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Neurology in Documentaries
Published in Eelco F. M. Wijdicks, Neurocinema—The Sequel, 2022
ALS should be distinguished from primary lateral sclerosis (pyramidal signs only), progressive muscular atrophy (peripheral signs only), and progressive bulbar palsy (lower motor neuron involvement of speech and swallowing). Primary lateral sclerosis may have a median survival of 20 years, but the other disorders progress similarly to ALS. Outcome is also better in specialized clinics, largely due to much better symptom management. Weight loss also carries a poor prognosis, but even if gastrostomy placement leads to adequate feeding, it only improves quality of life.
Spinal Cord Disease
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Primary lateral sclerosis: progressive symmetric spinobulbar spasticity and weakness of the limbs, more marked in the legs (spastic quadriparesis), and sometimes the bulbar musculature, causing a pseudobulbar palsy. LMN signs are absent and higher cognitive function is preserved. Linked to ALS by pathologic studies.
Discussions (D)
Published in Terence R. Anthoney, Neuroanatomy and the Neurologic Exam, 2017
Despite Gowers’ no doubt considerable influence in clinical neurology, I found no mention of “upper” or “lower” portions of the motor system, either as “segments” or “motor neurons.” in Starr’s comprehensive Organic Nervous Diseases (1903), in Church’s comprehensive Diseases of the Nervous System (1191) d iramianon from Die Deutsche Klimt"), or in the more basic-science-oriented texts of Villiger 09121 and Herrick 09ibi mentioned above. The earliest subsequent appearance of these terms 1 could find was in Tilney and Riley’s text (1921), also mentioned above. In their Fig. 209 (p. 218) of a patient with acute anterior poliomyelitis, they showed the region of “lower motor neurone paralysis.” Similarly, in discussing primary lateral sclerosis, they described “an upper motor neurone type of paralysis” (p. 230). The brief, casual way in which Tilney and Riley used these terms suggests to me that the terms were already well established by 1921. In Brock’s later text–The Basis of Clinical Neurology–also, the terms “upper motor neurone” and “lower motor neurone” were used almost entirely within the context of “upper and lower motor neurone paralysis” (1945. p. 40–42).
Longitudinal evaluation of upper motor neuron burden scales in primary lateral sclerosis
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2021
Patients with primary lateral sclerosis often live for decades after symptoms of spasticity begin. In a cohort of PLS patients followed longitudinally, we found that three upper motor neuron burden scales all changed more rapidly during the first approximately 8 years of symptoms than in later years. The clinical examination measures of spasticity on which the UMNB scales are based reach an apparent plateau after about 8 years. In some patients, the plateau was near the maximum score of the UMNB scale, and thus it is possible that continued worsening was masked by the scales’ ceiling. However, the slower decline after 8 years was also seen for a functional rating scale, the ALSFRS-R, as well as for quantitative measures of movement, supporting the explanation that disease progression slows or arrests over time (20). However, it is notable that the ALSFRS-R did continue to show some decline after 8 years, losing 1 point approximately every 4 years, whereas other measures, such as finger tapping speed, remained relatively stable after 8 years. The very slow decline in the ALSFRS-R is consistent with previous reports in patients with PLS (7) and supports using the PLSFRS, a more sensitive scale, in future studies (8).
Amyotrophic lateral sclerosis in Nordland county, Norway, 2000–2015: prevalence, incidence, and clinical features
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2018
Espen Benjaminsen, Karl B. Alstadhaug, Marie Gulsvik, Farid K. Baloch, Francis Odeh
The diagnosis of MND was based on findings of a progressive pure motor disorder with muscle weakness, atrophy, and fasciculations in the absence of sensory and sphincter disturbances. In primary lateral sclerosis (PLS), only the upper motor neuron was affected. The variant of progressive muscular atrophy (PMA) was suggested when the spinal innervated skeletal muscles were involved in the absence of central symptoms and the variant of progressive bulbar palsy (PBP) was suggested when only the bulbar muscles were involved. When the above findings were accompanied by pyramidal signs a diagnosis of ALS was set, in accordance to the revised “El Escorial”-criteria proposed by the World Federation of Neurology (8). The authors classified ALS in two sub groups, bulbar ALS (ALS-b) with symptoms or signs from motor cranial nerves at first examination and spinal ALS (ALS-s) with solely spinal symptoms or signs. They also noted if the patients had shown signs of cognitive impairment and also if the diagnosis of FTD (9) had been set, either by a neurologist, psychiatrist, or neuropsychologist.
Neuropathology of primary lateral sclerosis
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2020
There have been surprisingly few reports describing the postmortem neuropathology of patients with clinical primary lateral sclerosis (PLS), with many of these being published prior to the development of modern diagnostic techniques. Interpreting the pathological findings and how they might shed light on the relationship between PLS and other neurodegenerative conditions requires an understanding of the neuropathological features of classical amyotrophic lateral sclerosis (ALS), the overlap between ALS and frontotemporal dementia (FTD), and how this knowledge has evolved over the past decades.