Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria 11, 20133, Milan, Italy.
Published: July 2018
AI Article Synopsis
Article Abstract
Actual indications for surgery in tuberculosis are limited to obtaining a diagnosis, acquiring tissue for culture studies, treating hydrocephalus, aspiring a brain abscess, and reducing intracranial pressure in patients with multiple tuberculomas. Tuberculosis-related movement disorders are usually treated pharmacologically. We report on a child affected by post-tubercular generalized dystonia, who progressed to status dystonicus (SD) and underwent stereotactic bilateral pallidotomy. After surgery, SD resolved, and drugs were rapidly tapered. The successful reversal of SD and the motor improvement observed in our patient demonstrate the safety, feasibility, and clinical efficacy of pallidotomy in post-tuberculous-meningoencephalitis dystonia and SD.
Department of Human Neurosciences, Sapienza University of Rome, Viale dell'Università 30, 00185 Rome, Italy; IRCCS Neuromed Institute, Via Atinense 18, 86077 Pozzilli, (IS), Italy. Electronic address:
- Malignant syndromes are serious medical conditions that develop rapidly and can lead to life-threatening complications if not treated promptly.
- These syndromes share similar clinical features and mechanisms, affecting various neurotransmitter systems, which highlight the need for urgent medical attention.
- Understanding the clinical aspects and underlying causes of malignant syndromes is crucial for effective management and reducing the risks of severe health consequences.
Department of Neurology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China; Laboratory for Clinical Medicine, Capital Medical University, China. Electronic address:
The study aimed to evaluate the clinical features and underlying causes of KMT2B variants in Chinese children with dystonia or developmental delay, analyzing data from 27 patients.
A significant majority (26 patients) had various forms of dystonia, often worsened by factors like fever, and some also experienced additional movement disorders and developmental delays.
The research found 26 unique pathogenic KMT2B variants, with many linked to mitochondrial dysfunction, impacting cellular respiration and mitochondrial structure in patient fibroblasts.
Dystonia is a common disorder of movement and tone, characterised by sustained or intermittent muscle contractions causing abnormal movements, postures or both. Children and young people with dystonia can experience episodes of acute worsening tone, which require prompt treatment. When most severe, dystonia may become life-threatening-a state called 'status dystonicus'.
Currently nearly one-quarter of admissions to pediatric intensive care units (PICUs) worldwide are for neurocritical care diagnoses that are associated with significant morbidity and mortality. Pediatric neurocritical care is a rapidly evolving field with unique challenges due to not only age-related responses to primary neurologic insults and their treatments but also the rarity of pediatric neurocritical care conditions at any given institution. The structure of pediatric neurocritical care services therefore is most commonly a collaborative model where critical care medicine physicians coordinate care and are supported by a multidisciplinary team of pediatric subspecialists, including neurologists.
Functional Neurosurgery Department, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing, China; Laboratory for Clinical Medicine, Capital Medical University, Beijing, China. Electronic address:
Background: Pantothenate kinase-associated neurodegeneration (PKAN) is a type of inherited metabolic disorder caused by mutation in the PANK2 gene. The metabolic disorder mainly affects the basal ganglia region and eventually manifests as dystonia. For patients of dystonia, their dystonic symptom may progress to life-threatening emergency--status dystonicus.
