What causes hemiplegic migraines and disability

Hemiplegic migraines - migraines with a motor aura

The very rare hemiplegic migraine is characterized by motor failures in combination with at least one other aura symptom, such as a visual disorder. Paralysis of one or more limbs together with flashes of light are examples of an attack of hemiplegic or complicated migraines, just like headaches after the aura. However, it can also lead to severe, possibly life-threatening, migraine attacks.

Complicated migraines: this is how the seizures manifest themselves

Hemiplegic migraines are different from "normal" migraines. In contrast to the more well-known forms, muscle weakness and an aura occur here. It means thatParalysis of the arms or legs in combination with the following symptoms during a hemiplegic migraine attack:

  • visual aura with flickering, flashes of light or visual field loss; partially with loss of vision
  • sensitive disorders, for example numbness or tingling sensations
  • Language problems
  • Basilar migraine symptoms such as dizziness or tinnitus

All of these features, which can appear in addition to motor failures in a hemiplegic migraine, are reversible and therefore regress with the end of the migraine attack. While aura symptoms such as flashes of light or speech disorders completely disappear after 5 to 60 minutes, the motor problems usually last up to 72 hours.1

Headaches usually arise after the aura symptoms. But it is also possible that they do not appear at all or even before. Between the attacks of these complicated migraines, 80 to 90 percent of the patients show no symptoms of the neurological failures - 10 to 20 percent suffer permanently from the consequences of the attacks. Each attack and the associated symptoms can be noted in a migraine diary for a better overview.2 Severe cases of hemiplegic migraines can have complicated consequences for the patient: Persistent loss of motor skills, confusion, fever and even coma as well as symptoms that resemble a stroke are possible.3

The familial hemiplegic migraine

A distinction is made between two types of patients within hemiplegic migraine. Those who have a first- or second-degree relative in the family who also has complicated migraines are affected by what is known as familial hemiplegic migraine (FHM).4 The severity of symptoms can vary within the family and it often affects children and young adults. The most common cause of familial hemiplegic migraine is a mutation (change) in the genes FHM1, 2 or 3. Men and women are affected equally often.5 The gene is inherited as an autosomal dominant trait. That means:

For example, if the father has an altered (mutated) and an unchanged FHM gene and the mother has two unchanged (healthy) genes, there is a 50 percent chance that the child will also suffer from hemiplegic migraine.6 If the unchanged gene of the mother is inherited together with the mutated gene of the father, the disease breaks out in the child due to the dominant property of the FHM gene.

The sporadic hemiplegic migraine

If a patient suffers from hemiplegic migraine and does not have a relative with the same symptoms, the form is referred to as sporadic hemiplegic migraine (SHM). In this variant of the complicated migraine, mutations of the FHM gene occur in only 10 to 20 percent of cases.7 There is not yet sufficient data for the remaining cases. The patients usually suffer from the symptoms as a child. Then there are often persistent accompanying symptoms such as epilepsy. In contrast to the classic migraine with aura, the headache typical of migraines occurs during both the FHM and the SHM during the aura symptoms.

Diagnosis of hemiplegic migraine

The diagnosis of familial hemiplegic migraine is somewhat easier in contrast to sporadic hemiplegic migraine, since several family members are often affected. This allows the signs to be assigned more precisely. If no relatives have such symptoms, the characteristics of complicated migraines (for example, paralysis and visual disturbances) also resemble a stroke or other neurological disorders. This is why imaging methods of the brain (magnetic resonance tomography) and punctures of the cerebrospinal fluid (cerebrospinal fluid) are essential for diagnosis.

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