Clinical features of corticobasal degeneration
Onset between 45 and 75 years old, most commonly in 50s and 60s
Insidious onset and gradual progression
Early cognitive impairment, often at presentation
• Executive impairment
• Visuospatial impairment
Language abnormalities
• May occur apart from other cognitive impairment
Motor abnormalities
• Parkinsonism, usually asymmetric
• Dystonia, usually asymmetric
• Tremor
• Myoclonus
• Gait abnormalities, usually with late falls
Cortical abnormalities (other than impaired cognition)
• Apraxia
• Alien limb phenomena
• Cortical sensory loss
Absence of response to dopaminergic therapy
Office of Rare Diseases
neuropathological criteria for the diagnosis of corticobasal degeneration
Core features
Neuronal and glial lesions
with tau pathology demonstrated by Gallyas stain or tau immunostaining
• Thread-like lesions and
astrocytic plaques in gray and white matter
• Prominent tau
immunoreactivity in cell processes
Caudate and putamen
involvement with tau-immunoreactive lesions
Moderate to marked neuronal
loss in the substantia nigra
Cortical neuronal loss and
astrogliosis
• Most cortical pathology is
in the superior frontal, superior parietal, and pre- and post-central gyri
Supportive features
Gross findings
• Narrowing of cortical gyri,
most prominently in parasagittal regions, often peri-Rolandic
• Cortical atrophy,
particularly in the posterior frontal lobe
• Cortical spongiosis
• Thinning of the corpus
callosum
• Flattening of the caudate head
and small thalamic volume
• Pale substantia nigra
Swollen, “achromatic,” or
“ballooned” neurons
Neurofibrillary lesions in
brainstem monoaminergic nuclei (e.g. locus ceruleus and substantia nigra) (previously
called “corticobasal bodies”)
Oligodendroglial tau-positive
argyrophilic inclusions, sometimes named “coiled bodies”
Sparing of hippocampus and
parahippocampal gyrus
Exclusion criteria*
Senile plaques and
Alzheimer-type neurofi brillary tangles
Classic Pick’s bodies with
sharply defined margins
Lewy bodies, α-synuclein
pathology
Ubiquitin immunoreactivity
Infarcts, hemorrhages, and/or
focal ischemic damage
