. 2013 Jun 4;28(10):1325–1329. doi: 10.1002/mds.25490

Table 1.

The differential diagnosis of dystonic opisthotonus (incomplete list)

Differential diagnosis of dystonic opisthotonus	Further clues for the differential diagnosis
Drug-induced dystonia²³^,²⁸^,⁵³	Often also retrocollis
	History of drug consume
NBIAs	Oromandibular dystonia
	Parkinsonism
	Iron accumulation in brain MRI
Glutaric aciduria³⁶^,⁴⁰	Consanguinity
Maple syrup urine disease³⁵^,³⁷^,³⁸	Perinatal history
Maple syrup urine disease³⁵^,³⁷^,³⁸	Very early age at onset
	Possibly delayed motor milestones
	Truncal hypotonia
	Encephalopathic crisis
	Intermittent painful dystonic posturing exacerbated by fever, infections
Wilson's disease	Kayser-Fleischer rings
	Oromandibular dystonia
	Wing-beating tremor
Lesch-Nyhan⁵⁴	Oromandibular dystonia
	Self-injurious behavior
	Intellectual disability
Dopa-responsive dystonia (DYT5)³³	Levodopa response
Dopa-responsive dystonia (DYT5)³³	Perinatal history
	Delayed motor milestones
Tyrosine hydroxylase deficiency	Oculogyric crisis
Aromatic L-amino acid decarboxylase deficiency	Perinatal history
Aromatic L-amino acid decarboxylase deficiency	Delayed motor milestones
Sepiapterin reductase deficiency⁵⁵
Primary extensor truncal dystonia²⁶	No further signs
Others (eg, meningitis, encephalitis, etc)	Dependent on the underlying cause

MRI, magnetic resonance imaging; NBIAs, neurodegeneration with brain iron accumulation syndromes.