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…
Neurodegeneration with Brain Iron Accumulation Type 1 (NBIA 1) Definition Progressive neurodegenerative disease characterized clinically by extrapyramidal symptoms with dementia and by the presence of iron accumulation and axonal spheroids within the globus pallidus and substantia nigra histologically Previously called:…
Huntington Disease Definition Autosomal dominant neurodegenerative disease characterized clinically by choreiform movements, psychiatric symptoms, and dementia and genetically by expansion of the trinucleotide (CAG) repeat in the HD gene Clinical Features Epidemiology Age range: 2 to 85 years (mean age:…
Definition Progressive neuromuscular disease characterized by degeneration of upper and lower motor neurons resulting in progressive skeletal muscle wasting and weakness leading to respiratory failure and death; ALS subtypes: primary lateral sclerosis (mainly upper motor disease) and progressive muscular atrophy…
Parkinson's Disease Definition Progressive neurodegenerative disease characterized clinically by movement abnormalities including the triad of resting tremor, bradykinesia, and cogwheel rigidity with histologic findings of pigmented neuron loss within the substantia nigra and elsewhere Presence of α-synuclein immunoreactive Lewy bodies…
Definition Progressive neurodegenerative disorders presenting with behavioral or language abnormalities FTLD with motor neuron disease (FTLD-MND)—cognitive dysfunction with the following: Clinical features of motor neuron disease or Ubiquitin-immunoreactive, tau-negative inclusion bodies within motor neurons histologically (with or without symptoms of…
Corticobasal Degeneration (Rebeiz Disease) Definition Sporadic neurodegenerative disease characterized clinically by progressive asymmetrical involuntary movements, akinetic-rigid parkinsonism, symptoms of upper motor neuron dysfunction (dysarthria, aphasia), and variable dementia; pathologic findings are atrophy of superior frontal lobes, rostral parietal lobes, basal…