Doença do motoneuronio Flashcards
O que avaliar na história e no exame físico na suspeita de doença do motoneuronio?
História: Sx, distribution of sx, time course. Exposure to toxins/travel history/military history/family history; HIV risk f actors; h/o malignancy, systemic disease.
Exame: UMN: Weakness, spasticity, increased tone, hyperrefl exia, + Babinski. LMN: Weakness, fasciculations, decreased tone, hyporefl exia, − Babinski. Presence of hyperrefl exia: Babinski, palmomental, Hoffmann’s, brisk jaw jerk, clonus, brisk abdominal refl exes. Presence of pseudobulbar palsy. Due to involvement of corticobulbar tracts which exhibit supranuclear control over motor nuclei controlling speech, chewing, swallowing. Sx: Dysarthria, dysphagia, emotional lability, or emotional incontinence with spontaneous laughter or crying. ROS: Night sweats, fevers, weight loss, gynecomastia, impotence, rash, neck pain, sx suggestive of systemic disease.
Quais exames solicitar na suspeita de doença do motoneuronio?
Workup: Labs (chem 10, CBC, coags, CK, Lyme, ESR, CRP, HIV, SPEP, UPEP, IFE, B12, TSH); EMG/NCS; MRI brain or spine depending on clinical history & distribution of sx; consider CT chest, abdomen, or pelvis, or PET scan to evaluate for malignancy; genetic testing, depending on clinical history
Qual o manejo geral das doenças do neuronio motor?
Rx: For many of these illnesses, no Rx to slow progression of disease. For amyotrophic lateral sclerosis (ALS), Riluzole shown to prolong survival (see below in “Amyotrophic lateral sclerosis” section for details). Supportive care: PT, OT, speech therapy, walking assist devices, walking aids (cane, walker, or wheelchair), braces, trach/PEG if pt wishes, genetic counseling in appropriate cases, supportive groups. Agents for spasticity, medications to reduce drooling. Important to diagnose or excluded diseases w/ available Rx.
