Week 12 Neuro Part 2 Flashcards
Restless leg syndrome - what is it? When does it occur?
-common sensorimotor disorder associated with unpleasant sensations (prickling, tingling, crawling) and undesired periodic leg movements
-occur at rest and worse in the evening or at night
-powerful urge to move legs for relief-can significantly affect sleep/QOL
Are periodic limb movements always a part of restless leg syndrome?
No, can just be uncomfortable sensations that make you want to move your legs… periodic limb movements during sleep is considered its own disorder
What populations experience restless leg syndrome more commonly?
more common in women, during pregnancy, older persons and individuals who are iron deficient
When are the uncomfortable sensations of restless leg syndrome worse? (2)
- During rest/inactivity
- At night
How common is it for those with restless leg syndrome to also have periodic movements of sleep (PLMS)?
Approximately 85% of patients with RLS have periodic movements of sleep, usually involving the legs (periodic leg movements of sleep [PLMS]). PLMS is characterized by involuntary, forceful dorsiflexion of the foot lasting 0.5-5 seconds and occurring every 20-40 seconds throughout sleep.
Are sleep disturbances, daytime fatigue, and involuntary jerking movements (either while asleep or awake) part of the diagnostic criteria for RLS?
No, but they are features commonly associated with restless leg syndrome
What do you need to test for in all patients with RLS?
Iron deficiency
Do we routinely do sleep studies for people with RLS?
NO, but Polysomnography may be necessary to quantify PLMS or to characterize sleep architecture, especially in children and in patients who continue to have significant sleep disturbances despite relief of RLS symptoms with treatment
**Sleep disturbances may be associated with RLS but are not part of the diagnostic crtieria
What are some non-pharmacologic treatments of RLS?
Sleep hygiene measures
Avoidance of caffeine, alcohol, and nicotine in patients with mild RLS who are sensitive to these substances
Discontinuation, when possible, of medications that cause or exacerbate RLS, such as selective serotonin reuptake inhibitors (SSRIs), serotonin-norepinepherine reuptake inhibitors (SNRIs), diphenhydramine, and dopamine antagonists
Exercise
Physical modalities before bedtime, such as a hot or cold bath, whirlpool bath, limb massage, and vibratory or electrical stimulation of the feet and toes
What is Periodic Limb Movements Disorder? (PLMD)
Is sleep disorder
Often co-exists with restless leg syndrome, but is a distinct and mutually exclusive diagnosis
Movements occur AFTER You fall asleep (RLS is more about what leg discomfort BEFORE sleep)
characterized by a clinical sleep disturbance attributed to an increased number of PLMS (periodic limb movements of sleep), in the absence of alternative causes of the sleep complaints.
Cause of PLMS?
Causes largely not understood, but seems to be associated with many of same conditions as RLS: low ferritin levels, medications,
According to Wikipedia…PLMS seems to have an origin in the spinal cord. In fact, PLMS was suggested to be associated with increased spinal reflexes. Manifestations of PLMS seem to occur mostly in disorders associated with dopaminergic dysfunctions
Do periodic limb movements of sleep occur during REM or NREM sleep?
Occurs during non-REM sleep stages (do not occur during REM due to muscle atonia)
S&S of periodic limb movement disorder?
Usually manifests as daytime somnolence
Wake up with cramps or “Charlie horse” In legs
Often not aware of these movements at all, unless someone else points them out (bed partner may complain of person kicking them during sleep)
During night, leg twitching or jerking (usually flexion) movements during sleep occurring every 15 to 40 seconds.
Do you need to do a sleep study to diagnose perioid limb movement disorder? What is included in the diagnostic criteria?
Yes! (unlike RLS)
diagnosed based on a clinical history of sleep disturbance or daytime fatigue, combined with polysomnography (PSG) showing an excessive number of periodic limb movements of sleep (PLMS), and exclusion of alternative causes of the sleep complaints.
