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Current thumbnail: Malignant migrating partial seizures in infancy are an epileptic encephalopathy characterized by onset in the first 6 months of life, rapidly progressive appearance of partial seizures that become subcontinuous, the onset migrating from one area to the other of the cortex, with major deterioration of the psychomotor abilities. Seizures exhibit a good correlation between clinical semiology and topography of the discharge on EEG. No etiology or effective treatment has been identified. This update confirms that this condition has been recognized in various countries and is therefore a new syndrome.

Historical note and nomenclature
A report in 1995 by Coppola and colleagues described 14 infants who developed migrating partial seizures (Coppola et al 1995). The first seizures had occurred at the mean age of 3 months, and the full pattern was developed between 1 month and 10 months of age. Patients regressed developmentally. Three of them died between 7 months and 8 years of age. Only 2 patients had seizures controlled by medication, and 3 patients resumed psychomotor development. Then, cases were identified in Japan (Okuda et al 2000), European countries (Wilmshurst et al 2000; Veneselli et al 2001), Israel (Gross-Tsur et al 2004), and the United States (Marsh et al 2005).

Clinical manifestations
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 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 seizures within clusters, infants are floppy, drooling, often somnolent, and unable to drink and swallow. The course of the disease is usually characterized by alternating periods of worsening until the patient is totally floppy and has lost all abilities and periods of seizure freedom during which there is slight improvement until the next period of worsening occurs. Microcephaly progressively occurs. Only patients whose seizures are brought under control acquire the ability to reach for objects, recognize their surroundings, and, eventually, walk.

Clinical vignette
No information was provided by the author.

Etiology
To date, no etiology has been identified by history, biochemical, radiological, or histological investigations. No familial case or consanguinity has been reported.

Pathogenesis and pathophysiology
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. However, these considerations do not clarify the etiology that is clearly not due to any malformation or acquired brain lesion and could, therefore, result either from chromosomal aberration of from some de novo monogenic mutation, affecting the channel gene, for instance.

Epidemiology
The incidence of the syndrome is unknown, but the author is aware of cases occurring in various parts of the world, including the Philippines. It is, therefore, likely that this disorder affects various areas throughout the world.

Prevention
There is no known prevention.

Differential diagnosis
Other types of cryptogenic epilepsies with partial seizures may be wrongly diagnosed when faced with frequent partial seizures in a young infant; these other epilepsies include partial epilepsy and focal seizures preceding West syndrome. Severe myoclonic epilepsy in infants is unlikely to be misdiagnosed as migrating partial epilepsy in infancy because onset is later, and seizures are much less frequent during the first year of life.

The major difficulty is recognizing the involvement of both hemispheres in an infant with intractable partial seizures that could be amenable to surgery. The high frequency of seizures permits them to be recorded and, therefore, a way of recognizing the condition (Gerard et al 1999).

Diagnostic Workup
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.

Prognosis
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, only a single patient is said to have had normal development at age 7 (Marsh et al 2005).

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

Pregnancy
Not applicable.

Anesthesia
Precautions must be taken regarding seizures.

References
Coppola G, Plouin P, Chiron C, Robain O, Dulac O. Migrating partial seizures in infancy : a malignant disorder with developmental arrest. Epilepsia 1995;36(10):1017-24.

Gerard F, Kaminska A, Plouin P, Echenne B, Dulac O. Focal seizures versus focal epilepsy in infancy: a challenging distinction. Epileptic Disord 1999;1(2):135-9.

Gross-Tsur V, Ben-Zeev B, Shalev RS. Malignant migrating partial seizures in infancy. Pediatr Neurol 2004;31(4):287-90.

Marsh E, Melamed SE, Barron T, Clancy RR. Migrating partial seizures in infancy: expanding the phenotype of a rare seizure syndrome. Epilepsia 2005;46(4):568-72.

Okuda K, Yasuhara A, Kamei A, Araki A, Kitamura N, Kobayashi Y. Successful control with bromide of two patients with malignant migrating partial seizures in infancy. Brain Dev 2000;22(1):56-9.

Veneselli E, Perrone MV, Di Rocco M, Gaggero R, Biancheri R. Malignant migrating partial seizures in infancy. Epilepsy Res 2001;46(1):27-32.

Wilmshurst JM, Appleton DB, Grattan-Smith PJ. Migrating partial seizures in infancy: two new cases. J Child Neurol 2000;15(11):717-22.

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Abbreviations
EEG:electroencephalogram
MRI:magnetic resonance imaging

ICD-9 code
345.1

Associated disorders
West syndrome

Keywords
focal ictal discharges
hypotonia
infants
mental retardation
microcephaly
partial seizures
psychomotor deterioration
seizure clusters

Minor keyword descriptors
epilepsy
jerks
seizures
twitching

Age of presentation
0-01 month
01-23 months

Age of typical presentation
0-01 month
01-23 months

Population group(s) preferentially affected
none selectively affected

Occupation group(s) preferentially affected
none selectively affected

Sex
male=female

Family history
none

Heredity
none

Permuted topic, synonyms, variants
Migrating partial epilepsy in infancy
partial epilepsy in infancy, Migrating
epilepsy in infancy, Migrating partial

Related topics
Epilepsy

Differential diagnosis
Partial epilepsy
Focal seizures preceding West syndrome
Severe myoclonic epilepsy in infants

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