Typical manifestations | |
Migraine, usually with aura, as the first symptom in the third decade of life | |
Recurrent subcortical ischemic events (transient ischemic attack/stroke) in adulthood | |
Mood disturbances, apathy and depression among other psychiatric symptoms | |
Progressive cognitive decline, especially of executive functioning | |
Seizures, in a smaller but well-defined portion of patients | |
Atypical manifestations | |
Pathological gambling [128] | |
Recurrent status epilecticus [129] | |
Late onset [39] | |
Bipolar disorder [142] | |
Inflammatory-like presentation [143] | |
Acute vestibular syndrome [144] | |
Acute confusional migraine [147] | |
Sporadic hemiplegic migraine with normal imaging [148] | |
Post-partum psychiatric disturbances [149] | |
Parkinsonism [150] | |
Recurrent transient global amnesia [151] |