Biomedical
Primary lateral sclerosis plus parkinsonism: a case report
Abstract
Background The standard of diagnosing primary lateral sclerosis, the Pringle criteria, requires three years of purely upper motor neuron symptom presentation before confirming diagnosis. This classic standard has been questioned on occasion due to its restrictive range of both time period and symptomatic exhibition. Case presentation This case report will review a 57-year-old Caucasian female who presented with pyramidal and extrapyramidal features suggestive of the exceedingly rare disease primary lateral sclerosis plus parkinsonism. We will describe the mixture of upper motor neuron signs and striking parkinsonian symptoms experienced by the patient, as well as the full diagnostic workup leading to her preliminary diagnosis. The details of this case will then be utilized to explore the diagnostic criteria of primary lateral sclerosis, as well as to work through the differential of conditions resembling Parkinson’s disease. Conclusions The current criteria to diagnose primary lateral sclerosis may be excluding patients with the disease and is an ongoing area of investigation. A thorough differential including other neurodegenerative conditions is necessary to consider and requires long-term follow-up.
Key Questions about Primary Lateral Sclerosis and Parkinsonism
The article "Primary lateral sclerosis plus parkinsonism: a case report" discusses a case of a 57-year-old woman presenting with both upper motor neuron (UMN) symptoms and parkinsonism. It explores the diagnostic challenges and the need for more inclusive diagnostic criteria to account for atypical presentations of primary lateral sclerosis (PLS).
What are the diagnostic criteria for primary lateral sclerosis (PLS), and how do they apply to this case?
The Pringle criteria are typically used to diagnose PLS, which require at least three years of purely upper motor neuron (UMN) symptoms. In this case, the authors question the application of these criteria, suggesting that they may exclude patients who show both UMN and parkinsonian symptoms, as seen in this patient.
How do the clinical presentations of PLS and Parkinson's disease overlap, and what challenges does this pose for diagnosis?
The article highlights the clinical overlap between PLS and Parkinson's disease, which share symptoms such as bradykinesia, rigidity, and gait disturbances. This overlap complicates diagnosis, as distinguishing between the two conditions requires careful differentiation based on the patient's full clinical presentation and history.
What is the significance of the term "PLS-plus," and how does it relate to atypical presentations of PLS?
The term "PLS-plus" refers to cases where PLS is accompanied by additional features such as parkinsonism. This term reflects the diversity in how PLS presents clinically, and the authors advocate for expanding diagnostic criteria to accommodate these atypical cases, which may otherwise be missed or misdiagnosed.
What are the implications of the current diagnostic criteria for PLS on patient prognosis and treatment?
The restrictive diagnostic criteria for PLS may lead to delays in diagnosis and treatment, potentially affecting patient outcomes. The authors suggest adopting a more flexible approach to diagnosing PLS that includes patients with atypical features, facilitating earlier intervention and improving the management of the disease.
By addressing these questions, the article underscores the importance of recognizing the variability in how PLS can present. It calls for a broader understanding of the disease and diagnostic criteria to ensure patients receive timely and appropriate care.
