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Degenerative Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James A. Mastrianni, Elizabeth A. Harris
Epidemics of von Economo's encephalitis (encephalitis lethargica) swept Europe in the early 1920s with some patients developing progressive parkinsonism, not identical to PD. Postencephalitic parkinsonism is now very rare.
Biochemical Markers for Alzheimer Disease as Reflection of the Neuropathology in Cerebrospinal Fluid:
Published in Robert E. Becker, Ezio Giacobini, Alzheimer Disease, 2020
C. Bancher, H.M. Wisniewski, P.D. Mehta, K.S. Kim, I. Grundke-Iqbal, K. Iqbal
Any diagnostic test based on PHF must take the above mentioned conditions into consideration. Fortunately, all of these conditions with PHF can be clinically distinguished from AD. Down syndrome is identified by its characteristic phenotypic expression and karyotyping. Guam Parkinsonism-dementia complex is limited to the natives of the island of Guam (Chamorro Indians). Dementia pugilistica, also called punch drunk syndrome, is an extremely rare disease seen in professional boxers and horse jockeys. Postencephalitic Parkinsonism is rare and is distinguished from AD by its characteristic clinical symptomatology. Hydrocephalus is easily diagnosed with the help of neuroimaging. Lastly, the group of diseases in which only some cases show NFT are rare, occur generally at young age and are clinically distinguishable from AD.
Von Economo’s encephalitis
Published in Avindra Nath, Joseph R. Berger, Clinical Neurovirology, 2020
A literature survey by Duvoisin and Yahr in 1965 failed to identify any association between known viruses and post-encephalitic Parkinsonism [46]. Howard and Lees were unable to identify a viral agent in four cases reported in 1987 [47]. An extensive review of potential viral etiologies of postencephalitic Parkinsonism has failed to reveal an association with many other viruses [26].
Neuro-ophthalmology of movement disorders
Published in Expert Review of Ophthalmology, 2018
Dystonia of extraocular muscles can manifest as oculogyria, sometimes referred to as ‘oculogyric crisis,’ a sustained, involuntary, conjugate, painful, deviation of the eyes to one side or in upward or downward direction. It typically represents secondary dystonia such as drug-induced acute dystonic reaction, tardive dystonia in patients treated with dopamine-blocking medications, or in patients with dystonic tics due to Tourette syndrome [68]. Inherited disorders of monoamine metabolism, such as tyrosine hydroxylase deficiency, aromatic amino acid decarboxylase deficiency, pterin metabolism defect, can also manifest as oculogyria in children among other neurological symptoms [69,70]. Encephalitis lethargica, an extremely rare in present days, was presumed to be a viral disease resulting in postencephalitic parkinsonism; oculogyria was one the most common ocular manifestations [71].
Gabrielle Lévy and the Roussy-Lévy syndrome
Published in Journal of the History of the Neurosciences, 2018
In some patients the disease began abruptly and in others insidiously, like influenza. After the initial phase, they developed behavioral changes, cranial nerve palsies, and motor disorders, as well as variable degrees of lethargy. The seven patients presented by von Economo had in common a persistent, intractable stupor, although some were only mildly affected. In the years that followed, the disease spread and became pandemic, affecting approximately 5 million people. One-third did not survive the acute phase. Thousands of patients, often staying in nursing homes, suffered from postencephalitic syndromes that sometimes appeared after a latent period of years. In the late 1960s, some survivors with postencephalitic parkinsonism were treated with the new compound levodopa. Well-known neurologist and author Oliver Sacks (1933–2015) treated patients at Beth Abraham (New York) and observed spectacular results.17His book on this episode is well-known (Awakenings; Sacks, 1973), as became the homonymic movie (starring Robin Williams and Robert de Niro).
NeurHistAlert 24
Published in Journal of the History of the Neurosciences, 2018
Frank W. Stahnisch, Jyh Yung Hor
This article by two neurologists highlights an important historical contribution made by American neurologist and public writer Oliver Sacks (1933–2015). In research literature, all too often Sacks’s achievements in the history of neurology were reduced to simply popularizing neurology and psychology phenomena, rather than having made scientific research contributions to the field. As these Brazilian neurologists show, however, Sacks and his colleagues studied levodopa in clinical trials since 1969, in cases of postencephalitic Parkinsonism at the Beth Abraham Hospital in New York, and they pursued long-term observations on their patients. These insights led them to caution widespread application of levodopa in Parkinson’s patients due to frequent adverse long-term effects.