[Stroke in children and adolescents]. / Schlaganfall bei Kindern und Jugendlichen.

The occurrence of a stroke in children and adolescents constitutes a rare, critical event that is associated with substantial morbidity and mortality. In addition to the individual suffering for the young patient and the medical burden for the affected family, a stroke is also associated with high follow-up costs for the health system because of the necessary long-term rehabilitative treatment. Establishing an early and prompt diagnosis is of great therapeutic importance. Because of the rarity of the illness and the plethora of clinical manifestations, diagnosis is often delayed. The most frequent clinical presentation is an acute focal-neurological deficit, usually in the form of hemiparesis, but headache, seizures or alteration of consciousness may also be seen. Nowadays, the prompt performance of diffusion-weighted, blood-sensitive magnetic resonance imaging (MRI) constitutes the gold standard. The most relevant risk factors for the occurrence of a stroke in this age cohort are vasculopathies, infections, pathological cardiac conditions or coagulopathies. Recurrence of stroke is dependent on the underlying risk factors. In a substantial percentage of patients, residual neurological deficits are seen.Owing to a lack of randomized controlled trials in children and adolescents with stroke, the optimal treatment approach is still under debate. In addition to anti-platelet medication and heparinization, systematic intravenous thrombolysis and endovascular thrombectomy are other potentially effective treatment options. The long-term prognosis in children is dependent on establishing a correct, early diagnosis.

Familial Mediterranean fever in Germany: clinical presentation and amyloidosis risk.

OBJECTIVE: To characterize patients with familial Mediterranean fever (FMF) with and without AA amyloidosis living in Germany. METHOD: Clinical and genetic data from 64 FMF patients were analysed for amyloidosis risk factors. RESULTS: Fifty-five patients (85%) were of Turkish or Armenian origin. Thirty-one patients (48%) developed FMF symptoms before the age of 16 years. Sixteen patients (26%) became symptomatic after age 20. Symptoms reported were peritonitis (95%), fever (78%), pleuritis (59%), arthralgia (60%), arthritis (32%), erysipelas-like erythema (23%), and vasculitis (8%). FMF diagnosis was delayed for a median of 8.0 years. Genetic analysis confirmed M694V as the most prevalent Mediterranean fever (MEFV) gene mutation in 46 out of 59 patients (78%). M694V homozygosity was associated with an earlier FMF onset (median age 5.5 years, p = 0.0001) and a higher prevalence of peritonitis (p = 0.007) and pleuritis (p = 0.0007) compared to patients without an M694V mutation. AA amyloidosis was detected in 16 patients (25%) at a median age of 36.5 years and tended to be associated with a higher age at disease onset (p = 0.062) and a higher FMF activity score (p = 0.093). AA amyloidosis was significantly associated with a higher age at FMF diagnosis (p = 0.0022). CONCLUSIONS: Clinical symptoms of FMF-affected migrants living in Germany resemble those observed in their home country. In particular, patients with an onset of FMF symptoms after age 20 and a later FMF diagnosis have a high risk of AA amyloidosis. Symptomatic patients who originate from countries with a higher FMF prevalence should be screened for FMF and proteinuria.