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1 CJCASTILLO
SEQUENCE OF EVENTS LEADING TO MUSCLE WEAKNESS
CONTRACTION & PATHOPHYSIOLOGIC - NERVE symmetrical but distal, gloves & stockings
CORRELATIONS - MUSCLE symmetrical and proximal, arm & thighs
from exctitation to ATROPHY
contraction - NERVE early; dennervated
acetycholine release FASCICULATIONS nerve problem; motor neuron
myasthenia gravis SENSORY LOSS nerve
before you release the Ach AUTONOMIC nerve
vesicles, there is Ca channels CK ENZYMES dennervated at muscle; normal at nerve
involved
Ca should enter for the action DISEASES OF THE MUSCLE
potential of the nerve Inflammatory myopathies polymyositis & dermatomyositis
LEMS problem in the Ca
channel IDIOPATHIC POLYMYOSITIS & DERMATOMYOSITIS
LEMS presynaptic - Common diseases affecting striatal muscles & skin, &
MYASTHENIA GRAVIS post synaptic; Ach has been release sometimes connective tissues
but the problem is the binding of Ach to the receptors - May be associated with CTD (RA, RF, LE, scleroderma,
STEROID MYOPATHY weakness Sjogren, or mixed CTD)
2 CJCASTILLO
PATHOLOGIC CHANGES IN PM & DM SEX LINKED MUSCULAR DYSTROPHIES:
- PM widespread destruction of segments of muscles DUCHENNE & BECKER
(necrosis) with scattered inflammatory reaction - Duchenne muscular dystrophy (DMD) & Becker
(mononuclears, lymphocytes & plasma cells) muscular dystrophy (BMD)
- DM perifascicular muscle atrophy; infiltrates - Mutations in gene for dystrophin at Xp21
predominate in the perimysial connective tissue; - X-linked recessive inheritance (manifest in boys)
microvascular changes - Carriers (girls & women) may have mild symptoms of
high CK
ETIOLOGY & PATHOGENESIS - DUCHENNE more common
- Idiopathic o INCIDENCE: 1 in 3,300 male births
- Autoimmune mechanism (association with other o PREVALENCE: 1 in 18,000 boys
autoimmune disease) - BECKER: 1 in 20,000
- IMMUNOLOGIC DESTRUCTION - DYSTROPHIN a cytoskeletal protein located at
o DM humoral; IgG, IgM, complement (C3) plasma membrane, associated with glycoproteins linking
& membrane-attack complexes (MAC) are cytoskeleton to plasma membrane
deposited in the walls of venules & arterioles) - Mutations in dystrophin gene leads to absence of
o PM cellular; large numbers of activated T dystrophin in DMD; smaller size, reduced levels in BMD
cells mainly CD8; T cells & macrophages o The sarcolemma is unstable in contraction &
enclose nonnecrotic muscle fibers relaxation, liable to breakage, excessive calcium
influx, causing muscle necrosis
DIAGNOSIS & TREATMENT Treatment: genetic counseling
- DIAGNOSTICS CK, EMG, muscle biopsy
- TREATMENT DUCHENNE MUSCULAR DYSTROPHY
o CORTICOSTEROIDS first line; - The most frequent & best known among the early onset
prednisone 1 mg/kg; response is tested by MDs
muscle strength & CK levels - Usually recognized by 3rd year of life, almost always before
o HIGH DOSE METHYLPREDNISOLONE 6 yrs
for acute & severe cases; 1 gm IV infusion - Enlagement of muscles show rubbery feel
for 2 hrs OD x 3-5 days - Pseudohypertrophy
Out patient maintenance for methylprednisone - Proximal weakness with delayed walking; difficulty
o Intravenous immunoglobulin (IV Ig) running, toe-walking, waddling gait
o Plasma exchange (PE) - Progressive to overt symptoms before age 5; difficulty
o Immunosuppressants azathioprine, walking, climbing stairs, rising from chairs
methotrexate - Exaggerated lumbar lordosis, falls, use of arms to push up
on rising from the ground (GOWER SIGN)
PROGNOSIS - ECG is abnormal in most patients
- Generally favorable prognosis (except for those with - Cardiomyopathy with congestive heart failure
malignancy) e.g. polyomyositis with other CTD - Mental retardation in 33%
- CAUSES OF DEATH pulmonary complications, - OTHER POSSIBLE MANIFESTATIONS:
myocarditis Intestinal hypomotility Osteoporosis
Acute gastric dilatation Intellectual impairment
3 CJCASTILLO
THYROID MYOPATHIES
SEX LINKED MUSCULAR DYSTROPHIES: - Several myopathic diseases are related to alterations in