Diagnostic criteria = more than 15 periodic limb movements per hour of sleep time in adults (more than 5 in children), causing clinically significant sleep disturbance or impairment in function or wellbeing not explained by some other entity
PLMD is a diagnosis of exclusion and cannot be diagnosed in the context of other sleep disorders that have already been diagnosed, such as RLS, OSA, and narcolepsy (because these present with very similar movements)
What areas of muscle does the ulnar nerve innvervate?
some forearm and most hand muscles
- Flexor carpi ulnaris muscle
- The ulnar half of the flexor digitorum profundus muscle in the anterior forearm
- Most of the intrinsic hand muscles: hypothenar muscles, two lumbricals, adductor pollicis muscle, all the interossei muscles (palmar and dorsal)
What is happening when you feel like you “hit your funny bone”?
The ulnar nerve is vulnerable as it passes posterior to the medial epicondyle of the humerus
This causes the “I hit my funny bone” tingling sensation
What spinal nerves does the ulnar nerve originate from?
From the brachial plexus: spinal nerves C7, C8 and T1
The ulnar nerve is a terminal branch of the medial cord of the brachial plexus. It contains mainly fibers from the anterior rami of spinal nerves C8 and T1, but may sometimes carry C7 fibers as well.
Is the ulnar nerve motor or sensory or both?
Why is it considered one of the most clinically relevant nerves of the upper limb?
From its origin, the ulnar nerve courses distally through the axilla, arm and forearm into the hand. It is a mixed nerve and provides motor innervation to various muscles of the forearm and hand as well as sensory supply to the skin of the hand.
The ulnar nerve can broadly be described as the nerve of the hand, as it innervates the vast majority of the intrinsic hand muscles. It is one of the most clinically relevant nerves of the upper limb, due to its superficial course and clinically apparent role in hand function.
S&S of diabetes-associated peripheral neuropathy?
Distal symmetrical sensorimotor polyneuropathy with glove and stocking loss of sensations such as: pain, vibration, temperature, proprioception.
Loss of motor function with clawed toes and small muscle wasting in hands and flexor muscles
Charcot joints: loss of sensation results in joint and ligament degeneration, particularly of the foot
Numbness
Loss of balance
Tingling
Pain
Clinical tests to screen for diabetes associated peripheral neuropathy
Careful history
Assessment of small nerve fibre function by testing thermal or pin prick sensation and light touch perception with a 10 g monofilament on the dorsal aspect of the distal great toe
Assessment of large nerve fibre function by testing vibration sensation with a 128 Hz tuning fork, proprioception (joint position sensation), and deep tendon reflexes at the ankle as compared with more proximal locations
Ankle and patellar deep tendon reflexes
Extension of the big toe, ankle dorsiflexion, and walking on heels to test motor strength
Romberg test, normal gait, and tandem gait to assess balance and fall risk. Pt stands with feet together (a soldier at attention) then they close their eyes.
Clinical assessment of the lower legs and feet to look for any ulceration or deformity
Causes of upper motor neuron lesions? What areas would the lesions be in to cause upper motor neuron issues?
Any injury to brain (stroke, infection, tumors), brainstem, or white matter of spinal cord.
What is a lower motor neuron lesion? Where is it occuring?
- Any damage to the lower motor neuron axon, which goes from the spinal cord to the muscle.
- Any injury to the ventral grey matter of the spinal cord (because this is where the lower motor neuron starts)
What effect does an upper motor neuron lesion have on deep tendon reflexes? Superficial reflexes?
Deep tendon are HYPERFLEXIC –> reflex becomes amplified because upper motor neurons are not able to regulate reflex at that level.
Superficial reflexes - diminished or absent
(Babinski reflex is positive)
Do you see muscle atrophy more in upper or lower motor neuron lesions?
Lower, because lower is what would normally deliver impulse to muscles directly. (may see weakness but not so much atrophy in upper motor neuron lesions)
Would you see a plantar response (babinski) if lower motor neuros lesions?
No
What is the effect of lower motor neuron lesions on deep tendon reflexes?
Absent
Would you see fasciculations in muscles with upper or lower motor neuron lesions?
Lower…. in this case, muscle may be affected but are still some single muscle fibres that are still stimulated (still some transmission from lower motor neurons that are not injured)
How common is huntington’s disease? How likely are you to inherit it if parents have it?
~ 1 in 7000 people affected in Canada
Hereditary
If one parent has HD 50 % chance, it’s passed on –> Is autosomal DOMINANT trait
Patho of huntington’s disease (HD). What chromosome is affected?
