Acute phase of hemiconvulsion-hemiplegia epilepsy syndrome

Case contributed by Dr René Pfleger


Febrile episode, accompanied by prolonged unilateral seizures with status epilepticus and subsequent unilateral palsy without resolution. Initial partial status included the face.

Patient Data

Age: 18 months

Non-contrast CT head


Non-contrast CT performed acutely reveals no abnormalities. 
There is preservation of grey white matter interface. There is no dense MCA (MCA clot or dot sign), there is no evidence of swelling or hemorrhage. 

Conjugate eye deviation to the left is noted.

Non-contrast MR head with TOF MRA, performed 2 days after CT, 3 days efter seizures.


Sulcal effacement of the left hemisphere with slight grey-white dedifferentiation on T1WI, sparing the deep nuclei. Associated diffuse panhemispheric hyperintense left white matter changes on DWI with signal loss on ADC and slightly increased signal on T2WI/FLAIR, indicating diffusion restriction consistent with cytotoxic edema. No significant mass effect. The affected areas are independent of vascular territories and there is striking sparing of the deep nuclei including the basal ganglia. No cerebellar involvement.
No gross or petechial intracranial hemorrhages. No vessel or flow abnormalities on TOF angiography, nor evidence of dissection. No focal abnormalities or atrophy, including contralateral cerebellar atrophy.


Patient history, clinical picture and imaging findings suggest hemiconvulsion-hemiplegia-epilepsy syndrome in the acute phase.

Case Discussion

Patient history and MR findings are in keeping with acute phase of hemiconvulsion-hemiplegia epilepsy syndrome.

Aggressive anticonvulsive therapy is important to prevent/milden developing hemiatrophy and permanent epilepsy.

Possible development of focal seizures refractory to medical therapy may necessitate surgery.

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