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NEUROLOGY • head and neck muscles

Disorder of the Muscles


Jose C. Navarro, MD, FPNA Diagram: Myoneural Junction
2/13/06 • no anatomical continuity
• space between terminal boutton and pre
Diagram: and post- synaptic area
Problem with these areas:
• Ant. Horn cell lower motor neuron – alpha motor neuron,
• From peripheral nerve ex. Brachial plexus peripheral nerve, myoneural junction, muscle
• Myoneural Junction
• Muscles CLINICAL MANIFESTATION OF WEAKNESS
Common Manifestation: WEAKNESS 1. Inability to move
2. Gower’s Maneuver
If patient comes with weakness of whole 3. Respiratory Embarrassment – failure
extremity, it’s a muscle disease. (generalized • in severe disease
distribution) • will necessitate intubation/respiratory
support
If it’s just one arm or one leg, it’s not a muscle
disease. Gower’s Maneuver
• In patient w/ generalized weakness; seen in
CHARACTERISTICS OF MUSCLE DISORDERS both UMN and LMN lesion
1. Generalized • Not exclusively in muscle disease, seen
2. Distribution more proximal than distal also in peripheral nerve disease
• Ask for activities • Ask the child to sit on the floor, then get up
• Difficulty of raising hands over head; thus • If generalized weakness, develop diff
difficulty in combing hair and hanging techniques to get up
clothes • Turn; face floor; hands on the floor
• In the lower extremities: difficulty of
• Extend knees
climbing up stairs and especially when
• Hands on the knees
going down stairs
• In climbing, the distal muscles can help • Stand
pull up. In going down, there is a problem • As disase progresses: difficulty of
with quads and extensor muscles, so its getting up from a chair, then from a
much harder toilet bowl; later on difficulty of getting
• Difficulty in getting up from sitting a up from a stool
position • Protruding abdomen
** to sit, start with NECK MUSCLES • Lordosis exaggerated
(kala nyo sa hips or sa legs no… nope! It’s the neck • If with proximal weakness, thumb rotates
accdg. To doc) inferiorly; (N) Thumb always pointing
anteriorly
If Distal Muscles Affected:
• weakness makes are arm rotate internally
1. weakness at the fingers - difficulty in
• Weak shoulder, girdle muscles – arm
buttoning and strapping and unstrapping bras
rotates internally (like MONKEYS or
2. Foot drop – foot snaps while walking APES!!)
• To stand up, knees are buckled to maintain
3. difficulty in walking on toes and heels an erect position
• all done to achieve the lowest point of
*Peripheral Nerve Disease – generalized gravity of the body
(distal > proximal) • Walking
• (N) arm swing opposite
*Central – generalized (proximal > distal) • W/ muscle disease, throw their bodies
forward (Arms and legs go forward at
ANTERIOR HORN CELL DISEASE the same time) (remember Doc’s
• weakness of both proximal & distal sample walk)
muscles - “malikot” coz their trying to fix their point of
• segmental - manifestation correspond to gravity
area of spinal cord segment affected
• sensory function is spared Extraocular movements – “Dilat na dilat”!
ex. Poliomyelitis –affects usually one extremity
only • Progressive
• no longer able to walk
PERIPHERAL NERVE DISEASE • wheelchair-bound
• weakness w/ numbness, paresthesia • can no longer move due to generalized
• most of the time mixed nerve = motor, weakness
sensory, autonomic
DIAGNOSTIC TESTS
MYONEURAL JUNCTION • Creatine Kinase
• distribution of weakness are cranially • Electromyography
innervated muscles
• Pedigree/ Chromosomal Studies
• Muscle Biopsy – should be complementary Treatment: steroid & immunosuppressants
to clinical diagnosis only
• Biochemical Exam Course and prognosis
20% that develop P or D in early life will
Muscle Biopsy develop malignancy sometime in their life
• muscle located at periphery (N)
*doc also said: 15% malignancy
• If migrate to center  pathological
• Size of muscle fiber: 50 microns – same Picture of Polymyositis
size as a hair strand • inflammatory infiltrates
• some beginning to degenerate
*muscle biopsy is not done for diagnosis, it is
only for confirmation
*muscle cell nucleus is located in the sub-
sarcolemma, in the periphery of the muscle
fiber

INFLAMMATORY MYOPATHY
• progressive prox muscle weakness
• Dysphagia
• Myalgia
• Elevated CK
• Myopathic EMG
• Muscle Biopsy – inflammatory

*not caused by microorganisms

*Dysphagia – because part of the esophagus is


made up of skeletal muscle; thus cardiac and
smooth muscles are not involved

*since inflammation  pain

*There is a certain amount of creatinine kinase


in the blood, because CK can traverse the
sarcolemma; normal value = 40IU/microliter

*Elevated CK - muscle disease destroys


sarcolemma, allowing CK to go out, thus
increase CK

Biopsy
important characteristic of inflammation 
infiltration of different cells – neutrophils,
lymphocytes

PATHOPHYSIOLOGY OF INFLAMMATORY DISEASE


• autoimmune disease
• patient starts to degenerate
• antigen-antibody complex acts on muscle
membrane

DERMATO/POLYMYOSITIS
• Dx: progressive proximal muscle weakness
• Autoimmune disorder
• 3F:1M
• Skin lesions
• 40 y/o and above
• progressive proximal muscle weakness
• Raynauds phenomenon
• Dysphagia

*Dermatomyositis – younger age group


Polymyosistis –adults

*Similar, only difference is dermatomyositis


has rashes
PROGRESSIVE MUSCULAR DYSTROPHY
CLINICAL USUAL
PATTERN INHERTITANCE
*Duchenne X-linked
dystrophy
*Becker’s X-linked
dystrophy
*Limb-girdle Autosomal
recessive
Scapulohumeral Autosomal
recessive
Fasciohumeral Autosomal
dominant
Oculopharyngea Autosomal
l dominant

Read:
• Periodic paralysis
• Myasthenia Gravis
• Muscle dystrophy inheritance
• Duchenne
• Beckers
• Limb-girdle

- by Bee, Pauline n Liz


HAPPY BIRTHDAY LUWI and PAULINE!