HD defect is on chromosome 4
Short version: abnormal DNA sequencing –> abnormal protein formation –> altered motor & mental functions
Repeated sequence of DNA (CAG nucleotides) causes formation of abnormal Protein leading to altered motor and mental functions
People with HD have 36 or more CAG (Glutamine) repeats on Huntingtin (HTT) gene (also referred to Polyglutamine disease) Normal: 10-35 times in a row
Chains of glutamine stick together and contribute to neuronal loss
Progression leads to tissue loss in basal ganglia
Recap Basal Ganglia Function: involved in motor function by providing feedback mechanism to motor cortex for initiation & control of voluntary movements
Regions affected by HD have decrease GABA and Acetylcholine and increased Dopamine levels
When does huntington’s usually present? Is it fatal? Fast or slow to progress?
Progressive, slow, fatal
Onset is usually around 40 years of age
The more repeats of CAG the earlier the onset of symptoms
Neuroradiological abnormalities can occur up to 15 years before clinical symptoms
What are the motor symptoms of huntington’s disease
Movement inhibition
Excessive involuntary movements
Hypotonia with hyperreflexia (early symptom), chorea (ex. Face, head, neck, tongue, trunk, extremities), athetosis, dystonia, eye movement slows, gait abnormalities (unsteady, irregular -> bradykinesia, rigidity late in disease)
What are the psychiatric, cognitive, and physical appearance symptoms of huntington’s disease
Apathy, irritability, depression, delusions, aggressions, disinhibition, paranoia
Cognitive: Progressive dementia, inflexibility of thought, decreased insight and concentration, memory loss
Weight loss, cachexia
How to diagnose Huntington’s disease?
MRI shows atrophy of head of caudate nuclei
Genetic testing for CAG repeats in HTT gene. Normal = 10-35 repeats. >36 repeats = HD
Other: History, physical examination
neurological: motor and mental status exams
Common causes of peripheral neuropathy?
commonly from diabetes mellitus, alcohol abuse, HIV infection
Other systemic causes of neuropathy (mostly axonal polyneuropathies) include chronic HIV infection, critical illness, end stage kidney disease, amyloidosis, hypothyroidism, vitamin deficiencies, lyme disease
Autoimmune polyneuropathies (most notably Guillain-Barre syndrome) are mostly demyelinating neuropathies
Toxic polyneuropathies are mostly from toxins including alcohol, chemotherapy exposure, heavy metals (causes a predominantly axonal polyneuropathy that could be acute, subacute or chronic
How do we describe the typical distribution of onset of peripheral neuropathy? What symptom is characteristic of acute polyneuropathies?
Weakness of lower legs and hands start as the syndrome progresses (the classic “stocking and glove” distribution of sensory loss)
Some polyneuropathies are acute (mainly caused by toxins), and those are characterized by pain
In peripheral neuropathies, do you typically see onset of sensory symptoms or motor symptoms first? What cause is a relatively common exception to this?
Sensory
Guillain Barre syndrome mostly affects motor neurons, and is an acute demyelinating polyneuropathy, and tends to first present with weakness instead of sensory loss
Diagnostic testing for peripheral neuropathies?
Clinical exam, extensive diagnostic testing usually unnecessary if mild symptoms with a known underlying cause (ie. Diabetes, alcohol abuse or chemotherapy)
Full diagnostic testing is warranted for those with no clear cause, or if symptoms are severe and rapidly progressive
Electrodiagnostic testing (with electromyography- EMG- and/or nerve conduction studies are initial testing when there is no clear etiology, or symptoms are rapidly progressive or severe
What are some s/s of Obstructive Sleep Apnea Syndrome (OSAS)?
Excessive loud snoring
Gasping
Multiple apneic episodes (temporary pause of breathing) that lasts 10 seconds or longer
What are some related progressions/manifestations that may be seen/associated in a patient with OSAS?
Obesity hypoventilation syndrome: may be r/t leptin resistance (leptin is a resp stimulant).
OSAS leads to hypercapnia and decreased O2 sats -> eventually leads to polycythemia, pulmonary hypertension, systemic hypertension, stroke, right-sided heart failure, dysrhythmias, liver congestion, cyanosis, and peripheral edema.
