Mechanisms of Disease -Myopathies

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what are the 4 main myopathies discussed?: inflammatory
dystrophic
metabolic
corticosteroid
what are the two neuromuscular junction (NMJ) diseases discussed?: Myasthenias Gravis
Botulism
What Neuropathies were discussed?: median, ulnar, radial, peroneal nerve entrapment
what two forms of headaches were discussed? (besides the one you get from class): cluster
subaracnoid hemorrhage
what are the two inflammatory myopathies we covered?: dermatomyositis
polymyositis
name the myopathy: *presents ages 5-14yo *malar or heliotrope rash *edema of periorbital skin: dermatomyositis- an inflammatory myopathy
pseudohypertrophic: Duchenne MD
Rubbery Calves - disease and cause of: Duchenne MD
from fat replacing CT
subsarcolemmal cytoskeletal protein: Dystrophin
what disease does this pertain to: LABS sed rate, CPK-MM,Aldolase all increased TX: Steroids and inmmunosuppressants: dermatomyositis and polymyositis inflammatory myopathies
most common dystrophy- especially kids and more common in bosy: Duchenne Muscular dystrophy
Stabbing, penetrating, burning, explosive pain ONLY on one side of head Starts at eyes and spread to same side of head: cluster headache
secondary symptoms of cluster headache: stuffy or runny nose
redness or watering eyes
droopy eyelid
constrx of pupil
facial swelling
what disease? presents age 50-60 yo can be viral, bacterial, idiopathic: polymyositis
what disease? *axial muscle wkness *phonation and deglutination affected*dysphonia & dysphagia: polymyositis
in what disease is abscence or abnormal levels of Dystrophin an issue?: Duchenne MD, dystrophinopathies
T/F? dystrophin protein is absent in all types of dystrophies?: FALSE, the "muscular dystrophies" have abnormalities with the muscle, not the protein
innervates skin of hand flexor carpi digitorum hypothenar muscles: ulnar nerve
results to Gower's Maneuver: Duchenne MD, when unable to walk
innervates flexor abductor digitiminimi 3rd and 4th lumbricals interroseous muscles: ulnar nerve -
also innervates flexor carpi ulnaris
combined the lumbricals and interosseus help to: flex the MCP joint
extend the IP joints
compression of nerve and transverse carpal ligament: median nerve entrapment and carpal tunnel
foot drop: entrapment of peroneal nerve from wearing too tight boots
difficulty grabbing and opening a jar: symptom of carpal tunnel syndrome - median nerve injry
ape hand: median nerve entrapment
innervates skin of hand and most flexor muscles that flex fingers and wrist: median nerve
Claw hand: injury of ulnar nerve
Labs: CPK-MM increased to 50x Nl fibrosis necrosis and Fat infiltration seen on biopsy: Duchenne MD
abscence of this protein causes wkness of sarcolemma and permits rupturing of muscle: Dystrophin - sarcolemmal cytoskeletal protein
walks on toes, contracture of achiles tendon, waddling gait: Duchenne MD
onset around age 4 ambulatory loss by age 8-12, death in teens or 20's: Duchenne MD
signs/symptoms of carpal tunnel: pain and paresthesia in wrist and hand
pain may radiate to forearm, elbow, shoulder
increased by flexion and/or extension of wrist
wkness of grip
weak thumb abduction and opposition
restrictive pulmonary deficit from progressive kyphoscoliosis ----disease?: Duchenne MD
the most common nerve entrapment injury: carpal tunnel syndrome
causes of carpal tunnel: repetitive stress
excessive use of wrist
CT thickening of the ligament
**arthritic tenosysnovitis
symptoms include generalized muscle cramps and early fatigue from exercise - lack of energy: McArdles metabolic myopathy- due to inability to break down glycogen to glucose for energy
