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| Question | Answer |
| Weakness is in UMN or LMN? | Both (LMN is weakness + atrophy) |
| Hyperreflexia is UMN or LMN? | UMN |
| Hypotonia is UMN or LMN? | LMN |
| Areflexia is UMN or LMN? | LMN |
| Spinal nerve -related sensory loss is in a ____ patterm. | dermatomal |
| When someone has both UMN and LMN lesion, which one overrides? | UMN. |
| What is a common symptom in ALS/ MG/ MD? | NO SENSORY LOSS!!!!!!! |
| How do you differentiate carpel tunnel syndrome from ALS? | ALS has NO SENSORY LOSS!!!!!! Carpel tunnel syndrome has numbness/tingling |
| What is the UMN in body motor pathway? | LCST (lateral cortical spinal tract) |
| What is the UMN in face/head motor pathway? | corticobulbar tract |
| What are the LMN nuclei in body motor pathway? | anterior horn cell in SC |
| What are the LMN nuclei in face/head motor pathway? | cranial nerve motor nuclei (trigeminal, facial, ambiguus, spinal accessory, hypoglossal) |
| Which cranial nerves do not belong to corticobulbar tract? | CN III, IV, VI (visual pathway) |
| Where are the LMN axons for body motor pathway? | From spinal nerve root to peripheral nerves |
| Where are the LMN axons for face/head motor pathway? | cranial nerves (VI, VII, IX, X, XI, XII) - intracranial -extracranial |
| Where do the LMN terminate for all motor pathways? | NMJ |
| T/F: proximal muscle weakness is a common sign of a myopathy such as DMD. | T. |
| One of the confusing things about the diagnosis of ALS is | Might look like carpel tunnel. (some neurons are spared) |
| Name an acquired infectious motor neuron disease. | polio |
| Name an acquired autoimmune motor neuron disease. | Polymyositis |
| Name two acquired idiopathic motor neuron disease | ALS PLS |
| T/F: ALS is the most common adult motor neuron disease. | T. |
| T/F: ALS affects male more than female. | T. (1.5 :!) |
| 2/3 ALS has _______ onset while 1/3 ALS has _______ onset. | 2/3: spinal onset. 1/3: bulbar onset. |
| Mean age of onset for ALS is | 60 years for sporadic/ unknown cause 40 years for inherited ALS |
| Which structures are affected in ALS? | Motor neuron |
| Which part of motor neuron is damaged? | CELL BODY!!! |
| T/F: only LMN signs are present in ALS | F. ALL MOTOR NEURON CELLs are affected. |
| ________ degeneration occurs at the motor axon. | Wallerian |
| Which nuclei are spared in ALS? | 1. oculomotor (CNIII) 2. trochlear (CN IV) 3. abducens (CN VI) 4. nucleus of Onuf (bowel and bladder) |
| If someone comes in with motor dysfunction together with bowel/bladder issue, what Dx can you rule out? | ALS |
| What are the most common initial symptoms of ALS? | loss fo dexterity stiffness cramps fatigue |
| What are some possible progression of ALS before they become dependent for mobility and ADLs? | weakness, slurred speech, atrophy, spasticity, swallowing and respiration involvement. |
| How many years on average do patient typically have from onset of symptoms to death? | 3 -5 years |
| List all the functions that are spared in ALS. | 1. extraocular muscles 2. cognitive function 3. sensory function 4. bowel and bladder |
| The progression of motor dysfunction with spinal onset ALS is | distal to proximal (limb onset) |
| T/F: ALS with spinal onset always have symmetrical presentation. | F. Usually asymmetrical. |
| What are the two most common symptoms for bulbar onset ALS? | Dysarthria dysphagia |
| T/F: limb symptoms come at a later stage with bulbar onset ALS. | F. Usually simultaneous. |
| How is ALS typically diagnosed? | By exclusion. |
| What are the pathologies that need to be ruled out before giving an ALS Dx? | First look for spared extraocular, cognitive, sensory, bowel/bladder MRI to rule out cortical, brainstem, and cervical spine pathology Lumbar puncture to rule out inflammation Body tests to rule out toxic, metabolic, infections, inflammation and genetic disease. |
| What are some confirmative (NOT GOLD STANDARD) tests for ALS? | ENMG |
| Which results are likely in NCV tests for ALS patients? | 1. decreased CMAP 2. normal or slow nerve conduction velocity (normal early on, slowed in chronic) 3. NORMAL sensory NCV. |
| Why might ALS patients have normal motor NCV early on in the stage? | some normal axons may camouflage the loss |
| What are some EMG findings in ALS patients? | 1. Fibrilations and + sharp waves 2. fasciculations 3. interference pattern reduced (partial or sparse) |
