Status Dystonicus: A Rare Manifestation of Cerebral Palsy

Status Dystonicus (SD) is characterized by generalized muscle contractions in dystonic patients. We report 5 cases of SD, two of which in patients with dystonic cerebral palsy, one in a patient with primary segmental dystonia, one in a patient with Hallervorden-Spatz syndrome and one in a patient with Wilson's disease (WD). Three patients were admitted to an intensive care unit and treated with propofol and midazolam, and two were submitted to neurosurgical procedures (bilateral pallidotomy and bilateral pallidal deep brain stimulation). Triggering factors were identified in three patients as follows: infection, stress-induced and zinc therapy for WD. On follow-up, two patients presented with significant improvement of dystonia, whereas the other three cases the clinical picture ultimately returned to baseline pre-SD condition.

Download Full-text

Status dystonicus: a diagnosis delayed

BMJ Case Reports ◽

10.1136/bcr-2018-226531 ◽

2018 ◽

pp. bcr-2018-226531

Author(s):

Muhammad Fahad Arshad ◽

Ehtasham Ahmad ◽

Aiyappa Nanjappa Biddanda ◽

Muhammad Sharif

Keyword(s):

Cerebral Palsy ◽

General Hospital ◽

Functional State ◽

Delayed Diagnosis ◽

District General Hospital ◽

Full Recovery ◽

Athetoid Cerebral Palsy ◽

Status Dystonicus

Status dystonicus, also known as the dystonic storm or dystonic crisis, is rare but may prove fatal due to respiratory and bulbar complications. In adults, the condition is rare and possibly under-reported. The lack of awareness of this condition among emergency and acute physicians may lead to an incorrect or delayed diagnosis, which should be avoided. We report a case of a 23-year-old man with athetoid cerebral palsy who presented to a district general hospital with uncontrolled dystonic movements, which were diagnosed as status dystonicus. This was successfully treated with intravenous clonidine, with full recovery returning to baseline functional state.

Download Full-text

Prevalence of Speech Problems and the Use of Augmentative and Alternative Communication in Children With Cerebral Palsy: A Registry-Based Study in Norway

Perspectives on Augmentative and Alternative Communication ◽

10.1044/aac19.1.12 ◽

2010 ◽

Vol 19 (1) ◽

pp. 12-20 ◽

Cited By ~ 29

Author(s):

Guro Andersen ◽

Tone R. Mjøen ◽

Torstein Vik

Keyword(s):

Cerebral Palsy ◽

Motor Function ◽

Gestational Age ◽

Augmentative And Alternative Communication ◽

Alternative Communication ◽

Gross Motor ◽

Gross Motor Function ◽

Children With Cerebral Palsy ◽

Communicative Abilities ◽

Normal Speech

Abstract This study describes the prevalence of speech problems and the use of augmentative and alternative communication (AAC) in children with cerebral palsy (CP) in Norway. Information on the communicative abilities of 564 children with CP born 1996–2003, recorded in the Norwegian CP Registry, was collected. A total of 270 children (48%) had normal speech, 90 (16%) had slightly indistinct speech, 52 (9%) had indistinct speech, 35 (6%) had very indistinct speech, 110 children (19%) had no speech, and 7 (1%) were unknown. Speech problems were most common in children with dyskinetic CP (92 %), in children with the most severe gross motor function impairments and among children being totally dependent on assistance in feeding or tube-fed children. A higher proportion of children born at term had speech problems when compared with children born before 32 weeks of gestational age 32 (p > 0.001). Among the 197 children with speech problems only, 106 (54%) used AAC in some form. Approximately 20% of children had no verbal speech, whereas ~15% had significant speech problems. Among children with either significant speech problems or no speech, only 54% used AAC in any form.

Download Full-text