Part 2 - Intro to Neuromuscular Disease/Pathologies of the Motor Unit Flashcards
Lower Motor Neuro Disease Signs and Symptoms
Weakness (patchy, distal worse first), clumsiness in fine motor tasks, myoclonic jerks (“sleep starts”), painful muscle cramps (esp. at night or when stretching), muscle fasciculations (see PPT for definition)
Guillain-Barre Syndrome - signs and symptoms
First symptoms = fatigue and tingling sensations in legs
Symmetrical muscle weakness (LE first then rapidly progresses upwards)
Patients describe “rubbery feeling” in their legs; legs tend to buckle
Initial weakness may also have numbness and tingling associated.
LOWER MOTOR NEURON
S&S of Poliomyelitis Paralytic Form
Flaccid muscle paresis or paralysis (ventral horn motor neuron cell bodies are attacked)
Acute fever (~2 weeks) of fever with headache, sore throat, severe muscle pain to touch and stretch & flaccid paralysis of involved muscle
Highly variable degree of destruction
Acute Poliomyelitis
Same as LMN disease:
Weakness (patchy, distal worse first), clumsiness in fine motor tasks, myoclonic jerks (“sleep starts”), painful muscle cramps (esp. at night or when stretching), muscle fasciculations (see PPT for definition)
S and S of Post Polio Syndrome
Gradual or sudden onset
New muscle weakness or abnormal muscle fatigability (decreased endurance)
With or without:
- generalized fatigue
- muscle atrophy
- muscle and joint pain
- new difficulties with swallowing or breathing
Symptoms persist at least a year
Exclusion of other neurologic, orthopedic, or other medical causes of the signs and symptoms
LMN
Secondary Impairments of Paralytic or Spinal Polio
Symmetries in limb growth and scoliosis are very common in children affected from an early age.
People with mild disease could have significant loss of strength and still muscle test in the normal range
Secondary impairments of PPS?
Muscular weakness, low pain tolerance, easy fatiguability.
DO NOT OVER-EXERCISE
Signs and Symptoms of UMN Disease
Spasticity (in ALS only for this test - per Dr Charles
-Hyper-reflexivia
-Heaviness or stiffness of limbs
-Bulbar signs (impairments of lower CN/brain stem, and or medulla)
Dysphasia (swallowing problems)
Speech problems
Drooling
Increased cough reflex
Difficulty breathing
Signs and symptoms of Multiple Sclerosis
Fatigue Impaired ambulation Bowel/bladder dysfunction Visual disturbances Cognitive impairment Tremor Spasticity Weakness Pain Imbalance Incoordination Sensory changes Dysarthria Dysphasgia Sexual dysfunction Emotional changes (depression, bi-polar disorder)
Secondary Impairments in MS
DEPRESSION!!!! (and euphoria, affective release (pseudobulbar affect), antisocial behavior) Heat intolerance Bowel dysfunction Bladder dysfunction Vision problems -Optic Neuritis -Eye movement abnormalities Speech problems Dysphasia Sexual dysfunction
Slowed speed of processing Memory -Working memory -Secondary memory Attention and concentration, including -Sustained attention -Complex or divided attention Abstract reasoning and problem solving Verbal fluency Executive functions Visuo-spatial skills
Numbness Tingling Pain Dysarthrias -Electric shock-type sensation (L'Hermitte's Sign) -Banding-type sensation May be persistent or intermittent
Pain
Anxiety Sleep disturbances Impaired hand function Impaired balance Disuse Reduced safety
DEPRESSION!!!
ALS - signs and symptoms
Muscle gradually weaken! - atrophy and develop fasciculations
Brain loses ability to initiate and control voluntary movement
(Cognition and senses are kept intact)
Earliest: twitching, cramping, or stiffness of muscles; muscle weakness affecting an arm or leg and/or slurred and nasal speech
Most obvious symptom = weakness
75% limb onset; 25% bulb onset
Muscle weakness spreads as disease progresses
Difficulty moving, swallowing (dysphasia), and speaking or forming words (dysarthria)
Symptoms of UMN include tight and stiff muscles (spasticity) and exaggerated reflexes
Exaggerated reflexes = hyperreflexivia - including an overactive gag reflex
A Babinski’s sign indicates UMN damage
Symptoms of LMN degeneration include muscles hat can be seen under the skin (fascicultions)
round 15%-45% of patients experience pseudobulbar affect (also known as “emotional lability”, which consists of uncontrollable laughter or crying)
Symptoms are similar to other more treatable diseases and disorders
ALS - UMN or LMN?
To be diagnosed with ALS, patients must have signs and symptoms of BOTH UMN and LMN damage that cannot be attributed to other causes.
Secondary Impairments of ALS
As the diaphragm and intercostal muscles weaken, forced vital capacity and inspiratory pressure diminish
In bulbar onset ALS, this may occur before significant limb weakness is appearant.
Bilevel positive pressure ventilation (referred to by the trade name BiPAP) is frequently used to support breathing, first at night, and later during the daytime as well.
Most people with ALS die of respiratory failure or pneumonia, NOT FROM THE DISEASE ITSELF
S&S of PD?
Gradual onset (usually a barely noticable tremor in one hand)
Resting tremor
In early (and later stages), there will be little or no facial expression (aka facial masking)
Bradykinesia (slow movements)
Rigidity (Cogwheel)
Postural instability
Little to no arm swing while ambulating
Loss of automatic movements or inability to perform unconscious movements (solid unit - loss of rotation) such as blinking or smiling
Festinating (shuffling) gait
Clinical Soft Signs of PD?
Difficulty in buttoning, zipping
Cutting food
Changes in hand writing
Feelings of stiffness AND overall slowness
Difficulty in rising from low soft chairs/sofa
Getting in and out of cars
Losing balance while walking in a crowd
Family members will report changes in facial expression (masking)
Starting and non-blinking
