Essential Features: Myopathic Versus Neurogenic Changes Definition Myopathic changes are seen in disorders that primarily affect muscle Neurogenic changes in skeletal muscle result from disorders of innervation: peripheral nerve, motor neuron disease, or neuromuscular junction Clinical Features Epidemiology Depends on…
Basic Reactions in Peripheral Nerve Disorders Definition Pathologic changes seen in any component of the peripheral nerve Clinical Features Epidemiology Peripheral nerve disorders are common conditions affecting approximately 2.4% of the population Prevalence increases with age to up to 8%…
Neuronal Storage Diseases Definition Autosomal recessive lysosomal storage diseases primarily affecting neurons/gray matter (“poliodystrophies”) (the leukodystrophies are considered in the next chapter) Representative examples of gangliosidoses are presented here Clinical Features Epidemiology Specific enzyme deficiencies that cause impaired removal of…
Wernicke-Korsakoff Disease Definition A complex of neurologic deficits associated with vitamin B 1 (thiamine) deficiency Clinical Features Epidemiology Prevalence: 1% to 3% Age: onset ranges from 30 to 70 years Thiamine Dietary sources: pork and whole grains Absorbed from duodenum,…
Toxic Leukoencephalopathy Definition General term used here to describe a white matter demyelinating/destructive process caused by innumerable therapeutic and toxic agents Clinical Features Epidemiology May affect all ages, overall prevalence unknown Abused substances Inhalants (glue, spray paint, etc.) Cocaine, heroin,…
Definition Acute, inflammatory demyelinating disease, which presents as a solitary, large (>2 cm) focus of demyelination within a cerebral hemisphere with associated edema that may simulate neoplasm or abscess; synonyms include tumefactive multiple sclerosis lesions, tumor-like demyelinating lesions, and demyelinating pseudotumors…
Definition Hyperacute (fulminant) form of acute disseminated encephalomyelitis with perivascular hemorrhages, myelin destruction, necrotizing venulitis, and scattered ischemic lesions throughout the CNS Clinical Features Epidemiology Rare, sporadic disease Occurs in all ages but more common in childhood No gender predilection…
Definition Acute demyelinating inflammatory disorder characterized by perivascular (especially perivenular) myelin loss that is widely distributed throughout the brain and spinal cord and only affects the central nervous system (CNS); a monophasic disorder; occurs most often in children Clinical Features…
Multiple Sclerosis Definition Autoimmune, chronic inflammatory, demyelinating disease of the central nervous system; intermittent episodic neurologic impairments of varying severity caused by multiple demyelinating lesions, often of different ages Key pathologic features: Perivenular inflammation with primary myelin loss and relative…
Definition Vascular dementias are a group of chronic progressive dementing disorders resulting from hypoperfusive, occlusive, or hemorrhagic cortical or subcortical infarcts Binswanger disease (subcortical vascular dementia) refers to a widespread small-vessel disease affecting the deep white matter usually associated with…