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Questions for part B
Published in Henry J. Woodford, 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
The Neuropathology of Alzheimer’s Disease
Published in Zaven S. Khachaturian, Teresa S. Radebaugh, Alzheimer’s Disease, 2019
Suzanne S. Mirra, William R. Markesbery
Another neurologic disorder showing significant overlap with AD and other neurodegenerative disorders is progressive supranuclear palsy, characterized by supranuclear gaze palsy, postural instability, extrapyramidal signs, and cognitive dysfunction. Although the neuropathological features of progressive supranuclear palsy are relatively stereotypical, concomitant changes of AD, Parkinson’s disease, corticobasal degeneration, and other disorders are not uncommon. It is unclear whether this neuropathological overlap represents the coincidental occurrence of disorders frequently encountered in the elderly or whether they implicate common pathogenetic pathways. These disorders all share cytoskeletal pathology and tau-positive inclusions in neurons.
Case 63: Repeating the same story over and over again
Published in Barry Wright, Subodh Dave, Nisha Dogra, 100 Cases in Psychiatry, 2017
Barry Wright, Subodh Dave, Nisha Dogra
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].
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.
Emerging drugs for progressive supranuclear palsy
Published in Expert Opinion on Emerging Drugs, 2019
Nikolaos Giagkou, Maria Stamelou
Progressive supranuclear palsy is an orphan disease and thus potential disease-modifying therapies are candidates for orphan drug designation. Indeed, ABBV-8E12, BIIB092, and TPI-287 carry an orphan drug designation for PSP [80], and IONIS MAPTRx for frontotemporal dementia [80]. Orphan drugs can simultaneously be candidates for expedited approval schemes and thus an effective treatment can quickly reach the patients in need. Many challenges, however, await in the post-approval phase. Drugs for rare diseases can be particularly expensive and many might require lifelong administration [81]. Thus, the cost for many health-care systems will be substantial and many patients around the world might not be able to acquire access to such a treatment [81]. Moreover, depending on the expedited approval scheme, a drug might be licensed on the basis of early data, e.g., after demonstrating benefit on a surrogate marker of efficacy [82,83]. In these cases, post-approval data demonstrating efficacy are a regulatory requirement [82,83]. In the past, however, a few compounds have failed to demonstrate their efficacy in the post-approval period [84]. Comparisons of approved orphan and non-orphan medication for cancer and neurological conditions have shown that orphan drugs are more likely to be approved following uncontrolled, nonrandomized or open-label studies [85,86]. Fortunately, the two Phase 2 trials in PSP have robust designs and their primary endpoint, although a surrogate marker of efficacy, is clinically meaningful.