Primary lateral sclerosis (Q41049): Difference between revisions
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Esclerose lateral primária (ELP) é uma variante rara da doença do neurônio motor que se apresenta com sinais de NMS lentamente progressivos, como marcha espástica, espasmos bruscos intensos dos tendões e respostas extensoras plantares. O início é mais comumente com paraparesia espástica, mas os pacientes tipicamente evoluem para desenvolver envolvimento bulbar e de membros superiores. O aspecto característico da ELP é a completa ausência de envolvimento do envolvimento do neurônio motor inferior. Quando os sinais de LMN se desenvolvem durante o curso da doença, o diagnóstico muda para ELA, e eles são considerados um espectro do mesmo transtorno. | |||
| description / en | description / en | ||
Primary lateral sclerosis (PLS) is a rare motor neuron disease variant which presents with slowly progressive UMN signs, such as spastic gait, brisk deep tendon jerks, and extensor plantar responses. Onset is most commonly with spastic paraparesis, but patients typically progress to develop upper limb and bulbar involvement. The characteristic feature of PLS is the complete absence of involvement of lower motor neuron involvement. When LMN signs develop during the course of the disease, the diagnosis will change to ALS, and they are considered a spectrum of the same disorder. | |||
Revision as of 07:06, 13 August 2026
Primary lateral sclerosis (PLS) is a rare motor neuron disease variant which presents with slowly progressive UMN signs, such as spastic gait, brisk deep tendon jerks, and extensor plantar responses. Onset is most commonly with spastic paraparesis, but patients typically progress to develop upper limb and bulbar involvement. The characteristic feature of PLS is the complete absence of involvement of lower motor neuron involvement. When LMN signs develop during the course of the disease, the diagnosis will change to ALS, and they are considered a spectrum of the same disorder.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | 8B60.4 |
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| English | Primary lateral sclerosis |
Primary lateral sclerosis (PLS) is a rare motor neuron disease variant which presents with slowly progressive UMN signs, such as spastic gait, brisk deep tendon jerks, and extensor plantar responses. Onset is most commonly with spastic paraparesis, but patients typically progress to develop upper limb and bulbar involvement. The characteristic feature of PLS is the complete absence of involvement of lower motor neuron involvement. When LMN signs develop during the course of the disease, the diagnosis will change to ALS, and they are considered a spectrum of the same disorder. |