Hypersomnia (excessive daytime sleepiness) – can be so severe that people may fall asleep while talking, working, driving.
Impaired mood and cognitive function; symptoms include impairment of attention, episodic memory, working memory, and executive functions.
What are some treatment options for a person with OSAS?
CPAP, dental devices, surgery of upper airway and jaw if indicated, and obesity management
What is the most common complication of diabetes?
Peripheral neuropathy.
True or false?
Diabetic peripheral neuropathy incidence is higher in men, starts at an earlier age in men, and has a higher likelihood of causing muscle weakness in men. However, neuropathic pain is higher in women.
True.
____________ ___________ leads to gradual loss of integrity of the longest nerve fibres with symptoms starting distally in the toes and feet
Diabetic neuropathy.
What is happening to nerve cells when a patient is experiencing diabetic neuropathy?
Demyelination contributes to neural changes and delayed conduction occurs.
Both somatic and peripheral nerve cells show diffuse or focal damage resulting in polyneuropathy.
Describe sensory neuropathies.
Sensory Neuropathies: loss of pain, vibration, and temperature sensation usually in hands and feet first. Begins with being asymmetric however may become symmetric over time.
Describe motor neuropathies.
Motor Neuropathies: affect muscle groups, mostly in feet. These are common and may precede the sensory neuropathies or co-exist with them. Contributes to deformities and unstable balance. Alters the dynamics of walking and contributes to diabetic ulcers.
Describe autonomic neuropathies.
Autonomic neuropathies: delayed gastric emptying, diabetic diarrhea, altered bladder function, impotence, orthostatic hypotension, heart rate variability (both tachy and brady), sweating.
-Primary _____________ begins after age 40, increasing incidence after age 60
-More prevalent in males
-Second most common neurogenerative disorder (from Stats Canada)
Parkinson’s Disease
Describe the pathophysiology of primary Parkinson’s Disease.
Primary PD: -largely unknown
-Gene/environment interactions are probable cause
-Degeneration of basal ganglia with dysfunctional alpha-synuclein protein and loss of dopamine producing neurons in substantia nigra and dorsal striatum
-Depletion of dopamine and excess of cholinergic activity in the feedback circuit
In primary Parkinson’s Disease, individuals with tremor-predominant symptoms at diagnosis tend to have a quicker motor progression than those with balance and gait predominant symptoms.
True or false?
False.
Those with balance and gait predominant symptoms at diagnosis tend to have a quicker motor progression.
Patients diagnosed with primary Parkinson’s Disease in middle age tend to have a slower or quicker motor progression than those diagnosed later in life?
People who are diagnosed in middle age or earlier tend to have slower motor progression than those diagnosed later in life.
What effect does Parkinson’s Disease have on longevity?
Most individuals have normal or near normal life expectancy.
-Disease itself is not fatal, rather the complications (falls, etc)
What is myasthenia gravis?
Definition: chronic autoimmune neuromuscular disorder that affects the neuromuscular junction and is characterized by fatiguability and weakness of voluntary muscles
Hallmark: muscle weakness that increases during periods of activity and improves after periods of rest. Function best in AM, increasing weakness over course of day.
What age ranges in men and women is myasthenia gravis most commonly seen?
(5.3/million people in Canada are affected)
Two peaks in incidence:
- Women in 20-30’s
- Men in 60’s-70’s
Can occur at any age, but uncommon in youth.
What are some risk factors for developing myasthenia gravis (MG)?
Much is unknown
Frequently manifests for first time during pregnancy!
Women
Exposure to anaesthetic agents
Often associated with thymic hyperplasia or tumors (gland remains large and abnormal in MG)
Other autoimmune disorders (SLE, RA, thyroid disease)
What are some s/s of myasthenia gravis?
Classic triad:
- Ptosis
- Diplopia
- Dysphagia (sense of choking)
Difficulty chewing
Drooling
Slurring speech
Easily fatiguability
Symptoms more pronounced with fatigue or in evening
What might you see upon exam if a patient has myasthenia gravis?