DUCHENNE & BECKER thyroid function:
- INVESTIGATIONS o Chronic thyrotoxic myopathy
o Serum CK elevated (usually at least 20 times o Exophthalmic ophthalmoplegia (infltrative
the normal) orbital ophthalmopathy)
o DNA test for Xp21 mutation o Myasthenia gravis associated with
o Dystrophin assay by immunoprecipitation of thyrotoxicosis
muscle proteins from biopsy, or immunocytochemistry Myasthenia not actually a muscle problem but
on frozen muscle section the weakness
- MUSCLE BIOPSY o Periodic paralysis associated with
o GENERAL HISTOLOGIC FEATURES OF thyrotoxicosis work up: thyroid (1st), (2nd)
MYOPATHY random variation in fiber potassium, (3rd) adrenals
diameter, with both large & small fibers o Muscle hypertrophy & slow muscle
Fat deposition, Pseudohypertrophy contraction & relaxation associated with
oSome fibers have internal migration of nuclei, myxedema & cretinism
some are splitting or degenerating, & there is
an in connective tissue (Trichome stain; x PATHOGENESIS OF THYROID MYOPATHIES
200) - THYROXINE influences the contractile process but
- DIAGNOSIS does not interfere with the transmission of impulses in the
o Usually evident from clinical features peripheral nerve across the myoneural junction or sarcolemma
o CK values 20 times higher than normal - There is enhancement of the speed of the contractile
o Blood DNA testing usually confirmatory process & reduces its duration fatigability, weakness, loss of
If not, muscle biopsy is performed endurance
- DIFFERENTIAL DIAGNOSIS - The speed of the contractile process is related to the
o Spinal muscular atrophy quantity of the myosin ATPase which is in hyperthyroid
o Conditions causing CK elevation; Xp21 carrier muscle & in hypothyroid
state, idiopathic or symptomatic hyperCKemia,
dysferlinopathies, caveolin-2 mutations CORTICOSTEROID MYOPATHIES
- TREATMENT - CUSHING DISEASE MYOPATHY
o Prednisone therapy beneficial in controlled o Poor correlation between the dose of steroids
trials; limited by side effects of chronic & the severity of weakness
administration (osteoporosis, etc) o Discontinuation or reduction in steroids leads
o Rehabilitation to: to gradual improvement & recovery
Maximize function based on o POSSIBLE PATHOGENESIS there is in
preserved muscle strength the uptake of amino acids & protein synthesis
Prevent contractures by the muscle
o Braces, surgery for spine or limb deformities o EMG is normal or just with mild myopathic
o Social, emotional support, genetic counsel changes; no fibrillations, CK normal
o BIOPSY little or no necrosis, no
inflammatory cells
4 CJCASTILLO
- ACUTE STEROID MYOPATHY (Critical Illness oAcute onset of severe muscle cramps &
Myopathy) generalized weakness
o Acute & more severe o CHRONIC ALCOHOLIC MYOPATHY
o Brief periods of high dose steroids e.g. chronic painless weakness & atrophy of
asthma proximal limbs
o There may be critical illness polyneuropathy, - ALCOHOLISM one of the most common causes or
myoglobinuria, renal failure rhabdomyolysis
o Most of the patients recover in 6-12 wks
Stop 1st the steroids before going to extensive CASE 1
procedure - A 58 yr old diabetic female complained of tingling
o EMG fibrillations present sensation (paresthesia) over both feet for the past 6-8 months
o CK elevated - About 3 months ago, she started having numbness &
o BIOPSY varying degrees of necrosis & tingling sensation over the fingers of both hands (gloves &
vacuolation stockings)
- PERTINENT NEUROLOGIC EXAM:
MYOPATHIES CAUSED BY DRUGS & TOXINS o MMT = 5/5 extremities, proximal & distal
FEATURES OF TOXIN-INDUCED MYOPATHIES o DTRs +1 on both lower extremities; +1 to
Statin drugs, colchicines +2 both upper extremities
- SENSORY
NECROTIZING MYOPATHY o 50% deficit as to light touch (using Camel
- RHABDOMYOLYSIS myoglobin & other muscle hair) & vibratory sense over both feet & fingers
proteins enter the bloodstream & appear in the urine (burgundy of both hands
red or brown color) - WHERE WILL YOU LOCALIZE THE LESION?
