Dados do Trabalho

Título

STATUS DYSTONICUS IN A PATIENT WITH NEUROTUBERCULOSIS: A CASE REPORT.

Apresentação do caso único

Female patient, 7 months old, 36 weeks premature, with a history of developmental delay, hospitalized with miliary tuberculosis and neurotuberculosis. On the second day in hospital, she had a generalized tonic-clonic seizure, controlled by phenobarbital and phenytoin. She received antibiotic therapy due to a secondary infection and started an anti-tuberculosis regimen, as well as continuous sedation for three days with midazolam and fentanyl. After 22 days in hospital, she developed generalized dystonia, an opisthotonus position and intense crying. Therefore, her initial CPK level was 1562 U/L, characterizing dystonic status. She also received hyperhydration, continuous clonidine and optimization of the medications for dystonia: baclofen, biperiden, trihexyphenidyl and clonazepam. After 8 days, her dystonia improved and her CPK dropped to 1023 U/L, with preserved kidney function and no rhabdomyolysis. After 20 days of treatment, her CPK level of 32U/L had normalized, with no other complications.

Discussão

Dystonic status, also called dystonic storm, is a rare and underdiagnosed condition, considered a neurological emergency due to its potential lethality. It is also characterized by a progressive worsening of dystonia and a significant increase in CPK (>1000 U/L). It can occur in children and adults, secondary to intercurrent illness or infections. In children with secondary dystonia, the most common cause is cerebral palsy. In some cases, it evolves with severe complications such as rhabdomyolysis and respiratory and/or renal failure, or more commonly with bulbar weakness, exhaustion, pain and metabolic disorders. Acute treatment requires continuous sedation in the UCI, analgesia, hyperhydration and specific dystonia medications.

Comentários finais

It is essential to recognize dystonic status as a differential diagnosis of seizures in critically ill patients, in order to institute immediate treatment and prevent complications.

Referências

ALLEN, Nicholas M; LIN, Jean-Pierre; LYNCH, Tim; KING, Mary D. Status dystonicus: a practice guide. Developmental medicine & child neurology, [S. l.], p. 1-8, 7 out. 2013.

CENTENO-ARISPE, José J et al . Status distónico, a propósito de dos casos. Acta méd. Peru, Lima , v. 34, n. 2, p. 132-135, abr. 2017 . Disponible en . acesso em 4 jul. 2024.

Status distônico: Uma emergencia neurológica. Sociedade de Pediatria de São Paulo , [S. l.], p. 1, 11 out. 2023. Disponível em: https://www.spsp.org.br/anais-do-congresso/status-distonico-uma-emergencia-neurologica/. Acesso em: 8 jul. 2024.

JANKOVIC, Joseph. Hyperkinetic movement disorders in children. Uptodate, [S. l.], p. 1-33, 9 jan. 2024. Disponível em: www.uptodate.com. Acesso em: 4 jul. 2024.

DEIK, Andres; COMELLA, Cynthia. Etiology, clinical features,and diagnostic evaluation of dystonia. Uptodate, [S. l.], p. 1-37, 9 maio 2023. Disponível em: www.uptodate.com. Acesso em: 4 jul. 2024.

Palavras Chave

movement disorders; dystonia; Status dystonicus