Better understanding the neurobiology of primary lateral sclerosis
نویسندگان
چکیده
منابع مشابه
The natural history of primary lateral sclerosis.
OBJECTIVE To define the syndrome of primary lateral sclerosis (PLS) and disorders that contain features of both ALS and PLS, to determine the time beyond which PLS is less likely to become ALS clinically, and to determine the outcome of people with PLS and those who develop lower motor neuron (LMN) signs. METHODS The authors reviewed the records of all 39 patients initially diagnosed with PLS...
متن کاملDecreased thickness of primary motor cortex in primary lateral sclerosis.
BACKGROUND AND PURPOSE Primary lateral sclerosis (PLS) is a rare form of motor neuron disease characterized by upper motor neuron dysfunction. Because pathologic examination has revealed a loss of neurons in the motor cortex of patients with PLS, we sought to confirm and extend this finding by using MR imaging to measure cortical thickness. METHODS Seven patients with PLS and 7 age-matched ne...
متن کاملHemiparetic Primary Lateral Sclerosis: Revisiting Mills Syndrome
A slowly progressive hemiparesis beginning in a single limb with evolution to the ipsilateral limb was originally described in 8 patients in 1906 by Mills. We present 5 cases of progressive hemiparetic corticospinal tract degeneration, identified by the clinical presentation and the exclusion of other etiologies using serological, imaging, and electrodiagnostic studies.
متن کاملBlink reflex in primary lateral sclerosis.
OBJECTIVE To investigate the underlying pathophysiology of primary lateral sclerosis (PLS) regarding possible changes in blink reflex (BR) circuit. METHODS BR was studied in 10 patients in whom diagnosis of PLS was established by comparing them to two control groups: ten patients with amyotrophic lateral sclerosis (ALS) and 30 healthy volunteers. All patients were followed up annually for a 5...
متن کاملTDP-43—The key to understanding amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that causes motor neuron degeneration leading to progressive muscle atrophy, weakness, paralysis and death. The majority of ALS (>95%) shows intracellular aggregation of transactive response DNA binding protein (TDP-43) as a prominent pathological feature. TDP-43 is normally a nuclear protein. In ALS, TDP-43 accumulates an...
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ژورنال
عنوان ژورنال: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
سال: 2020
ISSN: 2167-8421,2167-9223
DOI: 10.1080/21678421.2020.1837175