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Start free with EleplanFamilial or sporadic hemiplegic migraine
ORPHA:569Disease
What it is
A rare variety of migraine with aura characterized by the presence of a motor weakness during the aura. There are two main forms depending on the familial history: patients with at least one first- or second-degree relative who has aura including motor weakness have familial hemiplegic migraine (FHM); patients without such familial history have sporadic hemiplegic migraine (SHM).
Key facts
- Prevalence
- 1-5 / 10 000 (Europe)
- Age of onset
- Childhood
- Inheritance
- Autosomal dominant
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
6Common30–79%
23- Cerebral edema
- Complex febrile seizure
- Confusion
- CSF lymphocytic pleiocytosis
- CSF pleocytosis
- Diplopia
- Dissociated sensory loss
- Dysarthria
- Facial tics
- Focal manual automatism seizure
- Focal pedal automatism seizure
- Hearing impairment
- Hemiparesis
- Increased CSF protein concentration
- Involuntary movements
- Metamorphopsia
- Paresthesia
- Photopsia
- Postural instability
- Progressive gait ataxia
- Scotoma
- Tongue fasciculations
- Vertigo
Sometimes5–29%
17- Alien limb phenomenon
- Aphasia
- Cerebellar atrophy
- Coma
- Decreased vigilance
- Distal upper limb muscle weakness
- EEG with generalized sharp slow waves
- Facial paralysis
and 9 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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