not due to cerebral ischemia Treatment Acetazolamide, Acetazolamide Acetazolamide, NSAIDs Acetazolamide Response to Ataxic symptoms carbamazepine, phenytoin Caffeine, triptans acetazolamide varies benefit from use of sulthiame, and codeine best from none to good 5-hydroxytryptophan, amphetamines avoided response buspirone or acetazolamide Pathogenesis Potassium Calcium Possibly potassium Vasoconstriction Calcium channelopathy Unknown Calcium channelopathy channelopathy channelopathy channelopathy Inheritance; Autosomal dominant; Autosomal dominant; Autosomal Not applicable Autosomal dominant; Autosomal dominant; Channelopathy arising gene KCNA1 gene on CACNA1A gene on dominant; linked to CACNA1A gene on genetically distinct from trinucleotide identified chromosome 12 chromosome 19 chromosome 1p chromosome 19 from other autosomal repeats in CACNL1A4 dominant ataxias gene on chromosome 19p13 Note: MRI = magnetic resonance imaging, SPECT = single-photon emission computed tomography, NSAID = nonsteroidal anti-inflammatory drug. Practice discussion of the differential diagno- Channelopathies, such as paroxys- sis of recurrent and relapsing ataxia mal kinesigenic dyskinesias, can be as- REFERENCES follows. sociated with migraine and can mimic 1. Gordon N. Episodic ataxia and channelopathies. Brain Dev 1998;20:9-13. Episodic ataxias are characterized by episodic ataxias. Clinical examination 2. Kullmann DM. The neuronal channelopathies. spontaneous paroxysmal periods of during the attack (see online video, Brain 2002;125(pt 6):1177-95. 3. Headache Classification Subcommittee of the In- ataxia that typically last from minutes available at www.cmaj.ca/cgi/content ternational Headache Society. The international to hours or days. Between episodes, the /full/177/6/565/DC1) confirmed the classification of headache disorders, 2nd edition. Cephalalgia 2004;24(Suppl 1):S9-160. patient is normal, except for the pres- presence of truncal ataxia rather

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