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Corresponding author at: University of California, San Francisco, Fresno Medical Education Program, 155 N. Fresno St., Fresno, CA 93701, United States.
Division of Neurology, University of California, San Francisco, Fresno Center for Medical Education and Research, 155 N. Fresno St., Fresno, CA 93701, United States
Division of Neurology, University of California, San Francisco, Fresno Center for Medical Education and Research, 155 N. Fresno St., Fresno, CA 93701, United States
A 71 year-old female with thyroid disease presented for rapidly progressive cognitive dysfunction. Three years prior, the patient began to experience difficulty with performing motor tasks and neglecting the right side of her body. Attention and concentration also declined. Gait slowed with shortening of stride length. Memory and ability to name objects remained intact. She had no hallucinations, gaze limitation, dream enactment behavior or myoclonus. She had been placed on donepezil and paroxetine with no improvement.
Neurologic examination revealed frontal release signs, ideomotor apraxia and hypomimia. Intermittent dystonia, rigidity and astereognosis of the right hand were seen. Gait examination revealed retropulsion and a slowed gait. Initial testing was within normal limits, including a basic metabolic panel, complete blood count, vitamin B12, thyroid stimulating hormone, Venereal Disease Research Laboratory test, C-reactive protein and erythrocyte sedimentation rate.
Cerebrospinal fluid (CSF) testing revealed one red blood cell/uL, one white blood cell/uL, protein of 29 mg/dL, glucose of 54. CSF culture, gram stain and 14-3-3 protein were both negative. Further CSF analysis revealed A-beta 42 (734.75 pg/ml), phospho-tau (70.55 pg/ml) and total-tau levels (453.95 pg/ml) with A-beta 42/total-tau index (ATI; 0.95), borderline for Alzhemier’s disease (Values for Alzheimer’s: phospho-tau >68 pg/ml; ATI >1.2). Magnetic resonance imaging (MRI) of the brain showed atrophy of the left parietal lobe (Fig. 1). Positive emission tomography (PET) demonstrated reduced uptake in the left parietal lobe (Fig. 2).
Fig. 1An axial (A) and coronal (B) T2-weighted brain MRI showed atrophy of the left parietal lobe out of proportion to that seen in the right hemisphere.
In the setting of asymmetric parkinsonism and dystonia, as well as ideomotor apraxia and a cortical sensory deficit on examination, the patient met criteria for probable corticobasal degeneration of the corticobasal syndrome subtype. This was further supported by asymmetric MRI and PET findings, as well as neuropsychological testing.
Corticobasal degeneration (CBD) is an atypical parkinsonian syndrome with marked asymmetry of symptoms related to deposition of hyperphosphorylated tau in the cortical and basal ganglia [
]. This is further subdivided into four phenotypes: corticobasal syndrome (CBS), frontal behavioral-spatial syndrome (FBS), nonfluent/agrammatic variant of primary progressive aphasia (naPPA), and progressive supranuclear palsy syndrome (PSPS) [
Corticobasal degeneration is poorly-responsive to levodopa and there are no disease-modifying treatments or approved pharmacological or therapeutic at this time [
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