Claustrum sign in a child with refractory status epilepticus after febrile illness: why does it happen?

Excerpt

A 6-year-old female patient with normal psychomotor development was admitted at our hospital with status epilepticus. The patient had been suffering from headache, anorexia and fever in the preceding week. On the day of admission, the patient developed focal tonic–clonic movements in the left superior limb that responded to intravenous diazepam. However, the patient did not recover to normal mental status. Therapy included other anti-epileptic drugs (valproate, phenytoin and levetiracetam), steroids, acyclovir and immunoglobulins. After the second day, these focal tonic–clonic episodes decreased in frequency and there was recovery to normal mental status. Electroencephalography (EEG) at days 6 and 15 revealed occipital intermittent rhythmic delta activity. Blood and cerebrospinal fluid studies did not present abnormalities compatible with inflammatory, infectious or autoimmune disease. Brain magnetic resonance imaging (MRI) at day 15 revealed bilateral claustrum hyperintensity on T2 and FLAIR, hypointensity on T1 inversion recovery (IR), no restriction on diffusion-weighted image (DWI) and no contrast enhancement (Fig. 1). The patient was asymptomatic since day 15 and was discharged at day 21 under levetiracetam, steroids and immunoglobulins. She remained seizure free in the next 6 months of follow-up. At 3 months, EEG was normal and brain MRI showed substantial reduction of the T2 and FLAIR hyperintense signal abnormalities in the claustrum (Fig. 2).

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