Movement disorder emergencies in childhood

Gabapentin can significantly improve dystonia severity and quality of life in children

“…35 Gabapentin may help reduce the frequency of cases of childhood dystonia presenting as movement disorder emergencies. 36 …”

Dystonic opisthotonus: A “red flag” for neurodegeneration with brain iron accumulation syndromes?

“…Opisthotonus also has been described in neurometabolic disorders (eg, glutaric aciduria, maple syrup urine disease, Lesch‐Nyhan, dopa‐responsive dystonias) (Table ). The very early age at onset, delayed motor milestones, truncal hypotonia, encephalopathic crisis, and intermittent painful dystonic posturing exacerbated by fever or infections are helpful clues to suspect a neurometabolic disease (see Table ) . Conditions that reportedly cause back arching because of different etiologies, such as tetanus, strychnine poisoning, meningitis, and encephalitis, or “arc‐de‐cercle” in psychogenic dystonia are less likely to pose differential diagnostic problems with NBIAs …”

Acute hyperkinetic movement disorders in Italian paediatric emergency departments

“…Consistent with published data,11 13 girls were more often affected (F:M=1.6:1). The remarkable frequency of cardiac involvement highlights the importance of its prompt exclusion in any patient with acute-onset chorea 6. Of note, in 5 out of 36 SC cases, tics were the presentation symptom on admission to pED.…”

“…In fact, differential diagnosis between SC and tic disorders related to streptococcal infections can be difficult, particularly at onset 14. On one hand, many patients with SC also have tics or psychological symptoms6; on the other hand, clinical distinction between chorea and complex motor tics could be difficult in an emergency setting. Consequently, SC should always be considered in children presenting with acute-onset tics.…”