| If a patient has carpel tunnel syndrome instead of ALS, what are the likely NCV results? | reduced NCV for both motor and sensory neurons. |
| What is the ONLY approved medication for ALS patients? | Riluzole |
| What are the symptoms that should and can be managed as the patient progresses along the ALS stages? | Sialorrhea Muscle cramps Spasticity Dyspnea Depression Swallowing (via food modification and PEG) Respiratory care (ventilation, PFT) |
| What is the major cause of death in ALS patients? | Respiratory failure |
| Life expectancy after diagnosis? | Spinal form: 3 - 5 years Bulbar form: 2 - 3 years 10%: > 10 years |
| What is a positive prognosis indicator? | younger age at onset |
| What is a negative prognosis indicator? | Early onset of respiratory dysfunction |
| What are some intervention options for ALS patients? | 1. psychological benefit of exercise and movement 2. energy conservation and safety 3. preserve and prevent 4. caregiver training 5. equipment |
| What is the most common NMJ disorder? | Myasthenia Gravis |
| What is the most likely age of onset for MG in women and men? | women: 20s and 30s men: 50s and 60s |
| What is the antibody that causes the autoimmune response for patients with MG? | AChR (Acetylcholine receptors) antibodies |
| 90% have AChR antibodies in ______ while 50% have AChR antibodies in ______. | 90%: generalized form 50%: ocular form |
| ___% patients have associated autoimmune disease while ____% have comorbid thyroid disease. | 5% have associated autoimmune 10% have thyroid disease |
| What are the most common thyroid diseases in MG patients? | 1. hyperplasia 2. thymoma |
| Why are patients with MG weak? How does it present clinically? | Their ACh receptors are blocked by antibodies MMT: first contraction is strong. Weakens with consecutive contractions. After rest gets strong again. |
| T/F: the onset of MG is sudden. | F. It is subtle. |
| What are some potential exacerbation factors of MG? | illness, pregnancy |
| Typical overall presentation after exacerbation of MG? | fluctuating weakness and fatigability with full body involvement within 1 year |
| When do patients usually reach maximum severity after exacerbation of MG? | 2 years. |
| T/F: MG is always deteriorating instead of exacerbation and remission. | F. May include episodic exacerbation and remission. |
| List some common clinical findings for MG patients. | 1. ptosis 2. diplopia 3. dysarthria 4. dysphagia 5. respiratory weakness 6. limb weakness *** all weakness are fluctuating *** |
| Pattern of weakness in MG patients 1. proximal vs distal 2. arms vs legs 3. flexors vs extensors | 1. proximal > distal 2. Arms > legs 3. extensors > flexors (head drop) |
| The most typical MG progresses from _______ to _______. | Face to LE. |
| What are some definitive tests for MG? | 1. tensilon test (if positive) 2. serologic testing for AChR-binding antibodies 3. slow repetitive nerve stimulation 4. CT/MRI to screen for thymoma |
| Which test is more sensitive to MG? Tensilon or slow repetitive nerve stimulation? | Tensilon. |
| T/F: both tensilon and slow repetitive nerve stimulation are highly specific to MG. | F. Both are not specific. |
| What is the main concern of tensilon test? | Cardiac side effects. |
| The most effective medical management for MG in early stages is __________. | Cholinesterase inhibitors. |
| What do cholinesterase inhibitors do? | They increase the concentration of ACh at the receptor |
| What are the side effects of cholinesterase inhibitors? | 1. diarrhea, cramping, excessive secretions. (muscarinic) 2. muscle fasciculations, cholinergic crisis (nicotinic) |
| What are the short-term stabilizers for myasthenic crisis? | plasmaphersis IVIG (IV immunoglobulin) |
| What are the pros of plasmapheresis and IVIG? | 1. clinical improvement within the first week of treatment 2. benefits last for 1-2 months 3. complications are uncommon |
| Which treatment are done if plasmapheresis and IVIG are not effective? | Prior to thymectomy. |
| Three immunosuppressive treatment for MG: | 1. thymectomy 2. corticosteroids 3. nonsteroidal immunosuppressive |
| Which treatment takes weeks to see improvement and which one takes months? | weeks: corticosteroids months: nonsteroidal |
| Risk/ side effects of corticosteroidal and nonsteroidal treatments. | corticosteroidal: only 50% patient enter remission. possible for multi-system complications. nonsteroidal: bone marrow suppression may occur |
