Abstract
Neuropathological data from the autopsy of a 5œ year-old female patient with the Wiedemann-Rautenstrauch
or neonatal progeroid syndrome are reported.
Extensive demyelination is found in the central nervous system with occasionally
a tigroid pattern and with large amounts of neutral fats and intermediate debris of
myelin breakdown in macrophages. These lesions are characteristic of pure sudanophilic
leucodystrophy.
Features differentiating the findings from those in Pelizaeus-Merzbacher disease
and in other disorders associated with sudanophilic leucodystrophy with various special
characteristics are presented and discussed.
Key words
Wiedemann-Rautenstrauch syndrome - Neonatal progeroid syndrome - Sudanophilic leucodystrophy
- Demyelination - Neutral fats
