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Flashcards in Neuromus Disorders 2 Deck (41):

Muscular Dystrophies & Atrophies

Deg disorders resulting in muscle weakness & decreased muscle mass d/t hereditary disease process - d/t absent muscle protein product: dystrophin; ^ levels of creatine kinase (CK) in blood - diff between congenital myopathies


Muscular Dystrophies & Atrophies diagnosis

Can begin at any age, may not show symptoms until 2.5y/0; ave age for dx is 5 unless known fam hx > progress may be rapid/fatal or remain stable


Duchenne's MD

Most common form of MD; detected 2-6y/o; inherited, sex linked & recessive occurring in males > rarely live past 20s


Duchenne's MD Symptoms

Pseudohypertrophy; weakness of prox joints progressing leading to signif fx'al mob impairments including: trendelenberg (wadling) gait, Gower's sign (using hands to crawl up thighs to get to standing); weakness of all vol muscles including heart/diaphragm; behavioral/learning & sometimes speech diffs



enlargement of calf muscles (at times forearm/thighs) giving appearance of being muscular/health


Becker MD

Variant of Duchenne MD - slower to progress, less severe & less predictable


Becker MD Symptoms

Loss of motor fx of hips/thighs/shoulders & pelvic area, enlarged calves, cardiac system can be involved > survival can be into late adulthood (nearly normal if min cardiac involvement)


Arthrogyposis Multiplex Congentia

Detected at birth and associated with loss of ant horn cells; May be stable, mildly progressive or improve; related probs: cardiac defects, spinal defects, torticollis (neck twist to one side w head tilt) & involvement of diaphragm


Arthrogyposis Multiplex Congentia Symptoms

Presents of weakness, deformities & joint contractures; in rot of shoulders w elbow exten and wrist flex at rest & flex/in rot of hips & club feet


Limb- Girdle MD

Onset between 1st-3rd dec of life. Prox muscles of pelvis & shoulders initially affected > typically progresses slowly


Fascioscapulohumeral MD

Occurs in early adolescence, involves face, U arms & scap region > causing masking, weakness, decreased mob of face & inability to lift arms above shoulder level > as (slowly) progresses weakness can extend to abdonm/hip muscles. Rarely affects cardiac/resp muscles = can be norm lifespan


Spinal Muscular Atrophy

Caused by a decrease in a MN protein (SMN= survival of MN), chromo 5; weakness of vol muscles of shoulders, hips, thighs & U back which can cause spinal curves; muscles for breathing/swallowing can be affected > earlier detection/greater severity of fx'al def leads to decreased life expectancy


Spinal Muscular Atrophy Types

I birth/infancy= Werdnig-Hoffman has life expectancy up to 2y/o; II children= intermed form is detected 6mo-3y and progresses rapidly w lift expectancy of early childhood; III older children= later onset, less severe; IV: adolescent/adult=later onset, less severe


Congenital Myasthenia Gravis

Disorder involving transmissions of impulses in neuromus junction > onset at birth and occurring more in males


Charcot-Marie Tooth Disease

Disease involving peripheral nerves marked by progressive weakness, prim inperoneal and distal leg muscles > typ in teen/earlier years



Similar to dystrophies but progress slowly resulting in better prognosis > weakness of face, limbs & neck are characteristic


Specific Symptoms for Dystrophies & Atrophies

Low mus tone/weakness contribs to abnorm mvmt patterns & delayed milestones, may be diff w oral motor . g-tube, deformities, diff w breathing > tracheostomies/vents


Med Mngt for Dystrophies & Atrophies

Meds to decrease pul/card comps, nutritional mngt (wt loss d/t inactivity), prevention of skin breakdown, steroids


Progressive Supranuc Palsy

Manifested by loss of vol but preservation of reflexive eye mvmts, bradykinesa, rigidity, axial dystonia, pseudobulbar palsy & dementia > occurs in later mid life w death in 15yrs


Huntingtons Chorea

Autosomal dom disorder, begins in mid age/ Characterized by choreiform mvmts and progress intellectual deterioration > psychiatric disturbance may precede mvmt disorder


Cerebellar/Spinocerebellar Disorders

Ataxia, dysmetria (under/overshooting), dysdiadochokinesia (inability to perform rapid/alternating mvmts), hypotonia, mvmt decomp tremor, dysarthria & nystagmus


Structural Cerebellar Lesions

Includes strokes (vascular lesions) and tremor deposits, producing symptoms/signs appropriate to locus w/in cerebellum.