pain and paresthesia in wrist and hand and first 3 1/2 fingers: carpal tunnel syndrome - median nerve entrapment
corticosteroid myopathy: complications of high dose steroids
Cushing's Syndrome
what treatments are available for carpal syndrome: splint wrist in neutral position
-steroid injection
-surgical decompression
division of carpal ligament
subsarcolemmal glycogen vacuoles seen histologically: McArdles-metabolic myopathy
treatment is limited to physical therapy and bracing: Duchenne MD
Tinsels test is diagnostic for: carpal tunnel syndrome
thenar atrophy is a late sign: carpal tunnel
caused by myophosphorylase deficiency: McArdles disease
what is Phalen's test?: carpal tunnel compression test -
an endocrine disorder in which the adrenal gland overproduces corticosteroids: Cushing's syndrome
causes muscle catabolism, atrophy of fibers, wkness in shoulder, hip and other areas: Cushing's Disease - a metabolic myopathy
symptoms can include: dysphagia, dyspnea, nasal speech, smile degeneration to snarl: myasthenias gravis
what gland is a major player in Myasthenias Gravis?: Thymus gland - it produces the Ab that kill the Nm receptors causing rapid fatigability, and motor deficits
describe in order the affects of Myasthenias gravis on the body: 1. eyelid-ptosis
2. extraocular - diplopia
3. bulbar muscles
-nasal speech
- jaw hangs open
4. dysphagia
mimetic muscle inactivity
smile degenerates to snarl
intercostal musclse and diaphragm depressed
death can be caused from respiratory muscle depression - cant take another breath in: botulism poisoning
rapid fatigability ptosis diplopia bulbar muscles affected: Myasthenias Gravis
bacteria that cx foamy frothy food: Clostridium botulinum - an anerobic bacteria implicated in Botulism poisoning
deep tendon reflexes or sensory fx not affected but muscles of facial expression are: myasthenias gravis - a NMJ disorder
treatment includes discontinuation of steroids: Cushing's syndrome
treatment includes pyridostigmine, plasmaphoreses, IV gamma globulin and immunosuppressants: Myasthenias gravis - NMJ disease
some symptoms of this disorder include: acute progressive wkness w/in 72 hours -alpha motor neuron and parasympathetic terminals affected -dilated pupils -dry mouth: botulilsm toxin poisoning - a NMJ disorder
'index muscle' is used for diagnosis of this: ulnar nerve lesion
arthritis tenosysnovitis: implicated with carpal tunnel syndrome
bilateral damage, may be an issue related to systemic conditions, diabetes, alcoholism: polyneuropathy
this causes loss of deep tendon reflexes (DTR's): botulism
unilateral affects: mononeuropathies
risus sardonicus is a rsult of ....: tetanus- Clostridium tetani
T/F, Tetanus is not a NMJ disease?: True, it is a result of bacillus bacteria invasion of the brain and spinal cord
destruction of cholinergic axon terminals causing a decreased release of Ach is a sign of: Botulism poisoning
what is the treatment for Botulism toxin?: IV antiserum
cardiorespiratory support may be needed
Bilateral damage distal sensory nerves Distal motor nerves Bottoms of feet are 1st, Dorsum and ankles Calves and/or hands: polyneuropathy
affects of this include blocking of inhibitory neurons, hyperreflexia, and muscle spasms: Clostridium tetani, Tetanus
this affects the cubital tunnel: ulnar nerve
this includes entrapment of compound nerves: ulnar, median, radial, peroneal and tibial: mononeuropathies - where nerve entrapment is the key issue
this nerve is implicated in 'saturday night palsey': Radial nerve entrapment
