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Episodic confusional states and other mental state changes as part of the migraine complex have been described since the 1800s. Confusion is defined as a state of altered consciousness in which patients are bewildered and misinterpret the world around them. Predominantly, there may be alterations in orientation, cognition, general behavior, motor activity, and features of psychotic or neurotic conditions [1]. Thinking becomes disorganized, and there is usually memory disturbance and a feeling of distress. The term 'acute confusional migraine'(ACM) was first introduced by Gascon and Barlow in 1970. They described four cases of childhood migraine presenting as an acute confusional state [2]. ACM accounts for one out of six migraine equivalents occurring in children [3]. It is a rare variant and, in contrast to the other pediatric migraine variants, is not mentioned in International Classification of Headache Disorders, 2nd edition (ICHD-II) [4]. In a database consisting of 5848 pediatric patients, of which 1106 were migraineurs, 108 patients (1.8% of total, 9.8% of migraineurs) were identified as having migraine equivalents, of whom five patients (0.45% of children with migraine) have been diagnosed with ACM [5]. In another, retrospective study, 22 out of 280 children and adolescents with migraine had ACM (7%) [6]. Until recently, ACM has been thought to almost only affect children and adolescents. However, our group described a series of ten adult patients [7] with similar symptoms, suggesting that the concept of confusional migraine should be expanded from the pediatric population to adults. We would consider confusional migraine, together with the hemiplegic migraine and the basilar-type migraine, as a complex migraine variant, and would therefore recommend adding it to the International Classification of Headache Disorders as a subtype of migraine with aura.
Phenotype
Information about confusional migraine is rare and mostly limited to case reports and case series. For a summary of the literature, see Table 1. The confusional state is a frightening presentation of migraine, for the patient as well as the physician. Phenotypically, this syndrome seems to have the following distinctive features: a sudden onset of confusion and agitation, often lasting longer than a typical migraine aura; followed or accompanied by typical migraine headaches, with a tendency to a more rapid recovery [8]. During a typical episode of ACM, patients are...