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Disease Profile
Familiar or sporadic hemiplegic migraine
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
1-5 / 10 000
Age of onset
Childhood
ICD-10
G43.1
Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Categories
Congenital and Genetic Diseases; Nervous System Diseases
Summary
Symptoms
- 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)
Motor weakness involves areas affected by sensory symptoms and varies from mild clumsiness to complete deficit. Affected people may also experience neurologic symptoms such as confusion, drowsiness, impaired consciousness, coma, psychosis, and/or memory loss. Neurologic symptoms can last for hours to days. Attention and memory loss can last weeks to months. However, permanent motor, sensory, language, or visual symptoms are extremely rare.[3]
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
Medical Terms | Other Names |
Learn More:
HPO ID
|
---|---|---|
80%-99% of people have these symptoms | ||
Focal motor |
0011153 | |
Focal sensory seizure | 0011157 | |
30%-79% of people have these symptoms | ||
Cerebral edema |
Swelling of brain
|
0002181 |
Complex febrile seizure | 0011172 | |
Confusion |
Disorientation
Easily confused
Mental disorientation
[ more ] |
0001289 |
CSF lymphocytic pleiocytosis | 0200149 | |
Diplopia |
Double vision
|
0000651 |
Dissociated sensory loss | 0010835 | |
Difficulty articulating speech
|
0001260 | |
Facial tics |
Cramping of facial muscles
Facial spasms
Jerking of facial muscles
Mimic spasms
Spasms of facial muscles
Twitching of facial muscles
[ more ] |
0011468 |
Hearing impairment |
Deafness
Hearing defect
[ more ] |
0000365 |
Increased CSF |
0002922 | |
Metamorphopsia | 0012508 | |
Paresthesia |
Pins and needles feeling
Tingling
[ more ] |
0003401 |
Photopsia | 0030786 | |
Postural instability |
Balance impairment
|
0002172 |
Progressive gait |
0007240 | |
Scotoma | 0000575 | |
Tongue fasciculations |
Tongue twitching
Twitching of the tongue
[ more ] |
0001308 |
Vertigo |
Dizzy spell
|
0002321 |
5%-29% of people have these symptoms | ||
Alien limb phenomenon | 0032506 | |
Cerebellar atrophy |
Degeneration of cerebellum
|
0001272 |
Coma | 0001259 | |
Decreased vigilance | 0032044 | |
Distal upper limb muscle weakness | 0008959 | |
Dysphasia | 0002357 | |
0011199 | ||
Facial paralysis | 0007209 | |
Gaze-evoked horizontal |
0007979 | |
Impaired thermal sensitivity | 0006901 | |
Nuchal rigidity | 0031179 | |
Seesaw nystagmus | 0012044 | |
Spontaneous pain sensation | 0010833 | |
Tinnitus |
Ringing in ears
Ringing in the ears
[ more ] |
0000360 |
Tremor | 0001337 | |
Vertical nystagmus | 0010544 | |
1%-4% of people have these symptoms | ||
Amaurosis fugax | 0100576 | |
EEG with focal sharp waves | 0011196 | |
First dorsal interossei muscle weakness | 0003392 | |
Mental deficiency
Mental retardation
Mental retardation, nonspecific
Mental-retardation
[ more ] |
0001249 | |
Status epilepticus |
Repeated seizures without recovery between them
|
0002133 |
Percent of people who have these symptoms is not available through HPO | ||
Agitation | 0000713 | |
Anxiety |
Excessive, persistent worry and fear
|
0000739 |
Aphasia |
Difficulty finding words
Losing words
Loss of words
[ more ] |
0002381 |
Apraxia | 0002186 | |
Ataxia | 0001251 | |
Auditory hallucinations |
Hallucinations of sound
Hearing sounds
[ more ] |
0008765 |
0000006 | ||
Blindness | 0000618 | |
Blurred vision | 0000622 | |
Drowsiness |
Sleepy
|
0002329 |
Dyscalculia | 0002442 | |
Episodic ataxia | 0002131 | |
Fever | 0001945 | |
Hemiparesis |
Weakness of one side of body
|
0001269 |
Hemiplegia |
Paralysis on one side of body
|
0002301 |
0003829 | ||
Migraine |
Intermittent migraine headaches
Migraine headache
Migraine headaches
[ more ] |
0002076 |
Migraine with aura | 0002077 | |
Nystagmus |
Involuntary, rapid, rhythmic eye movements
|
0000639 |
Photophobia |
Extreme sensitivity of the eyes to light
Light hypersensitivity
[ more ] |
0000613 |
Psychosis | 0000709 | |
Seizure |
Cause There are two types of hemiplegic migraine (HM), which are distinguished based on the
FHM may be caused by changes ( While SHM occurs in people with no history of the condition in their family, most cases are due to new ( Diagnosis Hemiplegic migraine is diagnosed based on the presence of specific signs and symptoms.
The diagnosis is based on the presence of having at least 2 attacks with:
Additionally, all other potential causes for the symptoms need to be ruled out (such as transient ischemic attack and stroke). While the criteria requires fully reversible symptoms, it is known that some people with severe attacks may have permanent neurological problems.[6] A diagnosis of Testing Resources
Treatment Treatment of hemiplegic migraine varies depending on severity and which symptoms are most problematic for the patient. In general, treatments aim to manage symptoms. Drugs that are effective in the prevention of common migraines may be used in hemiplegic migraine.[3]
There are a few articles describing the use of nasal administration of ketamine, intravenous verapamil, and triptans for treatment of aura in people with hemiplegic migraine. Use of triptans in hemiplegic migraine is controversial and may be contraindicated in people with severe attacks.[3] For further information on these and other treatments, we recommend that you speak with your healthcare provider. OrganizationsSupport and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD. Organizations Supporting this Disease
Social Networking Websites
Learn moreThese resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional. Where to Start
In-Depth Information
References
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