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Genetic and Rare Diseases Information Center (GARD)

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Familial hemiplegic migraine type 2


Other Names for this Disease

  • FHM2
  • Hemiplegic migraine, familial type 2
  • MHP2
  • Migraine, familial hemiplegic, 2
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Symptoms

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What are the signs and symptoms of hemiplegic migraine?

Symptom and symptom severity can vary considerably between people with hemiplegic migraine. Signs and symptoms of hemiplegic migraine are outline below.

Signs and symptoms associated with aura:[1]

  • Visual disturbance (e.g., blind spots, flashing lights, zigzag pattern, and double vision)
  • Sensory loss (e.g., numbness or paresthesias of the face or an extremity) 
  • Difficulty with speech (which usually occur along with right-sided weakness) 
Signs and symptoms associated with motor weakness:[1]

  • Hemiparesis (weakness of one limb or one side of the body)
Neurologic symptoms:

  • Confusion 
  • Drowsiness 
  • Impaired consciousness 
  • Coma
  • Psychosis
  • Memory loss

Motor weakness is always associated with at least another aura symptom. People with hemiplegic migraine often describe a typical sensory aura that starts as tingling in one of the fingers and gradually progresses to the other fingers, up to the arm, and then affects the face, tongue, and later the body and leg.[1] In some patients, paresthesias (e.g., tingling) is followed by numbness. For other people sensory loss (e.g., numbness) is always predominant.[1] 

Motor weakness involves areas affected by sensory symptoms and varies from mild clumsiness to complete deficit. Sensory-motor symptoms usually start in one hand and gradually spread up to the arm and the face. These symptoms can be restricted to one limb or can spread all over one side of the body. They can affect both sides of the body, occurring simultaneously or in succession, or remain unilateral, switching side from attack to attack, or always involving the same side.[1] Speech disturbances mostly affect expression, with rare comprehension impairment.[1]

Neurologic signs and symptoms can last for hours to days. These symptoms can outlast headache. Attention and memory loss can last weeks to months. Permanent motor, sensory, language, or visual symptoms are extremely rare. Some people with the familial form of hemiplegic migraine develop mild ataxia, usually in adulthood.[1]

Last updated: 8/26/2013

The Human Phenotype Ontology provides the following list of signs and symptoms for Familial hemiplegic migraine type 2. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Abnormality of movement 90%
Hemiplegia/hemiparesis 90%
Incoordination 50%
Nystagmus 50%
Abnormal retinal pigmentation 7.5%
EEG abnormality 7.5%
Neurological speech impairment 7.5%
Sensorineural hearing impairment 7.5%
Aphasia -
Apraxia -
Autosomal dominant inheritance -
Blurred vision -
Coma -
Confusion -
Diplopia -
Drowsiness -
Dysarthria -
Dysphasia -
Fever -
Hemianopic blurring of vision -
Hemiparesis -
Hemiplegia -
Incomplete penetrance -
Intellectual disability -
Intermittent cerebellar ataxia -
Migraine with aura -
Seizures -
Vertigo -

Last updated: 9/2/2014

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.


References
  1. Jen JC. Familial Hemiplegic Migraine. GeneReviews. September 2009; http://www.ncbi.nlm.nih.gov/books/NBK1388/#fhm.Clinical_Description. Accessed 8/26/2013.


Other Names for this Disease
  • FHM2
  • Hemiplegic migraine, familial type 2
  • MHP2
  • Migraine, familial hemiplegic, 2
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.