Neuromuscular Disorders and ALS

Neuromuscular Disorders

• Amyotrophic lateral sclerosis (ALS) typically causes both upper and lower motor neuron signs.
• A 60-year-old with asymmetric atrophy, weakness, fasciculations, swallowing difficulties, and strained speech is likely to be diagnosed with ALS.
• Pulmonary dysfunction early in the clinical course is considered a poor prognostic factor in patients with ALS.
• For patients with ALS the prognosis is 50% die within 3 years
• Riluzole is a pharmacologic agent approved for patients with ALS to slow the progression and improve survival.
• Werdnig-Hoffmann disease typically causes lower motor neuron signs and has the earliest disease onset.
• A 16-year-old using his hands and arms to "walk" up his own body from a squatting position most likely has spinal muscular atrophy (SMA) type III.
• A patient with spinal muscular atrophy (SMA) type II can usually achieve independent sitting.
• Kugelberg-Welander disease has the best prognosis of the motor neuron diseases listed.
• Baclofen is a first-line treatment for spasticity in patients with amyotrophic lateral sclerosis (ALS).
• Amyotrophic lateral sclerosis (ALS) is the most common presenting form of motor neuron disease in adults.
• Stability for approximately 5 years is NOT included in the Halstead and Rossi (1987) criteria in defining postpolio syndrome.
• Picornavirus has been implicated in the development of poliomyelitis.
• Myasthenia gravis is a disorder of neuromuscular transmission due to an autoimmune response against acetylcholine receptors on the postsynaptic membrane.
• Lambert-Eaton myasthenic syndrome (LEMS) etiology is a disorder of neuromuscular transmission due to an autoimmune response against the active sites on the presynaptic membrane.
• Disorder resulting in a decreased quantal response because of an autoimmune response against acetylcholine receptors on the postsynaptic membrane
• A 45-year-old with complaints of fatigue and weakness that are exacerbated with rest and often improve with exercise is likely to have Lambert-Eaton myasthenic syndrome (LEMS) presynaptic
• A 49-year-old woman presents to your clinic with complaints of fluctuating double vision and droopy eyelids most likely has Myasthenia gravis (MG).
• Trivalent ABE antitoxin is most appropriate for patients with complaints of fluctuating double vision 18. and droopy eyelids.

