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Images| Volume 62, P216-217, April 2019

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A case of profound asymmetry in motor skills

Published:January 03, 2019DOI:https://doi.org/10.1016/j.jocn.2018.12.021

      Keywords

      1. Clinical scenario

      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).
      Figure thumbnail gr1
      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.
      Figure thumbnail gr2
      Fig. 2Brain PET imaging showed reduced fluorodeoxyglucose uptake in the left parietal lobe.
      The most likely diagnosis is
      A: Rasmussen’s encephalitis
      B. Corticobasal degeneration
      C. Idiopathic Parkinson’s disease
      D. Progressive Supranuclear Palsy
      E. Alzhemier’s disease

      2. Answer

      B. Corticobasal degeneration

      3. Discussion

      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 [
      • Armstrong M.J.
      • Litvan I.
      • Lang A.E.
      • et al.
      Criteria for the diagnosis of corticobasal degeneration.
      ,
      • McFarland N.R.
      Diagnostic approach to atypical parkinsonian syndromes.
      ]. Onset of symptoms typically occurs in the sixth decade and includes asymmetric hand clumsiness, bradykinesia, tremor, and rigidity [
      • Armstrong M.J.
      • Litvan I.
      • Lang A.E.
      • et al.
      Criteria for the diagnosis of corticobasal degeneration.
      ,
      • McFarland N.R.
      Diagnostic approach to atypical parkinsonian syndromes.
      ]. Typical features on examination include tremor, dystonia, alien limb phenomenon, apraxia, myoclonus, cortical sensory loss, postural instability and gait dysfunction [
      • Armstrong M.J.
      • Litvan I.
      • Lang A.E.
      • et al.
      Criteria for the diagnosis of corticobasal degeneration.
      ,
      • McFarland N.R.
      Diagnostic approach to atypical parkinsonian syndromes.
      ].
      Previously a pathologic diagnosis, CBD has since been divided into probable and possible categories [
      • Armstrong M.J.
      • Litvan I.
      • Lang A.E.
      • et al.
      Criteria for the diagnosis of corticobasal degeneration.
      ]. 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) [
      • Armstrong M.J.
      • Litvan I.
      • Lang A.E.
      • et al.
      Criteria for the diagnosis of corticobasal degeneration.
      ,
      • McFarland N.R.
      Diagnostic approach to atypical parkinsonian syndromes.
      ]. At this time, imaging findings are not included as part of the proposed diagnostic criteria for CBD [
      • Armstrong M.J.
      • Litvan I.
      • Lang A.E.
      • et al.
      Criteria for the diagnosis of corticobasal degeneration.
      ]. However, T2-weighted and FLAIR images may reveal asymmetric atrophy of the posterior frontal and parietal lobes [
      • Ogawa T.
      • Fujii S.
      • Kuya K.
      • et al.
      Role of neuroimaging on differentiation of parkinson's disease and its related diseases.
      ]. Asymmetric hypometabolism can also be seen on 18F-fluorodeoxyglucose PET imaging [
      • Ogawa T.
      • Fujii S.
      • Kuya K.
      • et al.
      Role of neuroimaging on differentiation of parkinson's disease and its related diseases.
      ].
      Corticobasal degeneration is poorly-responsive to levodopa and there are no disease-modifying treatments or approved pharmacological or therapeutic at this time [
      • Lamb R.
      • Rohrer J.D.
      • Lees A.J.
      • et al.
      Progressive supranuclear palsy and corticobasal degeneration: pathophysiology and treatment options.
      ]. Treatment is typically supportive and aimed at optimizing quality of life [
      • Lamb R.
      • Rohrer J.D.
      • Lees A.J.
      • et al.
      Progressive supranuclear palsy and corticobasal degeneration: pathophysiology and treatment options.
      ].

      4. Contributors

      Drs. Zuzuárregui and Menezes were responsible for the concept design. All authors participated in the drafting of the manuscript.

      5. Conflicts of interest/disclosures

      The authors do not have any financial or other conflicts of interests to declare.

      References

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        • Lang A.E.
        • et al.
        Criteria for the diagnosis of corticobasal degeneration.
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        Diagnostic approach to atypical parkinsonian syndromes.
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        Role of neuroimaging on differentiation of parkinson's disease and its related diseases.
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        Progressive supranuclear palsy and corticobasal degeneration: pathophysiology and treatment options.
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