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88 Cards in this Set
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What is the anatoomical difference between upper and motor neuron diease and lower motoer neuron disease?
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patients with upper motor neuron involvment have pahology proximal to the anterior horn cell. LLower motor neuron diease begins at the anterior horn cell and extends distally to inclde the peripher nerve, neuromuscular junciton and myfibril
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Describe hte baseic nature of upper motor neuron disease
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THe basic nature of the upper motor neuron diesase involves spacticity. Patients my or may not have sensor loss . Ther ie relatively good preservation fo the soft tissues since the anteiro horn cell has been preserved.
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Whic h neuromjuscular diease characteristically shows mixed upper and lower motor finding.
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Amyotorphic lateral sclerosis.
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Describe ht basic nature of lower motor neuron diease.
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Lower moter neuron diesease involves flaccid paralyuis. It is charaterized by normal to low tone and significant muscle wsating since the nerve supply to the muslce has been interrupted. Thre is aols o sensor y loss and an absence of spasticity
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Name some common cause s of lower motor neuromuscular diease and ther leveol of involviement.
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From most proximal to idstal:
Spinal cor- anterior horn cell: Poliomyelitis, wernig hffman, spina muscle atropy, spina bifida Perpheral nerve: diabetes mellitus, vitamin deffiecncy (B1 , b6, B 12 most common) direc trauma, Charcot Marie Toothe disease Neurmuscular junction: myasthenia gravis, post poli syndrome Muslce (myopathy): muscular dystrophy, polymositis, dermatomysistis Myotonic dystrophy (adult muscular dystrophy) |
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What is the most common mucualr dystorphy in childhood and adults and how are they ineritid
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The most common musuclar dystropy in childhood is Duchenne' muscular dystrpy occuring 1 in 3500 newborn males by sex-linked inheritance. Becker's muscular dystrophy is also sex - lined and occurs in a bout 1 in 35,000 neborn males. On the molecular level, Duchenne's and becer' muscular dystropy is caused by a mutaiton i nthe dystrphin gene which codews for the dystrphin muscle proeing whis is dormally present it tnhe cell menbrane of normal muslce.
The most common muscular dystropy in adult si myotonic dystropy that cocurs by dominant inheritance. On the molecualr level myhotonic dystrophy occurs as a mutation on chromosome 10 and is dupicaiton of the cTG repeats. In non affect indiviuals thera are up to about 30 CTG repeats. IN indivuals with myotnic dystrphy thee number CTG reapeast in increase dramatically. |
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How is Duchenne muscular dystrophy diagnosed?
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It is intially suspect due to delayed ambulation and is always eveident by school age. Laboratory testing reals at least a 10 fold increase in serum creatinie phosphokinase (CPK). Muscle biopsy shows and absence of dystrolphin, na bnormal variotion of muscle cell size , nad an increased in connective and fat tissue. DNA testing is availabe to diagnose Duchenne muscular dystrophy and shows a mutation in the dystrophin gene. The dystrophin gene is one of hte larges genes which is why it is very vulnerable to mutation.
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How does Becker's muscular dystroph differ from Duchenne' muscular dystrophy?
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Becker's musuclar dystroph is more benign, occurs at a later age of onset, and progresses more slowly. Ther is a greater than five-fold increase in CPK and patients typically do not become wheelchair bound until theri teens. Patient with Becker's mucualr dystrophy frequently survie into theri fifth and sixth decade. However, htere is no diffenvce between Duchenne' muscular dyatrophy and Becker's muscualr dystroph on mucsle bipsy . Both diease are inthetid sex linked
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How ar eht muscualr dystorphie characterize?
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low tone, poroximal muscle weaknes, and calf hypertrophy
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How is myotnic dystrophy differnet form other muscular dystrophies?
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myotonci dystrophy is characterized by distal muscular weakness and is the only muscular dystrphy that may present with a foot drop.
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What are some addition problems that patient with muscualr dystrophies ofent have beside the primary mucle involvent?
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cariomyopath is common in sex-linked muscualr dystrophy. endcrine abnormalities are common in myotnic dystropy. Mental reatardation is associated with muscular dystropy.
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Name the 3 basic forms of myotonic dystrophy
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The classical form is the adult onset and characterized by myotonia and weakness of the distal muslces of the arms and legs iwth ocular and pharyngeal weankness and early onset of catarcts.
There is and early childhood form with onset between ages 5 and 10 years which is characterized by myotonia, mental retardation, and geralized weaknes. The most severe form of myonic dystrphy is the congential form which presents with sever neonatl weakness or stil birht. |
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What is the basic nature of inflammatory myopathies?