this can be seen and palpated on the thumb side of the 2nd metacarpal: index muscle - diagnostic for ulnar nerve lesion
symptoms include difficulty spreading fingers, 1st dorsal interooseus atrophy, wk or atrophied: ulnar nerve, cubital tunnel syndrome
prominent 1st dorsal interosseus atrophy is a sign of this: index muscle - early diagnosis of ulnar nerve paralysis
wrist drop: radial nerve entrapment
worst headache of my life: subarachnoid hemorrhage from ruptured cerebral aneurysm
innervates triceps, supinator, brachioradialis and skin of dorsum of hand: radial nerve
atrophy of lumbricals,interosseus and hypothenar eminence: ulnar nerve (mainly)
the MC cx of subaracnoid hemorrhage: rupture of a cerebral aneurysm
restlessness, agitation and nausea area result of this: cluster headache
signs are pain & paresthesia of dorsum of hand,fingers but not weak triceps, supination: injury at mid-humerus, from a blunt trauma
denervation causing muscle atrophy and tophic changes of the skin: atrophy of severe chronic denervation
this nerve innervates wrist and finger extensors and helps flex elbow and aids supination: Radial nerve
Lavisnky sign: take reflex hammer, stroked across ball of foot, and if upper motor neuron lesion in brain, the big toe flexes upward =
MC peripheral mononueropathy: proximal, middle,
which cranial nerves are ONLY sensory: CN I, II, VIII,
olfactory, optic, vestibulochoclear
smell, vision, parasympa to eyes: CN I, II, III
olfactory, optic, occulomotor
in a cranial neuropathy that affects CN 3, what is a deficit?: medial rectus muscle - ocular convergence
levator palpebral - troubel opening the upper eyelid = hanging eyelid
what causes hanging eyelid?: damage to CN 3, affecting the levator palpebral muscle
superior oblique muscle, action and innervation: CN 4,trochlear N. moves eye down and medially
olfactory N: CN 1
smell
Vision: CN 2, optic
parasympathetic to eye muscles: occulomotor CN 3
miosis: pupillary constrictor muscle CN 3
lens focus: CN 3, occulomotor
ciliary body
ocular convergence: medial rectus
opens upper eyelid: levator palpebral mucsle
superior oblique muscle action/nerv: occulomotor
moves eye down and medially
oronasal mucus membranes and teeth: sensory action from CN 5, trigeminal
corneal reflex: cornea - sensory of CN 5, trigeminal
facial nerve actions: para to lacrimal gland - tears
submaxillary and
lateral movement of eye: lateral rectus , CN VI
submaxillary and sublingal glands - innervation: CN , facial
motor to jaw muscles, masseter temporal and pterygoid muscles: CN 5, trigeminal
anterior 2/3 tongue, taste, pinna of the ear: CN 7, facial - sensory fx
obicularis orus closes eye in response to: CN 7, facial nerve motor function
facial expression: facial mumetic muscles
this .....muscle elevates the pharynx and larynx and is innervated by.....nerve: stylopharyngeus muscle - CN 9 glossopharyngeal
sensory from semicircular canals, saccule and utricle for dynamic and static balance: innervated by vestibulocochlear n
mydriasis/asnisocoria: pupillary constrictor, CN 3
ptosis: CN 3, levator palpebrae
what two cranial nerves fx in salivation: CN 7, facial = submaxillary and sublingual
CN 9, glossopharyngeal - acts at parotid gland - salivation
glossylpharyngeal ? sensory from: S from: posterior 1/3 tongue=taste
*orpharynx - swallowing
eustachian tube - middle ear pain
carotid sinus & body
CN 9, motor to: stylopharyngeal muscles-elevates pharynx and larynx
CN 10 - Vagus *para motor? * sympa motor to: para motor to abd & thoracic organs
*sympa motor - solft palate, pharynx, larynx
CN 10 is sensory from ?: aortic bodies and arch
