Hemiplegic migraine- familial type 1
Overview
A rare inherited form of migraine that characteristically causes temporary paralysis on one side of the body and involves the presence of an aura. A migraine episode may be triggered by minimal trauma to the head. The severity of the disorder is variable with some patients experiencing paralysis on one side of the body or coma for weeks.
Symptoms
* Recurrent migraines * Muscle weakness on one side of body * Aura * Blurred vision * Nystagmus
Causes
t is believed that FHM mutations lead to migraine susceptibility by lowering the threshold for cortical-spreading-depression generation. The FHM1 and FHM3 mutations are occur in ion channels expressed in neurons. These mutations may lead to both the hyper and hypoexcitable neurons that might underlie cortical-spreading-depression. It is even less clear how the mutations seen in FHM2 patients might lead to FHM symptoms as the gene mutated in FHM2 is expressed primarily in astrocytes. One proposal states that the depolarization of astrocytes caused by haploinsufficiency of the ATP1A2 Na+/K+-ATPase causes increased release of compounds such as adenosine from astrocytes. These compounds then interact with neighboring neurons, altering their excitability and leading to cortical-spreading-depression and migraine.
Diagnosis
Diagnosis of FHM is made according to the following criteria: * Two attacks of each of the following: * Aura with motor weakness accompanied by either reversible visual symptoms (flickering lights, spots, lines, etc.), reversible sensory symptoms (pins and needles, numbness, etc.) or speech symptoms.
Treatment
Pain Medicine