Neuropathology of superficial hemosiderosis and neuroferritinopathy

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Neuropathology of superficial hemosiderosis. Gross finding shows diffuse brownish discoloration at the surface of the cerebrum, cerebellum, brainstem and spinal cord. Severe atrophy and necrosis is present in the cerebellum. Extensive deposits of hemosiderin that are well recognized with Berlin blue and ferritin immunohistochemistry are present at the surface and in the superficial parenchyma of the cerebrum, brainstem, cerebellum and spinal cord. Although the pathomechanism of this disease remains unresolved, continuous or recurrent subarachnoid hemorrhage may be important for developing diffuse hemosiderin deposition. Neuroferritinopathy. Intranuclear and intracytoplasmic bodies are seen in glia and subsets of neurons in the central nervous system as well as in extraneural tissue. They are stained by Perls' method for ferric iron. The bodies are immunopositive against antibodies raised against mutated and wild type of ferritin light polypeptide as well as ferritin heavy polypeptide. It is suggested that loss of normal function and gain of toxic function may be crucial for neurodegeneration of neuroferritinopathy. Both diseases could be helpful to understand and clarify the pathomechanism of neurodegeneration associated with iron metabolism.
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