Myopathies and other disorders

• Mestinon is used to treat neuromuscular disorders
• Symptoms of botulism present 1 day after spore ingestion
• Dystrophic myopathy causes a 4-year-old difficulty getting up that needs to perform a maneuver to assist him in standing is caused by: Proximal leg weakness
• Duchenne muscular dystrophy (DMD) is characterized by a steadily progressive, X-linked muscular dystrophy is characterized by absent dystrophin or less than 3% that is normal.
• Becker's muscular dystrophy is characterized by:X-linked and reduced dystrophin
• Facioscapulohumeral dystrophy (FSHD) is not associated with cardiac abnormalities
• Duchenne muscular dystrophy (DMD) is characterized by cognition may be impaired
• All of the above, sudden cardiac death, contractures and scoliosis are risks for a patient with Duchenne muscular dystrophy (DMD) as his or her disease progresses
• Becker's muscular dystrophy (BMD) affects an 18-year-old boy with an X-linked genetic disease has a mild functional disability, proximal muscles weakness, prominent calves, lordosis, and a waddling gait. He is not wheelchair bound.
• Facioscapulohumeral dystrophy (FSHD) affects a 19-year-old female presents to your outpatient office with complaints of not not being able to smile or close her eyes
• Emery-Dreifuss muscular dystrophy (EMD) affects a teenager presents with an elbow flexion contracture and mild weakness that has affected his ability to walk
• Facioscapulohumeral dystrophy is characterized by an inability to extend the wrist, as a clinical feature
• Central core disease is a disorder of neuromuscular transmission due to an autoimmune response against acetylcholine receptors on the postsynaptic membrane except Mitochondria present on muscle biopsy
• Dermatomyositis is recommended in adult patients with newly diagnosed a judicious workup for carcinoma
• Bimodal aged is the age of onset for idiopathic inflammatory myopathies
• Primary idiopathic dermatomyositis has a heliotrope rash with periorbital edema.
• Inclusion body myositis does not treat treated primarily with corticosteroids or other immunosuppressive medications
• Pergolide is an ergot derivative and stimulates D2 receptors in the treatment of Parkinson's disease
• Impaired vibration or position sense is NOT a sign/symptom of Parkinson's disease
• Parkinson's disease results with decrease in dopamine input in the striatum and an increase in cholinergic input
• Subthalamic deep brain stimulation is recommended that may help reduce his symptoms for with advanced Parkinson's disease that interferes with his quality of life. What can you recommend that may help reduce his symptoms?
• Dopamine receptor antagonist treats Parkinson's disease can be effectively medically except
• Postural hypotension is a common side effect of anti-Parkinson drugs (e.g., Sinemet, Requip)
• Interferon beta-1a does not cause Parkinson's
• Huntington's disease results is linked to a trinucleotide CAG repeat HD gene.
• Relapses decrease during pregnancy is false regarding multiple sclerosis (MS) and pregnancy
• Glatiramer acetate is safe treatment option during pregnancy
• Multiple sclerosis (MS) etiology is thought to be an autoimmune response causing demyelination, axonal damage, and brain atrophy
• Affects males more frequently than females' is false regarding multiple sclerosis
• Relapsing-remitting is the most common pattern of multiple sclerosis
• Age of onset greater than 35 years is bad prognostic factors in multiple sclerosis
• Bowel/bladder dysfunction are common symptoms in patients with multiple sclerosis (MS)
• Decreased IQ is a symptom commonly seen with Neuromuscular Disorders
• Sensory disturbance is NOT a major problem affecting activities of daily living for MS
• Weakness and/or Lhermitte's sign can been seen in patients with multiple sclerosis (MS)
• The multifocal “bright” areas of hyperintensity on T2-weighted images, are pathognomonic test for multiple sclerosis (MS)
• To speed recoery
• Immunomodulator agents is is a first-line treatment choice that can reduce relapse
• Lateral medullary syndrome is diagnosis that a patient has if is is has ptosis, anhidrosis, and miosis on the left side of his face
• Decrease muscle strength on the contralateral side cannot be seen in lateral medullary (Wallenberg) syndrome
• Lateral medullary syndrome a patient has when presents to the emergency department with loss of muscle strength in his right upper and lower extremities.
• Contrlateral decreased pain and temperature is the symptom that Medial medullary syndrome does NOT characteristically presents
• Insult or injury to the CNS is permanent and functional ability cannot be altered with any type of is NOT a concept that
  Neuroplasticity refers to
• It is based on principles of an intensive the correct statement regarding constraint-induced movement therapy (CIMT)
• Redacting is NOT part of Neural strategies of functional improvement after central nervous system injury
• Allows for the simulation of task-specific walking movements is the benefits of partial body weight supported gait training include
• Compensation is the not positively influenced by Neuroplasticity
• Amantadine side effect is a side effects from the following medications should be considered in the differential diagnosis of Parkinson's disease except
• Microscopically noted to have intracytoplasmic eosinophilic inclusions called Lewy bodies in damaged cells is NOT a Huntington's disease characterized by
• Rapidly progressive onset is good for prognosis indicators in patients with multiple sclerosis
• Impaired convergence is Not a symptoms are seen in multiple sclerosis
• Lhermitte's sign is a passive neck flexion causing electrical shock-like sensation radiating to the spine, shoulders, and other areas that produce
• All the following are suggestive findings of MS except for choice Increased amounts of MS is NOT suggestive for the diagnostic

More Neuromuscular Disorders

• Corticosteroids is NOT used in the treatment of MS
• Uhthoff's phenomenon results in Worsening of neurological symptoms including visual problems seen with increased body temperature
• Faciculations are typical features of amyotrophic lateral sclerosis (ALS) associated with lower motor neuron disease
• Extraocular weakness is NOT characteristic is myasthenic syndrome or Lambert-Eaton syndrome (LES)
• multiple system atrophy (Shy- Drager syndrome) characterizes when the Parkinson plus syndromes is characterized by onset of 40 to 60 years of age, involving weakness in lower extremities, ataxia plus dysarthria
• Dantrolene sodium used for spasticity has the least amount ofsedation and cognitive impairment
• A 12-year-old boy complaint of progressive hearing facioscapulohumeral dystrophy (FSHD) deficit's and difficulty whistling is a symptom for this condition -
• Myelomeningocele is type II (the downward displacement of the medulla and brainstem through the foramen magnum causing kinking of the describes a Arnold–Chiari malformation that occurs on the brainstem)
• Cardiovascular complications is not the regarding spina bifida,
• Restrictive lung disease due to for pulmonary dysfunction seen in patients due to
• "all the above" are true about Duchenne muscular dystrophy are true
• Weber Syndrome Syndrome.
• Left Anterior Cerebral Artery is to be a lacunar syndrome.
• In The Miller Fischer Variant of Guillain-Barré syndrome the finding are _Ophthalmoplegia, ataxia, and areflexia
• "A common cause of Cerebral palsy "TORCH congenital infections.
• A.history of maternal, B. maternal bleeding,. history of maternal C.seizures, D.preamture births Are all associated with cerebral pasly but are non progressive In the immature brain! In the immature brain
• PVL more common in children with CP Friedreich's is an onset with cerebellar ataxia related fatigue

exertion fatigue- in myasthenia Gravis

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