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Neuronal ceroid lipofuscinoses Volume 18, supplément 2, September 2016

Figure 1

(A) Otto Christian Stengel; (B) Frederick Eustace Batten (first from the left, bottom row); (C) Walther Spielmeyer; (D) Jan Janský; (E) Max Bielschowsky; (F) Hugo Kufs; (G) Matti Haltia; and (H) Pirkko Santavuori.

Figure 2

Electron microscopy and pathology findings in NCL. (A) Granular osmophilic deposits (GROD) in a conjunctival biopsy from a patient with CLN1 mutations (bar is 500 nm); (B) Fingerprint profiles (FPP) and curvilinear profiles (CLP) in a conjunctival biopsy from a patient with CLN2 mutations (bar is 100 nm). The predominance of curvilinear profiles and the comparative scarcity of fingerprint bodies were consistent with CLN2. Should fingerprint profiles predominate, the biopsy would be more consistent with CLN3; (C) Vacuolated lymphocytes in a patient with CLN3 disease.

Figure 3

Classic EEG findings in CLN2 disease.