posterior 1/3 tongue
pharynx, larynx, ear cancal
anosmia: rare
CN 1 olfactory - cant smell
what innervates the tongue?: posterior 1/3 = CN 10
anterior 2/3 taste-CN 7
CN 12 - motor to tongue
motor action of sternocleidomastoid and upper traps: Cn 11, accessory
gag reflex: CN 10. cagus - via pharynx and layrnx
the difference b/t diplopia and blurred vision: diplopia is seeing two images from non fx EOM's and blurred vision is from failur of lens to accommodate - both CN 3
herperacusis (loudness): stapedius muscle neuropathy - CN 7
unilatery palsy of front of neck when frowning, unilateral facial wkness: platysma muscle - and facial muscles from motor fibers of CN 7
sluggish or absent light reflex of eye: deficit to pupillary constrictor muscle of CN 3
jaw deviates to one side "sidedness": CN 5 neuropathy of masticator muscles casuing wk or malaligned jaw
Tic douloureux: trigeminal neuralgia
short bouts of exruciating pain in any of the CN 5 facial divisions
what can cause Diplopia ?: EOM not fx due to
1. palysy of CN
2. entrapment of eye muscle
3. orbital fracture
from either CN 3, or CN 4
affecting medial rectus or superior oblique
Cn 6 - lacteral rectus
bilateral loss of corneal reflex , with numbness or pain: neuropathy of the facial dvision of trigeminal nerve
dry eyes, dry mouth: deficits of CN 7, facila nerve -
para to lacrimal gland, submaxillary, sublingual glands
vertigo and nystygmus: sensory fibers of CN 8 vestibular
severe pain in the ear for 2 days, vesicular rash in and external to ear: herpes zoster oticus
greatest cause of grey hair, hair loss, palsy of buttocks: this class - just checking to see if you were paying attention :-)
Bell's palsey: facial paralysis of cN 7
(w/assoc.drooling) cx by viral infx, entrapment of nerve (acoustic neuroma) lyme disease (bilateral palsy)
Meniere's Disease: recurrent episodes of low-freq hearing loss, tinnitus, vertigo, and nystygmus
cx by overproduction of endolymph in cochlear duct (CN 8)
sensoneural hearing loss: sensory from Cn 8, cochlear
causes of conductive hearing loss: -excessive cerumen
-otitis media
-congenital ankylosis of ossicles
-otosclerosis (older adults)
causes of sensorineural hearing loss: dying hair cells -neuronal fx abnormal from
1.presbycusis
2. ototoxic drugs
3. acoustic neuroma
4.Meniere's disease
what is Presbycusis: gradual dying off of cochlear hair cells with age
what are the ototoxic drugs?: aminoglycosides and diuretic
What is an acoustic neuroma?: slow-growing tumor (benign) of the Schwan cells (sometims schwannoma) surrounding CN 8, vestiublar portion
-unilateral deafness and nystagmus
what is nystagmus?: involuntary cyclic movement of the eyes
what is Meniere's Disease and how is it caused?: recurrent episodes of low-frequency hearing loss, tinnitus, vertigo and nystagmus
Cx: overproduction of endolymp in the cochlear duct
differentiate between true vertigo and vasovagal syncope: True vertigo- pt feels she is moving or her surroundings are moving, ALWAYS cx some nystagmus, vertical, rotary, or horizontal. Vasov. does not present with that
peripheral vertigo- cause: cx by abnl fx within the cochlea, semcircular canals or vestibular fibers of CN 8
peripheral vertigo - signs/symptoms: severe= pt falls; N/V, tinnitus or hearing loss and horizontal or rotary nystagmus
what are the 3 forms of peripheral vertigo?: Meniere's disease
Acoustic Neuroma
Vestibular neuronitis
Vestibular neuronitis: (labrynthitis): viral,bacterial or idiopathic
(labrynthitis)acute onset vertigo, postural imbalance, N/V and persistent horizontal or rotary nystagmus.