- MEDICAL CONDITIONS THAT MAY LEAD TO o Muscle
MYOGLOBINURIA crush injury; vascular diseases; repeated o Neuromuscular junction
injury to muscles such as in status epilepticus, generalized tetanus o Nerve
& malignant hyperthermia - GIVE YOUR BASIS
- TREATMENT alkalinization of the urine by ingestion o Sensory signs & symptoms, DTR,
of Na bicarbonate; dieresis; hydration distribution (gloves & stockings)
5 CJCASTILLO
THE SPINAL LEVEL - Flexion of the spinal cord or traction on a peripheral
Start of the spinal cord FORAMEN MAGNUM nerve (e.g. disc herniation or tumor) can stretch & irritate a
No gross differentiation of the medulla & the spinal cord spinal nerve & cause pain RADICULAR PAIN
Similarity of the lower medulla & upper spinal cord - GENERAL GUIDELINES FOR LOCATING THE
LEVEL OF A SPINAL CORD INJURY WITH RESPECT
SPECIAL PHYSIOLOGIC & ANATOMIC FEATURES OF TO THE VERTEBRAE:
THE SPINAL CORD o Between T1 & T10, add 2 to the number of
- Prominent function in sensorimotor conduction & the vertebral spine to determine the spinal cord
relatively primitive reflex activity segment at same location
- Its long, cylindrical shape with 2 enlargements EXAMPLE: gunshot wound at T2 T4 (add 2)
cylindrical & lumbar Spinothalamic ascend 2 levels before crossing
- Its small cross-sectional size o The lumbar segments of the cord are
- Its tight envelopment by the meninges approximately at the level of the spinous
- The peripheral location of myelinated fibers next to the processes of T11 & T12
pia o Sacral & coccygeal segments are at the level of
- The special arrangement of its blood vessels the L1 spinous process
- The relationship to the vertebral column o There are 8 cervical segments & only 7 cervical
vertebrae, so that the cervical enlargement at
REVIEW OF ANATOMY C7 cord segment is centered at the C7
- INTERVERTEBRAL FORAMEN the bony canal vertebral level
formed by 2 adjacent vertebrae
- From the foramen magnum up to the lower border of the DERMATOMES
1st lumbar vertebra - Each spinal nerve is distributed to a well-defined area of
- Cylindrical/conical in shape with 2 enlargements, the skin called DERMATOME
cervical (brachial plexus) & the lumbar (lumbosacral plexus) T4 - nipple
- Caudal end is called CONUS MEDULLARIS T10 umbilicus
From the conus medullaris, there is a long ligament plus the T7 xiphoid process
spinal nerve roots coming from the lumbar & sacral level L1 inguinal
CAUDA EQUINA L2 or L1 spinal nerve root
- 31 Spinal Nerves Umbilicus or going up spinal cord
(8 cervical, 12 thoracic, 5 lumbar, 5 sacral, 1 coccygeal) - Dermatomes of adjacent spinal nerves overlap. This
INTERVERTEBRAL FORAMEN formed from 2 adjacent overlap accounts for the variation in sensory loss seen in
vertebra; patients with spinal nerve lesions
TRANSVERSE FORAMEN where vertebral arteries will
pass thru BLOOD SUPPLY
- Because the nerve is surrounded by bony structure, it is - 1 anterior (2/3) & 2 posterolateral arteries (1/3)
particularly vulnerable to local compression by a tumor, Anterior more common to stroke
herniated disc or arthritic changes in the bones - The spinal arteries receive their supply from the vertebral
- Because there are only 7 cervical vertebrae, C1 to C7 arteries & from the intercostal, lumbar, & sacral arteries through
nerves exit above the vertebra while the C8 exit between C7 & 6-8 radicular arteries, the largest usually entering at the lower
T1 for localization thoracic or upper lumbar region of the cord
C2 exits at between C1 & C2
C7 exits between C6 & C7 INTERNAL MORPHOLOGY
