Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia
نویسندگان
چکیده
منابع مشابه
Assessment of the upper motor neuron in amyotrophic lateral sclerosis
Clinical signs of upper motor neuron (UMN) involvement are an important component in supporting the diagnosis of amyotrophic lateral sclerosis (ALS), but are often not easily appreciated in a limb that is concurrently affected by muscle wasting and lower motor neuron degeneration, particularly in the early symptomatic stages of ALS. Whilst recent criteria have been proposed to facilitate improv...
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The most common form of autosomal recessive hereditary spastic paraplegia is caused by mutations in the SPG11/KIAA1840 gene on chromosome 15q. The nature of the vast majority of SPG11 mutations found to date suggests a loss-of-function mechanism of the encoded protein, spatacsin. The SPG11 phenotype is, in most cases, characterized by a progressive spasticity with neuropathy, cognitive impairme...
متن کاملMotor neuron disease (amyotrophic lateral sclerosis) arising from longstanding primary lateral sclerosis.
Three men were initially diagnosed as having primary lateral sclerosis (PLS), but eventually developed amyotrophic lateral sclerosis (ALS) after 7.5, 9, and at least 27 years. Non-familial ALS and PLS might be different manifestations of a single disease or constitute completely distinct entities. The clinical diagnosis of PLS predicts a median survival that is four to five times longer than in...
متن کاملDifferentiation of hereditary spastic paraparesis from primary lateral sclerosis in sporadic adult-onset upper motor neuron syndromes.
OBJECTIVE To study whether clinical characteristics can differentiate sporadic presentations of hereditary spastic paraparesis (HSP) from primary lateral sclerosis (PLS). Differentiation between these diseases is important for genetic counseling and prognostication. DESIGN Case series. SETTING Tertiary referral center. PATIENTS One hundred four Dutch patients with an adult-onset, sporadic...
متن کاملObjective Measurements of Upper and Lower Motor Neuron Loss in Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive disease with an average surviving time of 3 to 5 years. The hallmarks of the pathological changes are loss of lower motor neurons in the spinal cord and upper motor neurons(UMN) in the motor cortex. However, structural MRI studies of the spinal cord and the brain often fail to show any changes. Due to lack of objective and quantitativ...
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ژورنال
عنوان ژورنال: Brain Sciences
سال: 2021
ISSN: 2076-3425
DOI: 10.3390/brainsci11050611