Acute phase of hemiconvulsion hemiplegia syndrome

Case contributed by Dr René Pfleger

Presentation

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

Patient Data

Age: 18 months

Non-contrast CT head

Modality: CT

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.

Modality: MRI

Sulcal effacement of the left hemisphere with slight grey-white dedifferentiation on T1, sparing deep nuclei. Associated diffuse panhemispheric hyperintense left WM-changes on DWI with signal loss on ADC and slightly increased signal on T2FLAIR, 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 deep nuclei including basal ganglia. No cerebellar involvement.
No gross or petechial intracranial hemorrhages. No vessel or flow abnormalities on TOF angiography, no evidence of dissection. No focal abnormalities or atrophy including contralateral cerebellar atrophy.

Conclusion

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

Case Discussion

Patient history and MR-findings are in keeping with acute phase of hemiconvulsion-hemiplegia epilepsy syndrome (HH/HHE).

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

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

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Case Information

rID: 29309
Case created: 15th May 2014
Last edited: 13th Mar 2017
Inclusion in quiz mode: Included

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