Striatonigral Degeneration

Striatonigral degeneration is a slowly progressive degenerative disease of middle age that greatly resembles Parkinson's disease. Rigidity, akinesia, and postural


Progressive supranuclear palsy (PSP). A 65-year-old man presented with a 6-year history of difficulty walking and frequent falls, inability to look up or down, difficulty speaking and swallowing, and declining memory. Family history was not contributory. On examination, he was not able to articulate words, but was able to follow simple commands. The vertical and horizontal eye movements were severely restricted. The muscle tone was increased in the neck, trunk, and extremities, but strength was good. No tremor was noted. He needed assistance to rise from a chair and ambulate. The gait was slow and shuffling. Tendon reflexes were brisk, plantar reflexes were flexor, and the vestibulo-ocular reflex was present. He died at age 66 following a 7-year clinical course. Midbrain shows degeneration of the substantia nigra (HE).


Progressive supranuclear palsy. Pons. A and B. The tegmental fiber tracts are demyelinated due to extensive and severe neuronal degenerations (LFB-CV).


Progressive supranuclear palsy. Medulla. The tegmentum stains faintly due to severe neuronal and myelin losses (LFB-CV).


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Progressive supranuclear palsy. Neuronal inclusions. A. Neurofibrillary tangles are present in the entorhinal cortex (Bodian stain). B. Tau-positive neurons are present in the thalamus (immunostain).


Progressive supranuclear palsy. Glial inclusions. A. Astrocytes display argyrophilic (Gallyas stain) and (B) tau-immunoreactive inclusions (astrocytic plaque; immunostain). C. Oligodendrocytes display argyrophilic coiled bodies (Gallyas stain).

instability are major clinical features. But, unlike Parkinson's disease, it is unresponsive to dopamine therapy. The pathology is characterized by neuronal losses, particularly severe in the striatum and variable in the substantia nigra, which displays no Lewy bodies. Stria-tonigral degeneration is a component of multiple system atrophy.

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