Outcome after Temporal Lobectomy

Thirty seven patients, ages 7 to 18 years, who underwent temporal lobectomy for intractable seizures at the Mayo Clinic, Rochester, MN, between 1970 and 1983, were followed for a mean of 19 years (range 4 - 27 years).

COMMENT. In this long-term study of children following temporal lobectomy for intractable seizures, of 88% with an initial excellent outcome by 5 years, half of the patients had recurrence of seizures by 15 years. The incidence of mesial temporal sclerosis in patients with long-term recurrence was only 2.7% in this series, compared to 14% in previous reported series. The poorer outcome with long-term follow-up should be considered in decisions to operate early in children with temporal lobe seizures.
Previous studies of the natural history of recurrent seizures after resective surgery for epilepsy have shown that the outlook is better if seizure recurrence is delayed until after the first postoperative year. Also, changes in antiepiieptic medication, dosages, or poor compliance may be important factors in recurrence of seizures, (see Wingkun EC, Awad IA et al. Epilepsia 1991; see Progress in Pediatric Neurology II. PNB Publishers, 1994;ppl36-7).

CAT SCRATCH DISEASE AND EPILEPSIA PARTIALIS CONTINUA
An 18-year-old female young adult with seizures associated with cat scratch disease is reported from Norwalk Hospital, Yale University School of Medicine, CT. She presented with difficulties in speaking followed by generalized tonic-clonic seizures and continuous right facial motor seizures refractory to antiepiieptic drugs. Audio-video EEG monitoring revealed the epilepsia partialis involving the right lower face and occasionally, the tongue and larynx, without surface EEG abnormalities. MRIs were initially normal, and later showed an increased signal on T2-weighted images in the left frontoparietal region. SPECT also showed a focus of increased activity in the same region. The patient had a new kitten at home and cat scratch disease (CSD) serology for Bartonella henselae was positive at a titer of Pediatric Neurology Briefs 2002 1:256 (cut off <1:64). Ehrlichia and Lyme serology were negative. Significant improvement in symptoms occurred within 3 weeks of starting antibiotic therapy, and a repeat CSD titer was 1:128. After 1 year follow-up and gradual tapering of antiepileptic drugs the patient remains seizure-free. Brain MRI and SPECT are now normal. (Nowakowski GS, Katz A. Epilepsia partialis continua as an atypical presentation of cat scratch disease in a young adult. Neurology December (1 of  COMMENT. Encephalopathy and seizures are an unusual presentation of cat scatch disease, and symptoms may be delayed for several weeks after the kitten scratch or bite. Diagnosis is dependent on the history of kitten exposure and positive serology. Spontaneous recovery usually occurs after several days without the need for antibiotics. In the present case, symptoms persisted for several weeks and recovery was prompt only after antibiotics were initiated. The differential diagnosis includes Lyme encephalitis which may occur concurrently. (See Progress in Pediatric Neurology II pp 421-423, for review of 4 previous articles on neurologic complications of cat scratch disease.

GENETICS OF SIMPLE FEBRILE SEIZURES
A clinical and genetic study of three families with simple febrile seizures (FS) and an autosomal dominant (AD) trait with high penetrance is reported from the Hopital Pitie-Salpetriere, Paris, France. Among 29 affected family members, FS ceased before 5 years of age, only one had rare afebrile seizures in addition, and none developed epilepsy. A genome-wide scan in two famiies identified a new locus on chromosome 6q22-q24. This linkage was absent in the third family, COMMENT. This mapping to 6q22-q24 is the first identified locus responsible for simple febrile seizures. Identification of the gene is ongoing. This phenotype differs from the known loci reported for FS and GEFS+. All modes of inheritance have been described, autosomal dominant, autosomal recessive and polygenic.
A nonsense mutation of the MASS1 gene is reported in a family with febrile and afebrile seizures, from University of Tsukuba, Ibaraki, Japan (Nakayama J et al. Ann Neurol Nov 2002;52:654-657).

ABSENCE EPILEPSY AND PAROXYSMAL DYSKINESIA
Six patients aged 6 to 27 years (mean, 14 years) with childhood absence epilepsy and paroxysmal dyskinesia (PD), identified at five European centers participating in a study group, are reported from Great Ormond Street Hospital, London, UK. The onset of absence seizures was early, at a mean age of 16 months (range, 3 months to 3 years 6 months), and seizures remitted between age 8 and 13 years. The types of associated PD included paroxysmal kinesigenic dyskinesia (1 patient), paroxysmal exercise-induced dystonia (3 patients), and paroxysmal tonic upgaze (two siblings). Apart from the siblings with tonic upgaze which had an earlier onset, PD developed after the onset of absence seizures, and continued after seizures had remitted. PD improved with increasing age and was not severely disabling. Seizures and PD were idiopathic and were thought to be genetic. Seizures were accompanied by a characteristic 3 Hz spike-and-wave EEG and they responded to ethosuximide. (Guerrini R, Sanchez-Carpintero R, Deonna T Pediatric Neurology Briefs 2002