In infancy and childhood, inborn errors of metabolism and developmental disorders such as neuronal migration anomalies and neurocutaneous
syndromes predominate as identifiable causes.
Neurofibromatosis (NF) is a congenital disease of the group of autosomal dominant neurocutaneous
phakomatoses including also the tuberous sclerosis complex, the Hippel-Lindau syndrome and the basal-cell nevi syndrome.
syndromes (tuberous sclerosis complex, Sturge-Weber, etc.
(lesions on skin and brain) genetic syndromes are associated with seizures.
Review Tuberous sclerosis complex and neurofibromatosis type 1: the two most common neurocutaneous
The neurofibromatoses are one of the common types of autosomal dominant neurocutaneous
disorders which can be divided into neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and 'segmental neurofibromatosis , with each types having their own special characteristics and association with other malignancies.
After the illness has resolved, the virus remains latent for life in the dorsal spinal ganglia and can be reactivated as a neurocutaneous
disease, herpes zoster, when immunity to VZV declines.
In 20 chapters, neurologists, neuroscientists, and pediatricians from the US and UK discuss its epidemiology and common comorbidities; the classification and definition of seizures and epilepsy syndromes; medical management; common associated genetic and neurocutaneous
disorders; evaluation, the use of EEG, imaging, and non-epileptic paroxysmal events; treatment, including pharmacology, the therapeutic efficacy of antiepileptic drugs, adverse effects, vagus nerve stimulation therapy and surgery, and dietary therapies; generalized seizures and epilepsy syndromes; partial-onset seizures and localization-related epilepsy syndromes; and epilepsies related to age, etiology, or duration.
NF remains the most common and surgically important of the neurocutaneous
Leprosy is a chronic infectious disease caused by Mycobacterium leprae that leads to neurocutaneous
damage in susceptible patients.
Biotinidase deficiency (BD) is a biotin responsive, autosomal recessive inherited neurocutaneous
, treatable metabolic disorder.
Kucukoduk S, Ozsan H, Turanli AY, Dinc H, Selcuk M: A new neurocutaneous
syndrome: nevus sebaceus syndrome.