Subacute sclerosing panencephalitis

Aims: SSPE is known as a late onset complication after measles infections with a lethality of 100%. The frequency of occurrence seems to be affected by the age at the time of infection.

Methods: A 13-year old girl of Kurdish origin, suffered from measles in Turkey at the age of two. Vaccination against measles twice after migration to Germany.

6–8 months before clinical admission decreasing general performance at school, disturbance of memory, lack of concentration. Decline of vision and imprecise articulation for 4 months. Several days before admission to hospital polytope myoclonias, hit her head on the left side while falling 3 days ago.

Physical examination: clear state of awareness, opisthotone posture, atactic gait pattern, tendency to fall backwards, polytope myoclonias, slight dysaesthesia at the inner thighs.

Initially laboratory diagnostics without pathological findings, including inflammatory parameters, metabolic and collagenosis diagnostics. No indication of neurotropic pathogens.

First EEG with typical epileptic pattern without a focus, suspicion of Radermecker-complexes and a MRI brain scan without pathological findings. During therapy with valproic acid progress of symptoms.

Radermecker-complexes in a repeated EEG. Serological detection of elevated measles specific IgG (290000mlU/ml), negative measles specific IgM. Pleocytosis and increased liquor protein and albumin ratio shown in the diagnostics of the cerebrospinal fluid; measles IgG-antibodies positive, measles IgM-antibodies negativ. No detection of measles by cerebrospinal fluid-PCR.

Results: The clinical symptoms, Radermecker-complexes in the EEG and the infectiologic and immunologic results lead to the diagnosis of SSPE stadium 2. During the current treatment with alpha-interferon intrathecally, Topiramat and Clobazepam, Dimepranolacedoben and Ribavirin further progress of symptoms can be seen so far.

Conclusion: In the presence of appropriate symptoms in times of still existing deficient measles vaccination status and many patients with migration backgrounds, SSPE needs to be considered as a differential diagnosis.

Subacute sclerosing panencephalitis