Dystonic storm: a practical clinical and video review

Table 2 List of scenarios for dystonic storm

Primary dystonia
	▪ DYT1 generalized dystonia [20, 21, 26]
	▪ Primary generalized DYT1-negative dystonia [1]
Structural brain injury
	▪ Cerebral palsy [15, 22]
	▪ Traumatic brain injury [12]
Metabolic disorders affecting subcortical areas
	▪ Mitochondrial disorders including Leigh’s syndrome [6]
	▪ Glutaric aciduria type I [44]
	▪ Wilson’s disease [13,14,15]
Progressive heredodegenerative dystonia
	▪ Pantothenate kinase-associated neurodegeneration [15, 31, 34, 40, 45, 46]
	▪ Neuroacanthocytosis [1]
	▪ Neuronal ceroid lipofuscinosis (Batten’s disease) [41]
Miscellaneous
	▪ Genetic syndromes e.g. ARX syndrome [47], megalencephalic leukoencephalopathy with subcortical cysts (MLC) [10], SOX-2 anophthalmia syndrome [48]
	▪ Tardive dystonia [30, 35]
	▪ Post-traumatic dystonia [1]
	▪ Ataxia-telangiectasia [49]
	▪ Familial idiopathic hypoparathyroidism [50]

This table demonstrates scenarios where dystonic storm emerges from. Of note, the scenarios represent dystonic disorders at baseline, rather than triggering factors (e.g. withdrawal from medications such as baclofen or deep brain stimulation battery failure) which are not included here

ISSN: 2054-7072