Pathology

Grossly, the cerebellar cortex is atrophic, the basis of the pons and middle cerebellar peduncles are shrunken, and the inferior olives are flattened (see Fig. 5.35). The substantia nigra and locus ceruleus are discolored, and the putamen is atrophic with grayish discoloration. Histologically, widespread degeneration of the neurons in three anatomic systems and the presence of distinct glial and neuronal cytoplasmic inclusions are the defining features.

Neuronal degeneration occurs in the olivopontocer-ebellar system, the striatonigral system, and the auto-nomic nervous system. Extensive degeneration of the Purkinje cells characterizes the cerebellar pathology. The basket cells are empty, and axonal spheroids (torpedoes) of the degenerated Purkinje cells are commonly found in the granular layer. A prominent Berg-

FIGURE 5.35

Multiple system atrophy. A 62-year-old man presented with a history of gait imbalance that began at age 58. At 60 years of age, he had lost sensation of the urinary bladder and gradually became incontinent. Impotence and constipation had begun a few years earlier. The incoordination gradually involved first the lower, then the upper extremities. His speech became slurred. The unsteadiness of gait progressed and, by 62 years of age, he was confined to a wheelchair. No similar disease had occurred in his family. On examination, his mentation appeared normal. His speech was severely dysarthric. He had severe cerebellar ataxia in all extremities and nystagmus on lateral gaze. Reflexes were symmetric, with plantar flexor reflexes. Muscle strength and sensation were normal. He was unable to walk and could stand only a few minutes with bilateral assistance. On changing from a supine to a sitting position, his blood pressure consistently dropped by 20 mm Hg while his pulse remained unchanged. A. MRI shows atrophy of the cerebellum and enlargement of the fourth ventricle. B. The cerebellar cortex is severely atrophic, the pontine basis is reduced in volume, and the inferior olives are flattened.

FIGURE 5.35

Multiple system atrophy. A 62-year-old man presented with a history of gait imbalance that began at age 58. At 60 years of age, he had lost sensation of the urinary bladder and gradually became incontinent. Impotence and constipation had begun a few years earlier. The incoordination gradually involved first the lower, then the upper extremities. His speech became slurred. The unsteadiness of gait progressed and, by 62 years of age, he was confined to a wheelchair. No similar disease had occurred in his family. On examination, his mentation appeared normal. His speech was severely dysarthric. He had severe cerebellar ataxia in all extremities and nystagmus on lateral gaze. Reflexes were symmetric, with plantar flexor reflexes. Muscle strength and sensation were normal. He was unable to walk and could stand only a few minutes with bilateral assistance. On changing from a supine to a sitting position, his blood pressure consistently dropped by 20 mm Hg while his pulse remained unchanged. A. MRI shows atrophy of the cerebellum and enlargement of the fourth ventricle. B. The cerebellar cortex is severely atrophic, the pontine basis is reduced in volume, and the inferior olives are flattened.

FIGURE 5.36

Multiple system atrophy. Cerebellum shows A. severe Purkinje cell losses and prominent Bergmann astrocytosis (HE), B. Axonal spheroid (torpedo) of the degenerated Purkinje cells (Bodian stain). C. Argyrophilic (Gallyas stain). D. Ubiquitin-positive cytoplasmic inclusions in oligodendrocytes of the white matter (immunostain).

FIGURE 5.36

Multiple system atrophy. Cerebellum shows A. severe Purkinje cell losses and prominent Bergmann astrocytosis (HE), B. Axonal spheroid (torpedo) of the degenerated Purkinje cells (Bodian stain). C. Argyrophilic (Gallyas stain). D. Ubiquitin-positive cytoplasmic inclusions in oligodendrocytes of the white matter (immunostain).

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mann astrocytic layer marks the site of Purkinje cell losses (Fig. 5.36). The neurons in the griseum pontis degenerate, and their cerebellar projections—the transverse pontine fibers and middle cerebellar peduncles—

are demyelinated (Fig. 5.37). The medulla shows degeneration of the neurons in the inferior olives and variable myelin losses in the inferior cerebellar peduncles (Fig. 5.38).

FIGURE 5.37

Multiple system atrophy. A. Pons shows extensive neuronal losses in griseum pontis compared with (B) normal control (HE), (C) demyelination of transverse pontine fibers and middle cerebellar brachia (LFB-CV); (D) astrofibrosis at the site of transverse pontine fibers and middle cerebellar brachia (Holzer stain); (E) argyrophilic conical cytoplasmic inclusions in oligodendrocytes (Gallyas stain); and (F) a-Synuclein-immunoreactivity of inclusions (immunostain).

FIGURE 5.37

Multiple system atrophy. A. Pons shows extensive neuronal losses in griseum pontis compared with (B) normal control (HE), (C) demyelination of transverse pontine fibers and middle cerebellar brachia (LFB-CV); (D) astrofibrosis at the site of transverse pontine fibers and middle cerebellar brachia (Holzer stain); (E) argyrophilic conical cytoplasmic inclusions in oligodendrocytes (Gallyas stain); and (F) a-Synuclein-immunoreactivity of inclusions (immunostain).

FIGURE 5.38

Multiple system atrophy. Medulla shows (A) loss of myelin in olivocerebellar fibers and inferior cerebellar brachia (LFB-CV), (B) neuronal losses in the inferior olivary nuclei (LFB-CV), and (C) dense astrogliosis (Holzer stain).

Neuronal losses and gliosis are prominent in the putamen and variably severe in the substantia nigra (Fig. 5.39). Changes in the autonomic nervous system consist of losses of sympathetic neurons in the interme-diolateral columns of the thoracic and lumbar spinal cord and losses of parasympathetic neurons in the sacral cord (Fig. 5.40).

Cytoplasmic inclusions in the oligodendrocytes and neurons (Papp-Lantos inclusions), are the hallmark lesions of MSA (Table 5.19). They are argyrophilic, best demonstrated using sensitive silver stain (Gallyas method) and appear as round, oval, conical, flame- or crescent-shaped structures. The inclusions are distinguished by immunoreactivity for a-synuclein and ubiquitin (see Figs. 5.36 and 5.37). They commonly occur in the frontal motor cortex and adjacent white matter, basal ganglia, brainstem reticular formation, pontine basis, cerebellar white matter, and middle cerebellar brachia.

Multiple System Atrophy Histology

FIGURE 5.39

Multiple system atrophy. Midbrain shows moderate losses of pigmented neurons of the substantia nigra (HE).

FIGURE 5.39

Multiple system atrophy. Midbrain shows moderate losses of pigmented neurons of the substantia nigra (HE).

FIGURE 5.40

Multiple system atrophy. Thoracic spinal cord shows loss of sympathetic neurons in the intermediolateral column (LFB-CV).

FIGURE 5.40

Multiple system atrophy. Thoracic spinal cord shows loss of sympathetic neurons in the intermediolateral column (LFB-CV).

Constipation Prescription

Constipation Prescription

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