Questions for part B
Henry J. Woodford in Essential Geriatrics, 2022
An 81-year-old woman was diagnosed with Parkinson's disease a year ago. Since then, she has had little response to levodopa and has had a rapid decline in her mobility and cognition. Which MRI scan finding would be supportive of a diagnosis of progressive supranuclear palsy?Anterior temporal lobe atrophyEmpty delta signHot cross bun signHummingbird signPulvinar sign
Epidemiology and subtypes of dementia
Marjolein de Vugt, Janet Carter in Understanding Young Onset Dementia, 2021
Progressive Supranuclear Palsy (PSP) is a rare progressive neurodegenerative condition that presents with postural instability, supranuclear gaze palsy, dementia, and progressive axial rigidity and bulbar palsy (Steele et al., 1964). PSP is pathologically defined by the accumulation of tau protein and neuropil threads, mainly in the brainstem (Höglinger et al., 2017). PSP is clinically heterogeneous; however, ocular motor dysfunction, postural instability, akinesia, and cognitive dysfunction are key clinical predictors of PSP (Höglinger et al., 2017). Significant overlap exists between the PSP and the CBS syndrome, which can make it difficult to distinguish (Höglinger et al., 2017; Williams & Lees, 2009). Therefore, the gold standard of diagnosing PSP remains post-mortem pathological examination (Höglinger et al., 2017).
Case 63: Repeating the same story over and over again
Barry Wright, Subodh Dave, Nisha Dogra in 100 Cases in Psychiatry, 2017
Alzheimer dementia is the most likely cause. There is no evidence of Parkinson disease or Huntington disease on examination, and Creutzfeldt–Jakob disease is unlikely given the history. The absence of lethargy or disinhibition makes frontotemporal dementia unlikely. It will be important to exclude depression by further history and discussion with his wife. Dementia with Lewy bodies usually involves parkinsonian features (although these may not be evident early in the illness) and often involves visual hallucinations, alongside attentional problems, fluctuating abilities and planning (executive functioning) difficulties. Cortico-basal ganglionic degeneration also involves parkinsonism and signs of cortical atrophy including dyspraxia, dysphasia, cortical sensory loss and action tremor. Progressive supranuclear palsy would usually involve regular falls, slow blinking, dry eyes, tunnel vision, slurred speech and swallowing problems followed by further movement problems including walking difficulties. Vascular dementia is typically described as progressing with step-wise deterioration. It is more likely in the presence of a history of hypertension, diabetes, vascular disease or smoking.
Low intensity exercise training in patients with amyotrophic lateral sclerosis – factors influencing eligibility and compliance to the clinical trial
Published in Neurological Research, 2023
J. Sznajder, K. Barć, M. Kuźma-Kozakiewicz
We have found a long commute to the study site and an overall disease progression to be the main real-life reasons preventing ALS patients from participation and retention in a non-commercial study. It is in line with a report by Drory et al. who listed breathing difficulties, loss of mobility and death as reasons for the dropout [12], while Dal Bello et al. found disease progression, depression and lack of benefit from participation as the main reasons [13]. A similar tendency was found in other neurodegenerative diseases. In an 18-month-long study in patients with progressive supranuclear palsy, Pereira et al. reported a 53% dropout due to severity of neurological symptoms/death or revision of the original diagnosis [21]. In Parkinson’s disease, a cognitive impairment and a sedentary lifestyle were the main reasons for dropping out from the 6-year study [24].
Training high level balance and stepping responses in atypical progressive supranuclear palsy: a case report
Published in Physiotherapy Theory and Practice, 2023
Earllaine Croarkin, Krystle Robinson, Christopher J. Stanley, Cris Zampieri
Progressive supranuclear palsy (PSP) is a neurodegenerative disease and the most common atypical Parkinsonian syndrome (Litvan et al., 1996). It has been referred to as a “Parkinson-plus disorder” (Agarwal and Gilbert, 2020). Initial motor symptoms are symmetrical and axial with associated gait difficulties and falls (Agarwal and Gilbert, 2020). As the condition progresses, a differentiating sign of slowed vertical gaze worsens along with dysarthria, dysphagia, and frontal cognitive difficulties (Agarwal and Gilbert, 2020). It is rare. The highest prevalence rate, found during a brief literature review, was reported at 6.4 per 100,000 (Schrag, Ben-Shlomo, and Quinn, 1999). Multiple PSP phenotypes have been noted, making diagnosis difficult (Agarwal and Gilbert, 2020; Lopez, Bayulkem, and Hallett, 2016). Thus, diagnosis is usually made by the presence and progression of multiple signs including, but not limited to: postural instability, supranuclear opthalmoplegia primarily of vertical gaze, pseudobulbar dysfunction, dystonic rigidity of the neck and upper trunk and mild cognitive dysfunction (Richardson, Steele, and Olszewski, 1963). Additionally, ruling out other diagnoses by noting treatment response, is a common approach in diagnosing movement disorders (National Institute of Neurological Disorders and Stroke web-site https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Progressive-Supranuclear-Palsy- Fact-Sheet#3281_5).
Parkinson’s disease dystonia as a cause of respiratory distress and stridor
Published in Canadian Journal of Respiratory, Critical Care, and Sleep Medicine, 2021
Mohammed A.M. Farooqi, Ahmed Attar, Arun Mensinkai, Gerard Cox
She presented two additional times with similar presentations that required brief noninvasive positive pressure ventilation. Within 12 hours, her symptoms would resolve, and no longer had stridor by the time she was assessed for nasopharyngoscopy. Of note, she continued to have significant dystonic symptoms during her presentations, and Neurology was consulted and recommended a reduction in her Levodopa-Carbidopa dosing to half the current dose. An MRI head confirmed no features suggestive of multi-system atrophy or progressive supranuclear palsy. She was never noted to have signs of dysautonomia, such as labile blood pressure. During this time, she was also worked up for a deep brain stimulator. She received the deep brain simulator to her subthalamic nuclei bilaterally, and since then her Parkinson’s symptoms and episodic shortness of breath significantly improved, requiring no further hospital admissions for ventilatory support.
Related Knowledge Centers
- Neurodegenerative Disease
- Brain
- Balance Disorder
- Hypokinesia
- Ophthalmoparesis
- Cognitive Impairment
- Parkinson's Disease
- Frontotemporal Dementia
- Alzheimer's Disease
- Tau Protein