Practical neuropathology of central nervous system for clue to clinical diagnosis

Abstract

Neuropathological diagnosis has been still gold standard for neurodegenerative disorders. Clues of microscopic findings appear on macroscopic pictures. Characteristic macroscopic features are closely related to pathological lesions and distributions, clinical sings, and neuroimaging. Parkinsonism is one of the most frequent and complicated symptoms for clinical diagnosis. Abnormal pallor on substantia nigra is consequent of loss of pigmented neurons. Depigmentation of substantia nigra is essential feature of clinical presentation of Parkinsonism, such as Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, progressive supranulear palsy, and corticobasal degeneration. Differential diagnosis is based on macroscopic appearances of brainstem. Depigmentation of substantia nigra and locus ceruleus without atrophy of pontine base or brainstem tegmentum suggests Lewy body pathology including Parkinson's disease and dementia with Lewy bodies. Multiple system atrophy is consisted of degeneration of olivopontocerebellar system, striatonigral system and autonomic nervous system in various degree. When atrophy of pontine base and cerebellum is distinctive, in addition to depigmentation of substantia nigra and locus ceruleus, differential diagnosis may include multiple system atrophy. While atrophy of midbrain tegmentum and relative preservation of locus ceruleus is clear, progressive supranulear palsy may be contained in differential diagnosis. Therefore, macroscopic findings of central nervous system are potential clues for practical clinical diagnosis.