Spinocerebellar Degen

group of degen disorders characterized by progressive atxia d/t degen of cerebellum, BS, SC, peripheral nerves and BG.


Friedrich's Ataxia

Autosomal recessive inheritance; onset in childhood or early adolescence. Prototype of spinal ataxia. Characterized by gait unsteadyiness, UE ataxia, & dysarthria; could include: tremor (but minor), areflexia, loss of large fiber sensory modalities > scoliosis and cardiomyopathy care common with progession


Cerebellar Cortical Degen

Onset between 30-50 > changes seen in cerebellum & inf olives


Multiple Systems Degen

Aka olivopontocerebellar artophies; characterized by spasticity, extrapyramidal, sensory, LMN & autonomic dysfunction


Amyothrophic Lateral Sclerosis (ALS)

MN disease characterized by progressive deg of corticospinal tracts & ant horncells or bulbar efferent neurons. More prev in men, onset usually about 57, death usually 2-5 after dx.


Symptoms of ALS

Mus weak & atrophy (evidence of ant horn destruction) often begins distally and symmetrically. Cramps & fasciculations (brief spontaneous contraction) precede weakness. Signs usually begin in hands. LMN signs are soon accompanied by spasticity, hyperactive deep tendon reflexes, and evidence of corticospinal tract involvement. Dysarthria & dysphagia. Sensory systems, eye mvmts & urinary sphincters are often spared.


ALS Fx'al Rating Scale

Symptoms quantified include: speech, salivation, swallowing, handwriting, cutting food, dressing/hygiene, turning in bed, walking, climbing stairs, dyspnea (SOB), orthopnea (SOB lying flat), resp insufficiency,# of years w symptoms


Tx for ALS

Aimed at tx secondary complications such as spasticity, prevention of aspirations, prevention of decubiti/contractures and paint mngt


Brachial Plexus Disorder Causes

2nd to traction during birth, invasion of metastatic cancer, after radiation tx 2nd to fibrosis or traction injury


Brachial Plexus Disorder Symptoms

Mix motor/sens d/o of corresponding limb, Rostral injuries produce shoulder dysfun while caudal injuries produce dysfun of hand


Erb's Palsy

Paralysis of U Brach Plex including 5th/6th CNs, C& sometimes involved. Muscles most often paralysized will include: surpra/infraspinatus, deltoid, biceps, brachilais & subscap > arm cannot be raised, elbow flex is weakened (protraction/retraction of scap may be weak too); Arm presents w arm straight & wrist fully bent (waiter's position). After 6mo contractures may present.


Kulmpke's Palsy

Paralysis of L Brach Plex including 7/8th CNs & 1st thoracic nerve. Relatively rare, results in paralysis of hand/wrist often w ipsialat Horners syndrome (miosis, ptosis & facial anhidrosis)


Simple Partial Seizures

abnorm electrical impulses occur in localizes area of brain (often motor strip of frontal lobe). Invol, repetitive jerking of left hand/arm but can maintain interaction w environment > if becomes more generalized= LOC


Complex Partial or Psychomotor Seizures

Symptoms vary; alterations in consciousness and unresponsiveness. May appear daze/confused. Automatic/repetitive movements occur. Visual/auditory sensory occurs just prior


West Syndrome - Infantile Spasms

Infantile myoclonic seizures or jackknife epi. Begins at 3-9mo. Drop of head and flex of arms occur. Seizures occur 100x a day and prognosis is poor. Spasms sometimes decrease but are often replaced by another type of seizure d/o


Lennax-Gastaut Syndrom

Children w severe seizures, MR and specific EEG pattern. Seizures of diff types occur for first 3 years and are hard to control.


Landau-Kleffer Syndrome

Acquired epi aphasia. Progressive encephalopathy, loss of language skills, auditory agnoisa


Simple Febrile Seizures

Most common seizure type precipitated by fever. Seizure lasts less than 10 and includes LOC and invol gen jerking > usually do not cause damage or lead to epi


Status Epilepticus

Prolonged seizures or in rapid succession. Can be life threatening; sometimes triggered when abruptly stopping meds. Typically occurs w tonic-clonic seizures that are not well controlled.