Harry's Journey

ABOUT MIGRATING PARTIAL EPILEPSY

I am not medically trained and as as such i have taken this information from literature that i have at home. I will ask during our next Neurologists appointment whether he has any information that i can use on this part of the site.

The age of onset for migrating partial seizures in infancy ranges from 13 days to 7 months, and frequency increases progressively over a mean period of a few weeks (7 days to 3 months). Between 1 month and 10 months of age, the full pattern of the disorder is reached. Seizures are then almost continuous, and the patients experience psychomotor deterioration.

Early seizures have motor and autonomic components, consisting of apnea, cyanosis, and flushing. Later, seizures are more polymorphic, varying from one seizure to the next in a given patient. Clinical features consist of lateral deviation of the eyes with eye jerks, twitching of the eyelids, limb jerks, chewing movements, apnea, flushing, and salivation. Secondary generalization may occur. The combination of these different features produces a wide range of manifestations, but many seizures are clinically mild and easily overlooked without EEG recording. Seizures last a few minutes. They tend to be more frequently generalized as time goes by. Myoclonus is rare, and spasms are most exceptional. By the end of the first year of life, seizures become almost continuous, occurring in clusters of 5 seizures to 30 seizures several times a day or in series of 2 days to 5 days in a row.

The course is characterized by clusters of seizures lasting several to a few weeks, during which the infant deteriorates considerably, and followed by disappearance of seizures during a few weeks with slow improvement of the condition. Between seizure clusters, infants are floppy, drooling, often somnolent, and unable to drink and swallow. Microcephaly progressively occurs.

There is no clue as to the neurologic pathogenesis and pathophysiology of this condition, but the occurrence of partial seizures beginning in various parts of the brain suggests that the whole brain cortex is affected by hyperexcitability. Although the whole brain is affected, seizures do not generalize because, due to the young age, there is insufficient maturity of the pathways involved in generalization. In addition, the short age range for onset suggests that the disorder is determined by some maturation-related phenomenon as in other generalized epilepsy syndromes beginning in infancy.

Diagnosis is based on the combination of early onset, partial seizures affecting various parts of the brain, and seizures occurring in clusters. Interictal EEG may be normal during the first weeks of the disease. Multifocal spikes, particularly in the rolandic and temporal areas, and diffuse slowing of the background activity occur within a few weeks on both awake and sleep recordings. Later, all recordings are abnormal with multifocal spikes not activated in sleep. Sleep-wake differentiation is only visible during seizure-free periods, and spindles are rare.

Ictal EEG shows apparently random involvement of various areas of the brain, but video-EEG shows clear clinical-EEG correlation. Each given seizure consists of rhythmic theta activity starting in one region and progressively affecting adjacent areas as the frequency of the rhythmic activity decreases steadily. Subclinical discharges may also occur. Consecutive seizures in one recording may affect different areas and overlap, a seizure starting before the preceding one has finished. Thus, the area involved shifts from one area to the other throughout the recording. However, this particular pattern may not be seen before several weeks of the disease, and at first, seizures may affect mainly 1 area.

The course is most severe because, in most cases, seizures never come under control. For the rare patient whose seizures are controlled before the end of the first year of life, walking is possible. To date, however, no patient has acquired the ability to speak. 

There is no specific treatment of this condition. Open data of the effect of the combination of clonazepam and stiripentol have been reported. Drugs for partial epilepsy seem to worsen the condition.