Paroxysmal Sympathetic Hyperactivity

in a Child with Moyamoya Disease

Dhaval Shukla*, Akhil Deepika*,

Madhusudan Reddy #,

Departments of Neurosurgery*, and Neuroanesthesiology#,

NIMHANS, Bangalore

Presentation

• 6-year-old girl

• Right MCA infarct due to moyamoya disease

• Admission mRS 2 (left hemiparesis)

• Underwent bilateral EDAMS

• Seizures with new onset right hemiplegia

• Rapidly deteriorated in sensorium

• E4 VT M4

Paroxysmal Sympathetic Hyperactivity (PSH)

• Fever

• Tachycardia

• Hypertension

• Tachypnea

• Hyperhidrosis

• Posturing

Infection ruled out

DexmedetomidineMetoprololClonidine

Expired on PO day 28

Perkes I, et al. Ann Neurol. 2010Rabinstein AA, et al. Curr Treat Options Neurol. 2008

Discussion

• Causes of PSH

– TBI

• Adults - 8% to 33%

• Children - 12%

– Stroke - 5.4%

• Stroke has not been reported as a cause of PSH

in children

• Our patient had multiple strokes leading to

severe diffuse brain insult

• These could have caused damage to cerebral

autonomic nervous system pathways leading to

wide-spread autonomic dysregulation as seen in

patients with severe diffuse TBIRabinstein AA. Neurol Res. 2007

Krach LE, et al. Neurorehabil Neural Repair. 1997

Deepika A, Reddy M, Shukla D. Paroxysmal sympathetic hyperactivity in a child with moyamoya disease. J NeurosurgAnesthesiol. 2014;26:87-8.