- Below T1 spinal nerves exit below the vertebrae of same - WHITE MATTER Outer
number o Longitudinally arranged myelinated &
- Nerve roots are anchored in the intervertebral foramina by unmyelinated nerve fibers, with an abundance of
the DENTICULATE LIGAMENT myelin that gives it a glistening white appearance
6 CJCASTILLO
- GRAY MATTER inner if a patient has weakness or numbness (legs first) ascending
o A longitudinally continuous matrix of neuronal compression is coming from the outside
cell bodies, dendrites, myelinated & unmyelinated if a patients presentation is at the arm first, then gradually
nerve fibers, with an abundance of myelin that gives involving the legs medially
it a glistening white appearance Intramedullary spared initially the sacral (sacral sparing)
o Divided into dorsal horns, intermediate gray & Extramedullary roots may be compressed (e.g. herniated disc),
ventral horns prominent pain (radicular pain)
o 2 MAIN TYPES OF NEURONS are
Projectional neurons & Interneurons OVERVIEW
- 2 DISTINCT PATTERNS OF ABNORMALITY
INTERNEURONS OCCUR:
- Interneurons have axonal projections that remain in the o SEGMENTAL: produces signs localized in a
spinal cord & constitute the bulk of spinal neurons single segment of the body horizontal
- Interneurons integrate segmentally & intersegmentally, Flaccid weakness, atrophy, loss of
both ipsilaterally & contralaterally reflexes, loss of sensation
o LONGITUDINAL: produces deficit for all
REXED LAMINAE functions below the level of the lesion vertical
- LAMINA II SUBSTANTIA GELATINOSA Weakness (spastic type), no atrophy
o Receives input from unmyelinated nociceptive initially, reflexes, presence of Babinski signs, loss
C fibers (carrying pain & temperature) of sensation, autonomic disturbances (bowel,
- LAMINA IV Contains the Clarkes column bladder, sexual dysfunctions)
proprioception C6 carry the biceps, brachioradialis
The cells are tract cells that sends axons to the
o
cerebellum via the dorsal spinocerebellar tracts THE SYNDROME OF ACUTE PARAPLEGIA OR
- LAMINA IX Contains the motor neurons (alpha) QUADRIPLEGIA CAUSED BY TRAUMATIC & OTHER
anterior motor neurons PHYSICAL FACTORS (TRANSVERSE MYELOPATHY)
- LAMINA X Surrounds the central canal
I. TRAUMA TO THE SPINE & SPINAL CORD
SPINAL CORD LAMINATION OF TRACTS A. MECHANISMS OF SPINE & SPINAL CORD
major ascending (blue) - USEFUL CLASSIFICATION fractures-dislocations,
& descending (red) pure fractures, pure dislocations (3:1:1) more common
tracts of the spinal cord - Most injuries are the result of force applied at a distance
white matter. C, - FLEXION INJURY the head is bent forward when the
cervical; L, lumbar; Th, force is applied; the adjacent cervical vertebrae are forced
thoracic; S, sacral together at the level of maximum stress; the posterior part of the
fractured vertebral body is displaced backward & compresses the
spinothalamic pain going up, carrying pain & temperature spinal cord
- fibers from the sacral, lumbar (from the legs) are laterally - HYPEREXTENSION INJURIES the head is in an
located extended position when the force is applied; stress is mainly on
- fibers from the upper extremities, arm, cervical fibers are the posterior elements of the midcervical vertebrae (C4-C6)
medially located which may be fractured; spinal cord damage is caused by sudden
same on motor going down inward bulge of the ligamentum flavum (vertebral column;
- fibers going to the upper extremities medially located posterior ligament; intersegmental)
- fibers from the legs (lumbosacral) for the strength, motor
laterally located
