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