NO hearing loss
CENTRAL VERTIGO: abnl fx in the vestibular nuclei- brainstem, cerebellum or cerebral hemispheres
Benign Positional Vertigo: common in the elderly
acute change of head position getting into or out of bed
-due to carbonate crystals breaking off the otoliths in the utricle and clogging the posterior semicircular canal.
symptoms of central vertigo: mild, no N/V tinnitus or hearing loss;
-nystagmus can be rotary or vertical
in what diseases is central vertigo present?: *multiple sclerosis
*TIA, brainstem stroke
*cerebellar hemorrhage
what signs/symptoms often accompany central vertigo?: dysfunction of CN other than CN 8:
diplopia, dysarthria, dysphagia, facial wkness or numbness
VESTIBULAR NEURITIS: â€“ SIGNIF N/V, AND LATERAL NYSTAGMUS,
pt's vertigo is due to stx such as cerebellum, brainstem, etc , this is .....: central vertigo
vestibular apparatus is composed of: 3 semicircular canals
utricle
saccule
if pt's vertigo is due to a problem anywhere from the root of CN 8 - toward and into the labyrinth, it is..: peripheral vertigo
deficient or absent gag reflex stems from what neuropathy?: dificit in CN 9, or 10
severe pain in throat, tongue, and ear on swallowing: glossopharyngeal neuralgia
constant pain in one ear, severe pain in throat and tongue: possibly cancer of the pharynx
paralysis of soft palate, nasal speech, deviation of uvula toward normal side onn phonation - due to what nerve issue?: CN 10 vagus
hoarseness of voice results from: paralysis of larynx
weakness turning head to the right: paralysis of LEFT sternocleidomastoid muscle of CN 11 accessory nerve
weakness shrugging left shoulder: paralysis of left trapezius- CN 11, accessory
deviation of protruded tongue toward weak side, & may possibly see fasciculations and atrophy of paralyzed side of tongue: CN 12 neuropathy
mononeuropathies: unilateral, asymmetric
-distal axons are sick -starts in feet -symmetric,larger, fast-conducting, sensory fibers affected -affects DC-ML and DTR: peripheral neuropathies
prototype for all peripheral neuropathies: diabetic neuropathy
2 phenomena of diabetic neuropathy: 1. microangiopathy
2. direct axonal damage
microangiopathy: happens to caps supplying distal nerves
-non-enzymatic glycosylation (caramellization)
-advanced glycosylation endproducts
-LDL trapped - atherosclerosis os small vessels
what tissues do not require insulin in order to take up glucose from the blood?: nerves, kdiney, vessel walls, lens of the eye
what happens when plasma glucose is too high for too long?: and increased amount of glucose diffuses into nerve, kidney, vessal walls and lens of eye (tissues)
negative effects of increased intracellular sorbitol and fructose?: sorbitol cx degeneration of schwann cells - demyelination
both: inc'd intracellular osmolarity>osmosis>CELL LYSIS & SCWANN CELL INJURY
signs and symptoms of diabetic neuropathy: large fiber neuropathy :
DC-ML fibers
sensory>bottoms of feet>lower leg>hand>
stocking and glove presentation: sensory:painles paresthesias, numbness, dec'd proprioception & vibration sense
motor:dec'd or absent ankle flexion, wk plantar/dorsiflexion
amyloidosis: presents with small fiber neuropathy
A-delta, C-fibers==
dysesthesias
distal pain and temp loss
proprioception/vibration preserved
Nl reflexes
dysesthesias: unpleasant abnoarmal sensation produced by normal stimuli to skin
focal neuropathies: acute onset of neuropathies -
acute vascular occlusion-ischemia-pain and neuro deficient
diabetic opthalmoplegia: CN 3 cranial neuropathy
unilateral eye pain
-diplopia
-ptosis
neurological issue associated with diabetes: stocking and glove
amyloidosis
focal neuropathies
cranial neuropathies
risk of carpal tunnel
autonomic neuropathies
hyperglycemic unawareness
neuroglycopenia
hyperglycemic unawareness: unaware of early signs of low glucose:
-wkness,tremor, nervousness,tachycardia
sudden neuroglycopenia:
-visual disturbances
-confusion, bizarre behavior
-amnesia,seizures,coma
T/F beta blockers are a recommended treatment?: FALSE!!!