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infalmatory myopathies present with normal to low muscle tone and z proximal symmetircalmotor weakness. Patients have muscle soreness and terndernes as well as markely elvated CPK levels, in ohter words, similar presentation to muscular dysrophhies
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What ocnditions are inflammatory myopathies assciated wht?
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interstial lugn diease, cariomypathies with arrhytmias, esphageal dysmotility, and various autoimmune syndromes.
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Describe the orhtopedic manifestaions of neurofibromatosis.
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neurofibromatosis is a deiase process characterized by cafe ual lait spots, subcuatneous nodules and neurofibromas. There may be soft tissue hypertrophy which can involve on extremity on the entir half fo the body. The most importnat skeletal manfiestions are scoliois and congeital pseudoarthrosis of the tibvia. Frequnelty. The family hisotry is positive for this disorder. Crowe's critera are used to diagnose the ocndition. Two of the following should be present:
1) a positive family history 2) a positive biopsy of a lesion 3) a minmum of 6 cafe au lait spots, greater than 1.5 cm in diameter or 4) multiple subctaneuos nodules |
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Give a gernal description of poliomyelitis.
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Pliomyelits is an infection caused by Type I, Type II, or Type III poliomyelitis virus, a memboer ogt the nterovirus gorup. The virus gains entry into the body through the gastrointestingal or respiratory tract, casuing an acute infectious precess which has a special affinity for the naterior horn cells of he spinal cord as well as for hte bulbar nuclei of hte brain stem. Infection cause necrosis of these cells with loss of innervatio n to the motor units. Sensation remains intact. Widespread prophylactic caccination has greatly reduced the incidence of htis disease, but patients are still occasionally seen due ot the lack of proper immunizaiotn.
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Discuss the differential diagnosis of a patient with muscualr dystrophy.
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The diffrential diagnosis of a specific tyuep muscualr dystrophy is usualy based on clinical findigh MYUOTONIC DYSTROPHY is hte only myopathy characterized by distal muscle weakneks.s Findings other than myonia includ temporalis and masseter atrophny which causes a skull like facies and cataracts and testricular atrophy and premature forntal bladness n males. Common to all muscualr dystrophis is sparing of the neck extensors , gastronemius, psoterior tiebial and toe flxors.
DUCHENNES' S MUSCULAR DYSTROPHY IS CHARATERIZED BY EARLY U NIQUE INVOLVEMENT OF HTE NATERIOR NECK FLEXORS, STERNOCLEIDOMASTOID, ANTERIOR ABDOMINAL MUSLCES, GLUEUS MEDIUS AND Tensor fascial late. Nthe sohoulder , the lower and middle trapezus are nvolved, as are the rhomboids latissimus dorsi and interal rotators. The hamstirngs in Duchenne's are uniquely spared, which is an important finding. In LIMB-GIRDLE MUSCULAR DYSTROPHY , the early unique involvemnt is mainlyu the iliopsoas. Other msucles that tend o to be involved early are hte lower and middle trapezus , rhomboids, litissimus dorsi and internal rotators of the shulder. the glutus maximaus and the hip abducts are involved in teh lower extemiteis. Uniquely, the brachiradialis spared early in lomb-girdly dysrphy\ FASCIOCAUPULOHUMERAL DYSTROPHY is characterized by unique early involvemnt of hte upper pectroalis major and anterior tibialis muscles. There is more sparing early on in the is disease than in any of hter type of dystrphy |
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Discuss the diagnosis of a patient with Guillain - Barre Syndrome
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Guillain - Barre is a neuromuscular diease of acute onset and short duration charaterized by acute demyelination fo athe anterio ramus or motor division fo the spinal cord. The posterior ramus and the sensory divison fo the anteir horn are involved by retrograde extension onyhl. Clinically, patients present wth falcccid paralyis which usually begins in the upper extremities. Bulbar involvemnt may cause repiratory paralys necessitating tracheostomy. Characteristically the patient is bedfast for 5 to 8 weeks and 3 to 12 months elpsing before they are able to regain enough function to be independent. Recovery continues over the next year and is considered to have pateraued when the motor development does not change over a 16 month period of tiem. Inchildren, the pronosis is worse. Weak dorsiflexors iwth equines deformity one of the more characteristic problems. If there is no return in 4 months htere probably will be no return. poor intreinsic hand functin is the most frequent upper exttermity resdiual.
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Pateints with dermatomyistis have an increased risk of ?
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cancer especially of hte lung, gynecologic, prostate nd gastrointiesting which ranges from 5 to 50
5 in dermatomyosits |
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Describe the mangement of inflammatory moypathies.
