Title
A descriptive study of "Migrating Partial Seizures in Infancy" in the UK
Description
This study has been submitted on behalf of the BPNA Epilepsy Interest Group. The aim of this study is to obtain a profile of all cases of MPSI in the UK. This will include the collection of data including clinical details, a description of the clinical course, EEG findings, results of other investigations, response to anticonvulsant and other treatments for epilepsy and developmental outcome. We hope that the outcome of this survey will result in an improved understanding of the clinical course of what appears to be a specific epilepsy syndrome and also lead to its earlier recognition. It is also possible that a larger collection of children with MPSI syndrome may eventually result in the identification of a specific aetiology of this epilepsy syndrome.
Lead Clinician
Dr Rachel Kneen, Consultant Paediatric Neurologist, Royal Liverpool Children's Hospital
Disease name
Migrating Partial Seizures of Infancy
Case Definition
Case definition is taken from the original description by Coppola and Dulac in 1995 but has been widened slightly to maximise case ascertainment. Cases will be definite if they fulfill the essential criteria or probable if they fulfill most of the essential criteria. Both "definite"
and "probable" cases can be included.
Essential Criteria:
- Onset of seizure disorder before 9 months of age (incl. neonatal period)
- Focal motor seizures at onset
- Multifocal seizures which are from the outset or subsequently become intractable to conventional anti-epileptic drugs.
- Initial interictal EEG may be normal but will subsequently develop characteristic changes, including interictal multifocal spikes and ictal independent unilateral and migrating involvement of different cortical areas with clinical-EEG correlation. These migrating ictal seizures are essential for the diagnosis. However, if a case is suspected before ictal EEG has been captured, then this case should be deemed "probable" until more information becomes available and can be included.
- Following seizure onset, developmental progress is delayed or children may show signs of psychomotor regression
- No other identified epilepsy syndrome or alternative diagnosis.
Supporting Criteria (not always present):
- Declining occipito-frontal circumference
-Seizures may have an autonomic component, including facial flushing, epiphora (tearing), fleeting rash, apnoea.
- Development may be normal or delayed before seizure onset
- MRI may be normal or, over time, may show changes consistent with cerebral atrophy. However, recently, a single case has been reported with left temporal lobe atrophy and hippocampal sclerosis. Therefore focal MRI changes do not exclude the diagnosis.
Inclusion Criteria
Any patient who fits with the case description above and who has a probable or definite diagnosis of MPSI. If any doubt over whether to include the patient, their case could be discussed with the Lead Clinician on behalf of the BPNA Epilepsy Interest Group.
Patients may be living or deceased and may be included retrospectively or prospectively.
Exclusion Criteria
Any patient with epilepsy who does not have a probable or definite diagnosis of MPSI