Guillian-Barre' Syndrome: peripheral neuropathy
acute inflammatory demyelinating polyneuropathy
aka postinfectious polyneuropathy
affects of Guillian-Barre' syndrome: rapidly progressive, ascending motor wkness of:
legs,arms,resp myo, = paralysis
early & general HYPO- or areflexia
early paresthesias
NO true sensory loss
causes may include: viral URI or GI infx, campylobacter jejuni infx, and may occur w/ SLE, or Lymphoma: Guillian-Barre' Syndrome
signs and symptoms of GB syndrome: symmetri or asymmetric ascending weakness
-legs,arms,resp. myo
-paralysis in 50%~1wk,90%1mo.
-early & genera. hypreflexia or areflexia, and paresthesias
1/3 on vent. support, most 2-4 wk, paralysis all 4 limbs
5% die
no true sensory loss,
definitive signs and labs of GBS: inc'd CSF protein w/out pleocytosis
-ALWAYS present w/early and gen. hyporeflexia or areflexia- if wk patient has NL reflexes its NOT GBS
prognosis and treatment for GBS: tx: leave it to the neuro team
prog: most recover completely w/in 3-6mo, some have residual weakness
Lou Gerhrigs disease - Amyotrophic lateral sclerosis(ALS): cx relentless progressive deterioration upper and lower motor neuron
CN with parasympathetic motor fibers: Cn 3, occulomotor
CN 7, facial n
CN 9 glossophyrngeal
CN 10 Vagus -
primary headache: benign, no organic pathology
tention, cluster,migraine, rebound,caffeine withdrawl
secondary headache: underlying disease or pathology
-glaucoma, temporal artiritis, meningitis, intracranial hemorrage
sinusitis, influenza,severe HTN, lyme disease are what?: other secondary headaches
what are the majority of h/a classified as?: benign, primary
features of the h/p exam that indicate the h/a is secondary to serious pathology: marked change in h/a pattern
rapidly incrs. freq of h/a
sudden onset of severe
new onset after age 50
persistent h/a after trauma
what combination of other symptoms makes you suspect secondary?: h/a + fever, nuchal rigity, pailledema, clumsy, seazures, impaired mental status, focal neuro signs
T/F pain from the h/a stems from neuronal structures of brain parenchyma: FALSE, there are no pain receptors there
instead it comes from cranial structures
what cranial structures are implicated in the cause of pain of a headache?: 1. head and neck muscles
2. scalp and vessels
3. meningeal & cerebral arteries
4. venous sinuses
5. pain AFFERENTS of CN 5,9,10
6 parts of dura matter at base of brain
cause of tension h/a: stress, anxiety, depression,poor posture, alcohol, various meds, osteorathritis
signs and symptoms of tension headache: from none to moderate throbbing
dull, pressing or band-like pain
bilateral
treatment for tension h/a: self treat with aspirin, acetaminophen or ibuprophan

Rx: anaprox (naproxen)
antipyretic, analgesic, anti-inflammatory: NSAIDS, aspirin, but NOT acetamenophin - not anti-inflammatory
butalbital: barbiturate that has potential for addiction
what happens if you use butalbital more than 2x week?: risk of progression to chronic daily h/a
= rebound h/a
define chronic daily h/a: presence of tension h/a at least 6 days/week
what Rx treatments for tension h/a?: NSAIDS, muscle relaxant
pericranial muscles: cause of tension h/a
they include the :
1. frontalis
2.temporalis
3/ sternocleidomastoid
r. trapezius
T/F tension and cronic daily h/a's are avascular?: true
migraine - where: vascular h/a
cerebral cortex is hyperexcited, unilartery
cephalgia: head pain aka cephalalgia
mood change, blurred vision, flashing lights, photophobia, tingling, numbness: migrain headache
vasocontrictive phase: causes pts auras
vasodilatory phase: causes the headache itself
signs and symptoms of migrained: throbbing, unilateral,photophobia +/or phonophobia
wavy lines-teichopsia
weakness, clumsy

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