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The mangement invilves pharmcologic agents uch as prendisione and immunospupresants and physical therapy.
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Nmae some of the causes of disease occuring at hte neuromuscualr junciotn.
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Myasthenia gravis, post polio syndrom, botulinum toxin, nerve gas and insectice all affect the neuromuscualr junction directly.
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How does myasthenia garvis affect hte nuromuscular junciotn?
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both presynaptically and postynaptically.
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How is diagnosis ofmyasthenia gravis made?
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dagnosis of mayshtenia gravis ois based on a positve tensilon test and th presence of serum antibodies
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What is the pharmacological treatment of maysthenia gravis?
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pharmacologic treatment ofr myashtenia gravis includes anticholinesterase inhibitors and immunosuppressants.
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What is ht ecause of post poli syndrome?
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psot poli syndrome is casued by inasddquate transmiions at he neuromuscular junction.
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What are the major peripheral nerve disease?
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focal
sytemic demyelinating or axonal |
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In parital sciatic nerve injureis , which of hte tow division sof th sciatic nerve is most affected?
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The town divison sof hte sciateic nerve are the peroneal nerve and the tibial nerve. In partial sciateic nerve injuries the peroneal nerve suually more severly affected, thus foot drop is almost always more comon than calcaneal gain itn perpheral nerve njuuries.
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Why is the peroneal nerve usually more severly affectd thant the tibial nerve in partial injuris of hte sciateic nerve?
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The peronal nerve is more perpheral thant the tibial nerve making it more vulnerable to njureis by injection Ther peroneal nerve is has less fibrous tisuse protect int. The peroneal nerve is injured more easily than the tibial nerve and een when both nerves are njured, the peroneal nerve is lsoer ot heal.
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What type of nerve diease is Guillain - Bsarre syndrome?
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inflammatory neruopathy, systemic with demyelination and axonal components.
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What ar eth eclinical mafiestationfo Gillain - Barre syndrome?
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usually acute onset with weakness, atrophy, and sensory loss.
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What is the most common peripheral neuropathy?
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Diabetic peripheral neuropathy.
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Describe diabetic peripheral neuropathy.
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Diabeti perpheral neuropathy involves all extremities, withth distal lwoer extemities bieng involved first. It is a stocking glove distribution with the sensory componet more sever thant th emotor compnent. It is an axonal neuropathy.
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What is the isolated severe involvement of only a few nerves with realtive sparing of ther nerves which occurs n diabetes mellitus known as?
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monomeuropathy multiplex.
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What are some dieases associated with immune peripheral neuropathies?
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rheumatoid arthritis, systemic lupus erythematosus, payareterits nodisa, amyloidosis
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How is the diagnois sof immune perepheral neurpathy made?
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Diagnosis is based upon clinical presentation, elecrodiagnoesi, and frequently requires bipsy of nerve and musle especially for chronic forms.
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What is the treatment of immune peripheral neuropathy?
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treatment is pharmacological and depends upon specific etiology, but tyhpically includes nsoteroidal anti-inflammatory drugs, prendisone, and other immunosuppressants. Plasma exhange may benefit some patients.
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What is the most common inheritied peripheral neuropathy?
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Charcot Marie Toothe diease also known as hereditary motor sensory neruopathy? (HMSN)
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Describe Hereditary Mostor Sensory Neruopathny (HMSN) type I.
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HMSN I is a hypertrophic and dmyelinating neuropathy whic hresults in significantly slowed nerve conduction velocity. INheritance is autosomal dominant.
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Describe Hereditary Motor Sensory Neuropathy (MSN) TYpe II.
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HMSN II is a a predominately axonal neuropathy with only minimal slowing of nerve conduction velicites but with signifcant reductio nof evoked potential amplitudes. INheritance is autosomal dominat.
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Describe Hereiditary Motor Sensory Neuropathy (HMSN) TYPE III
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HMSN TYpe II also know asDejerine-Sottas Disease is a seve demyelnatin nonprosseiveneuropathic disease that manifest itself in infancy. Weaknes sis diffuse and and more pronounced distally. Motor milesontes are delayed. Inhertan ce is dominant and recessive
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Name the hreditary sensor neuropatheis.
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Friedrich's ataxia, ataxia telancictasia, Tangier's diseas and Gabry' sdisease. Thes ediease are rare hereditary diease and charaterized by ataxia, long tract signs acardivasular diease and visceral involvement.