only different is the dorsal column or the posterior column
7 CJCASTILLO
WHIPLASH INJURY (TRAUMATIC CERVICAL II. STAGE OF HEIGHTENED REFLEX ACTIVITY
DISTORTION) o Babinski sign
- WHIPLASH OR RECOIL INJURY most often due o Return of DTR hyperreflexia
to vehicular accidents, sudden forward flexion of the neck o Withdrawal reflexes flexor spasms
followed by retroflexion, the occipitonuchal & the o Reflex defecation & urination (automatic
sternocleidomastoids are affected much more often than the emptying of the bladder)
spinal cord or roots o AUTONOMIC DYSREFLEXIA occurs in
response to a certain stimulus such as
B. PATHOLOGY OF TRAUMATIC SPINAL CORD distended bladder; composed of hypertension,
INJURY reflex bradycardia, cutaneous flushing,
- Squeezing or shearing of the spinal cord destruction of headaches; secondary to release of adrenaline
gray & white matter; hemorrhage (traumatic necrosis) from adrenal medulla & norepinephrine from
hematomyelia (nu surgery) disinhibited sympathetic terminals
intramedullary
- Maximal at the level of injury & at 1 or 2 segments above TRANSIENT CORD INJURY (SPINAL CORD
& below CONCUSSION)
- As the lesions heals, it leaves a gliotic focus or cavitation - Recovery within minutes or hours but may persist in
progressive cavitation (traumatic syringomyelia) mild form for days or longer
develops after few months or years - Transient symptoms such as bibrachial weakness,
- In most traumatic lesions, the central part of the cord quadriparesis, paresthesias & dysesthesias
suffers the most - Usually observed in some sports such as football, hockey
& rugby
C. CLINICAL EFFECTS OF SPINAL CORD INJURY
- THREE DISORDERS OF FUNCTION: MANAGEMENT OF SPINAL INJURY
1. Immediate loss of all voluntary movements below - Immobilization of the spine
the lesion - High dose methylprednisolone bolus of 30 mg/kg
2. Sensation below the level of the lesion is abolished followed by 5.4 mg/kg every hour, beginning within 8
3. Reflex functions in segments of the isolated spinal hr of injury & continued for 23 hrs
cord are suspended (spinal shock) deep tendon - Radiologic examinations
reflexes & autonomic reflexes - MRI to see the cord & the nerves
- If with vertebral dislocation, neck traction for
SPINAL SHOCK immobilization & maintain spine alignment use of
- VARIABLE DURATION 1-6 wks or even longer halo brace (4-6 weeks)
1. STAGE OF SPINAL SHOCK - SPINE SURGERY most delay the operations or
o Loss of motor function (quadri- or para- operating only in those with compound wounds or with
plegia), flaccidity progression of neurologic deficits
o Immediate atonic paralysis of the bladder & - The greatest risks to the patient occurs in the 1st 10 days
bowel (gastric dilatation, ileus, shock, infections)
o Gastric atony - Management of bowel & bladder dysfunction
o Loss of sensation below the level of lesion - Prevention of complications of prolonged
o Almost complete suppression of all spinal immobilization such as pulmonary embolism, bed sores
segmental reflexes - Nutrition
o Hypotension - Pain management
o Due to sudden interruption of suprasegmental - Control of spasticity baclofen, diazepam
descending fibers
8 CJCASTILLO
III. RADIATION INJURY OF THE SPINAL CORD
- TRANSIENT RADIATION MYELOPATHY
o 3-6 months after radiopathy
o Spontaneous, uncomfortable sensations in the
extremities; may be provoked by neck flexion
- DELAYED PROGRESSIVE RADIATION
MYELOPATHY
o 6 months or more after the radiotherapy
(usually 12-15 months)
o Insidious onset of sensory symptoms
(paresthesias, dysesthesias) followed by
weakness of both legs
9 CJCASTILLO