Acute phase of hemiconvulsion-hemiplegia epilepsy syndrome
Citation, DOI and case data
Febrile episode, accompanied by prolonged unilateral seizures with status epilepticus and subsequent unilateral palsy without resolution. Initial partial status included the face.
2 case question available
Non-contrast CT head
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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.
3 case question available
Non-contrast MR head with TOF MRA, performed 2 days after CT, 3 days efter seizures.
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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.
4 case question available
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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- 4. Singer OC, Humpich MC, Laufs H et-al. Conjugate eye deviation in acute stroke: incidence, hemispheric asymmetry, and lesion pattern. Stroke. 2006;37 (11): 2726-32. doi:10.1161/01.STR.0000244809.67376.10 - Pubmed citation
- 5. Jeon YW, Kim SH, Lee JY et-al. Dynamic CT perfusion imaging for the detection of crossed cerebellar diaschisis in acute ischemic stroke. Korean J Radiol. 2012;13 (1): 12-9. doi:10.3348/kjr.2012.13.1.12 - Free text at pubmed - Pubmed citation
- 6. Förster A, Kerl HU, Goerlitz J et-al. Crossed cerebellar diaschisis in acute isolated thalamic infarction detected by dynamic susceptibility contrast perfusion MRI. PLoS ONE. 2014;9 (2): e88044. doi:10.1371/journal.pone.0088044 - Free text at pubmed - Pubmed citation
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