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Where is the level of Cauda Equina Syndrom
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Intraspinal nere at or below L1. tHE SPINAL CORD ENDS ATHE L1 LEVEL AND INUIES AT OR BELOW l1 AFFECT THE CAUDA EQUINA NERVES SINCE THE NERVES ARE not actually part o fthe spinal cord, lesions of hte cauda equna regerate and heal as perpheral nerve injury. Thus a person with cauda equina syndrome may have total paralys from the wasit down, but with time and physical therapy, hte nerves will regereate and the person will walk again. THis is in contrast with lesion so fhte spinal cord itself which are part of the central nervous system and do not regenerate.
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What are the clinical patterns of spinal cord disease?
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Depending on the levael nd type of injury they may be upper motor neuron, lower motor neruon, or mixed.
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Describe the Spinal muslce atrophies.
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Spinal Muscular atrphy (SMA) type I knon as Werdni-Hoffman and SMA type II affect he naterior horn cell of the spinal cord directly. THey manifest themselves in infancy and in early childhoo dna result in early death
SMA tye III (Kugleberg-WElander) occrus much later int eh childhood and progresses more slowly. SMA tyep IV is the adult onset spinal muscualr atorphy with onset int he thrid to fifht decade. THe diesase is lwoly progressiv eand life spanis unaffected. |
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How are the spinal muscular atrophies diagnosed?
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D
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Describe amyotrophic latral sclerois (ALS)
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ALS (lou gerhig's disease) is an adult motor neuron disease which is a nonfamiial disease of middle age. Distal extremity weakness, partiuclarly in the hands, is a frequent presentign complaint. Weakness preesses proximally and patients eventually have crial (bulbar) involvemnt. Muscle fasculaiton and atrophy are common. Uper motr neuoron fidngs include hyperreflexia
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How is amyotrophic lateral scerosis dignoses?
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diagnosis is based on clinical presentation and elctrodiagnosis and biopsy confirms a neuropathic preces
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What is the course of amyotrophic lateral scerosis?
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the disease is progressive and dath occrus 3 to 5 years after diagnosis
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What arethe sequelae of postoperative menngomyelocels
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leg-lenght discrepancies, contractures, seve atropy, sensor deficits, neurogenic bowel and bladder.
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how does intracranial disaes affect the lower extremity?
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gernally with spacticity. Middle crebral artery lesions affect hte distal extrejmity muscle. anterior crebral artery lesion saffect htre proximal extemity muscles.
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What is the most common cause of itnracraial disease in children?
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crebral palsy.
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What type of crebral palsy is seen in premature bbies?
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spastic diplegia
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Wwhat is the cuase of spastic diplegia cerebral palsy?
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paraventricular ischemia/hemorrhage
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name some exmaples of acquire intracanial patholgy
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stroke, brain injury, multiple sclerosis, brain tumor
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What is the distribution f muscle weaknees iin nuroptayhy
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distal weakness
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Whata re the electrodiagnostic findings in myopathic dieases
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small polyphasic poeitnials with a yopatihic (early) recuritment pattern and if sever positive eaves and fibrillation potienticsl
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What does nerve conduction velocity (NCV) study considt of
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THe NCV consisists of the gbeneration of the wensory nerve action potiential (SNAP) of sensory nerves nad the compond muscle action potiential (CMAP) of motor nerves. This study consists of the application of a burs of electrical current to the skin or soft tissue overlying a peripheral nerve and recordingof he susequent compound action potential from the skon or soft tissue oeverlying the nerve a t adistat site.
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What is the cause f arthrogyphosis
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early intrauterinie paralysis
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What is spasticicty?
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Spasticity means htat stretchy relflesex limitt the range and speed of movement due to an uppoer motor neuron lesion . Because the muscles are never able to lenghten fully they grow less than they should and fixed deformity develps. Teh boens become abnormally shaped.
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What is the incdence of crebral palsy?
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about 2 per thousand lvie birhts.
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What are the basic kinds of cerebral pals?
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Spastic diplegia isn paralys of both legs and is themost common pattern for preatuer and low birth weight infants. Spastic diplegic ooccurs shroly after birht. Total body involvment affects pseech, trun, feeding and arms with no walking potientisal.
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Describ eht "total body iviolvemnt" pattern of cerebral palsy
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The case is due to birth asphyxia or prenatal encephalopathyl.. The schildren never walk and have difficulty swallowing and feeidng is ddfficult and slow. The are thin. Gasgtric reflux may cause choking and aspiration pneumonia. They may require diapering. They have no balnce and cannot sit up independently. The limbs remain immobie and contractreus develop raildy. The incidenced ohip dislocatio nand sociosi is greter than 50%.
