Abstract
BackgroundNeurodegenerative disorders are characterized by insidious progression with poorly-delineated long latent period. Antecedent clinical insult could rarely unmask latent neurodegenerative disorders. Here, we report an autopsy-proven case of corticobasal degeneration which was preceded by a lacunar infarction.Case presentationA 58-year-old man presented with acute ataxia associated with a lacunar infarction in the right paramedian pons. His ataxia persisted with additional progressive gait difficulty and left arm clumsiness. Six months later, a follow-up neurological examination showed asymmetrical bradykinesia, apraxia, dystonic posturing, postural instability, and mild ataxia of the left limbs. Cognitive examination revealed frontal executive dysfunction and visuospatial difficulties. Dopamine transporter imaging scan demonstrated bilateral reduced uptakes in mid-to-posterior putamen, more prominent on the right side. Levodopa-unresponsive parkinsonism, asymmetric limb dystonia, and ideomotor apraxia became more conspicuous, while limb ataxia gradually vanished. The patient became unable to walk without assistance after 1 year, and died 4 years after the symptom onset. Autopsy findings showed frontoparietal cortical atrophy, ballooned neurons, and phosphorylated tau-positive astrocytic plaques and neuropil threads with gliosis and neuronal loss, confirming the corticobasal degeneration.ConclusionsThe case illustrates that precedent clinical events such as stroke might tip a patient with subclinical CBS into overt clinical manifestations.
Highlights
Neurodegenerative disorders are characterized by insidious progression with poorly-delineated long latent period
The case illustrates that precedent clinical events such as stroke might tip a patient with subclinical CBS into overt clinical manifestations
* Correspondence: taebeom.ahn@khu.ac.kr 1Department of Neurology, Kyung Hee University Hospital, Kyung Hee University College of Medicine, 23 Kyungheedae-ro, Dongdaemun-gu, Seoul 02447, Republic of Korea Full list of author information is available at the end of the article of Corticobasal degeneration (CBD) are heterogeneous in clinicopathologic studies, none has been reported clinical evolution of CBD consecutive to precedent non-neurodegenerative event [3]
Summary
Neurodegenerative disorders are characterized by insidious progression with poorly-delineated long latent period. Conclusions: The case illustrates that precedent clinical events such as stroke might tip a patient with subclinical CBS into overt clinical manifestations. The clinical presentation of CBD is a variable combination of asymmetrical akinetic-rigid syndrome, ideomotor limb apraxia, and other clinical features including cortical sensory deficits, dystonic posturing, myoclonus, and cognitive impairment [2].
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