| ___% of patients with focal disease eventually develop generalized MG. | 80 |
| When does MG progress to maximal severity? | within 2 years of onset |
| What is a negative prognostic factor for MG? | thymoma |
| T/F: patients with MG has decreased lifespan. | F. Normal lifespan. |
| What are the clinical patterns of MG patients? | 1. fluctuation and fatigable weakness. 2. proximal > distal weakness 3. UE> LE 4. bilateral asymmetric ocular weakness (with pupil sparing) 5. nasal quality in voice |
| How do you modify your treatment with patients with MG? | 1. consider fatigue and give breaks (MMT throughout session) 2. focus on energy conservation |
| Which muscles are weaker in patients with muscular dystrophy ? | proximal more weak than distal. |
| Is muscle tone high or low in patients with muscular dystrophy? | LOW |
| What is a sign that occurs in patient's calves which may give you a false impression that they are strong? | Pseudohypertrophy |
| Is sensation affected in muscular dystrophy? | NO |
| Is the sphincter function affected in muscular dystrophy? | No |
| What are the two categories of muscular dystrophy? | hereditary and acquired |
| What are some common hereditary mypathies? | 1. congenital myopathies 2. muscular dystrophies 3. myotonias and channelopathies 4. primary metabolic myopathies 5. mitochondrial myopathies |
| What are some acquired myopathies? | 1. inflammatory myopathies 2. drug-induced and toxic myopathy 3. secondary metabolic myopathies 4. endocrine myopathies 5. infectious myopathies |
| What is the most common and severe form of childhood muscular dystrophy? | Duchenne Muscular Dystrophy |
| T/F: DMD is X-linked. | T. |
| T/F: All DMD are hereditory. | F. about 30% spontaneous mutation |
| What is going on at the muscular level of DMD? | 1. slow progressive muscle weakness 2. fibrosis, degeneration and regeneration 3. proliferation of fatty and connective tissue 4. decreased in dystrophin |
| T/F: DMD is symptomatic in newborn. | F. normal at birth |
| When do the kids with DMD start to have delayed motor milestones? | At 1 y.o |
| What are some signs that will likely surface during years 3-7? | 1. toe walking 2. Gower's sign 3. Lordosis 4. Scoliosis |
| When do kids with DMD typically stop walking? | 7- 10 years |
| Current life expectancy for DMD patients? | in the 20s. |
| Reason for death in DMD patients | fatty infiltration of heart and respiratory infection |
| what are the patterns of weakness in muscles? | 1. proximal weaker than distal 2. pseudohypertrophy in calves |
| What are some clinical signs/ symptoms of DMD? (CCC DOGS) | Contractures Cognitive deficits Cardiac dysfunction Decreased respiratory capacity Obesity GI dysfunction Scoliosis/ lordosis |
| What are some possible types of diagnostic tests for DMD? | 1. serum enzyme levels 2. muscle biopsy 3. genetic testing |
| Which markers are abnormal in DMD patient's serum? | Elevated creatine kinase |
| What are some pathological changes observed in muscle biopsy? | 1. variations in size of muscle fiber 2. fat and connective tissue deposits 3. dystrophin reduction/ absence |
| Which gene is abnormal in DMD patients? | dystrophin. (chromosome Xp21) |
| What is the goal for medical management for DMD patients? | - maintain the highest quality of life achievable, at each stage of the disease. - slowing rate of progression - prevent contractures/ deformity - maintain function and participation - allow compensations - prevent pulmonary infections, secretion management and airway clearance - manage scoliosis - monitor cardiac functions |
| Which medication can prolong walking for up to 3 years in DMD patients? | Prednisone (0.75 mg/kg/day) |
| describe the expected level of function at the following ages for DMD patients: 1. 1-10 2. 10 - 13 3. 13 - 17 4. 17 - 20 5. 20 - early 20s 6. 20s - 30s | 1. 1-10: pathological gait 2. 10 - 12: wheelchair (skeletal deformity) 3. 13 - 17: very limited use of arms 4. 17 - 20: ventilation at night 5. 20 - early 20s: 24 hr ventilation 6. 20s - 30s: death |
| What should we encourage/ discourage during clinical treatment for patients with DMD? | Encourage: assistive technology, orthosis, daily stretching, standing program Discourage: resistive exercise, eccentrics |
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