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What happens in spina bifida?
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In spina fifida the neural tube fails to close uing th 4th week. Teh is paralys of he legs, anestheia , and bowel and bladder paralysis =to some degeee. If hte cord supplying hte legs is destryed thre is comple flaccid paralyis
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Wha is ht eincidence of spina bifida?
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1 in 700 babies are born withspina fifida. The chace of having a second baby with spina fifida is 1 in 40.
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What tiests prenatally can detect spian bifida?
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alpha fetoprotien dietermination in maternal blod and amniotic fluid, and ultrasound
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How is theneurological deficit classfied in spina bifida?
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Spina bifida is calssfied by te level of the lesion. The level of the last fucntion root is used to describe hte level of ht paraplegia. For example if L1 is the last working root, the child is descreibed as having L` prapplegia.
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Why is htere ovleap beween the central nervous system and the prepheral nervous system.
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The is overlap because the spinal cord is classically part of the central nervous sysstem , but contains elements of the pereipheral nervous system (eg. thenaterior horn cells or nerve roots. )
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A piainful sensation fo tingling , burning aor cold is known as Dysesthesia.
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dysesthesia
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allodynia
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What is a painful sesnastin casued by non-noxixiousl stimuli, for example , bed sheets rubbing agaisnt the feet producitn sever pain in patients with perpheral neuropathy.
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What is theMedical research council (MRC) scale of msucle strenght that was develped durong wolrd war II
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full muscle power
resistance agianst force resisntance agianst gravmity movement with out gramvity twitch no movement |
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what is bilateral foot pain, numbness,and waknkess in a disrtribution of a prerripheral nerve associated withn?
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pewripheral neuropathy
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What are some caue of disuse atrophjy?
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both nervous patrhology and ano nervous pathology can reuslt indisue atrophy. Immmobiilty by casting, bedrest, arthritis, nd stroke can all result in diuse atrophy.
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What is clonus?
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clonus is ht erepetive movemen t fo ahnd foot or similar body part when the tendon is tretched. It is and UMN sign. sometimes patients complaint that " my foo tshakes wehn I waalk"
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What is the babisnki sign
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UMN sign. great toe godorsiflexes whn latar lant aspect of foot is scratch
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Ipsilatera absent ankle jer, gstocnemiu atrophy and weaknes mostly in plantarflexion suggest
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S1 nerve root lesion
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What are the hallmark reature of perpheral neuropathy
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numbess, tingling, pian, wankess , an d areflexia. Atrophy occurs wehnthe ocniditon si sever or prolonged.
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What casues a demuyelinating neruoay.
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destruction mfo myelin, weakness and sensory loss proxima or distal tosite of demyelination.
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What are the halmark featurs of peripheral neuropathy?
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numbness, ntigling, pain, weakness,and areflexia. Atrophy occurs whenthe ocndition is severe or prolonged.
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What caues a demyelinating neuropathy?
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A demyelinating neuropathy is cased by loss or destrctionof melin. The weakness and sensory loss may be proximal or distal depdnign on th esite of demyelination.
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What is the msot common cuase of acute demyelination?
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Guillain - Barre Syndrome
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What is a weidspread neuopathy in which only a few preripheral nerves are affected?
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mononeuropathy multiplex.
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is a classic pathologic process associatd with mononeuropathy multiplex?
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vasculits.
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What are the characteristic features of readiculopathies?
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In radiculopathies, the pain is ususally, posistional, worswe while standing or with prolonged sitting, and impovedin the supine position. Increasing the intra-abdominal pressure by coghing , sneezin or th valsalva maneuver aggravates the apin. Sensory loss i sin a dermatomal distribuitonand weakness is in a myotomal distribution. Radiculaopathies generally casue more pain thatn weakness and occur in young adults.
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Whata are charcteristics of anterio horn cell disorders
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they casue painless weakness.
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What is themost comon anterio horn cell diease in adults?
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AMYOtrophic lateral sclerosis
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What are the basic charcteristics of spianl cord dieases?
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melopathies or dieases of the spinal cord, typically cause limb weaknes, sensory symptoms, urinary incontience nad back pain. pure spinal cord diease isusually spastic.
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What is reflex syamptheitic dystrophy?
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Reflex sympathetic dystrophy is defined as continuous pain in a portion of an extemity after taruma that may inlude fractuer but does not involve a maor nerve associated with sympathtic hyperactivity.
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What are some signs of REflex Sympathetic Dystrophy?
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Atrophy of skon on arms and leg, cool, red , and cllammy skin, disuse atrophy of deep structures.
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