Normal to increased DTR (I don’t know what DTR stands for -Gayle)
Normal coordination, sensory perception, and pupillary response
Eye movement dysfunction: upward/lateral gaze cannot be maintained for >30 sec
Ptosis with repetitive lid closure
Unable to sustain arm raise
Voice quality or speech change when counting to 100
Myasthenic crisis is life threatening and poses risk of respiratory arrest.
Occurs when respiratory muscles weakened to point of needing ventilator.
Usually triggered by infection, fever, or adverse reaction to medication.
Signs of impending MG crisis are?
Sudden onset inspiratory distress
Difficulty swallowing
Visual difficulty
Tachycardia
Rapid onset weakness
Quadriplegia/paresis
Myasenthia gravis is an antibody (Ab)-mediated autoimmune attack on acetylcholine receptors (AchR) at postsynaptic membrane of neuromuscular junction.
What type of hypersensitivity reaction is this?
Type 2
The presentation and time course of myasenthia gravis can be highly variable, this condition can be difficult to diagnose. REFER TO NEUROLOGIST.
If you suspect MG, what is a highly sensitive test you can perform in clinic?
What labs can you order?
Clinical exam: Ice pack test: apply ice to closed eyelid X 2 mins—> very sensitive test with less ptosis post ice exposure. Thought that by cooling muscle tissue, acetylcholinesterase is inhibited.
Lab test:
Ab titre for Ach-receptor Ab—> positive in 90% of MG patients
Anti-muscle-specific receptor tyrosine kinase Ab titre
What are the four stages of sleep?
N1, N2, N3 (non-rapid eye movement stages) and REM
Describe NREM N1 sleep stage. Which types of brain waves predominate this stage? What are some unique features of this stage?
Lightest stage of sleep, between wakefulness and sleep.
Brain produces theta waves
May experience hypnagogic hallucinations – hearing or seeing things that are not there
May experience hypnic jerks – feelings of falling/muscle jerks before falling asleep
Describe NREM N2 sleep stage. Which types of brain waves predominate this stage? What are some unique features of this stage?
Slightly deeper stage than N1, harder to wake
More theta waves, sleep spindles and K complexes
K complexes suppress cortical arousal to help keep you asleep and assist with memory consolidation
Describe NREM N3 sleep stage. Which types of brain waves predominate this stage? What are some unique features of this stage?
Very deep sleep, difficult to wake
Slow wave sleep, shows Delta waves
The stage during which sleep walking or talking will happen
Describe REM sleep stage. What are some unique features of this stage?
Also known as paradoxical sleep – because the brain is active but most muscles are paralyzed
Most dreaming occurs during this stage
Eyes move rapidly beneath eyelids, muscle relaxation, loss of temperature regulation, altered heart rate, BP and respirations, penile erection and clitoral enlargement
What is the most diagnosed sleep disorder?
Obstructive Sleep Apnea (OSA)
What are major risk factors for sleep apnea?
Obesity
Male gender
Older age
Outline the pathophysiology of OSA
Develops when there is a blockage of the upper airways
Allergies- cause swelling in the tissues of the nasopharynx
Swollen adenoids/tonsils – due to infection
Overbite – pulls jaw back and blocks the airway
Overweight/obesity – too much weight in the soft tissues of the neck which can weigh down the airway, especially when lying down.
The above are made worse by hormonal changes at night – causes the muscles around the airway to become slightly less stiff while sleeping, less able to keep the airway open and can become obstructed.
Differentiate central sleep apnea from OSA
Refers to the fact that the problem stems from the CNS
Brain intermittently stops making an effort to breathe for 10-30 seconds
The characteristic “hand of benediction” or “bishop’s hand” is the presentation of which arm nerve injury?
Median nerve injury can result in this position by causing an inability to flex the middle and index fingers
Describe the origin and function of the median nerve
Arises from the lateral and medial cords of the brachial plexus, enters the arm at the axilla and travels with the brachial artery down the humerus, into the cubital fossa and through the carpal tunnel into the hand. Innervates muscles of the anterior forearm and hand
What percentage of ALS patients have familial ALS?
What percentage of ALS patients have familial ALS?
What is the pathophysiology of ALS?
Motor neurons gradually breakdown and die, brain can no longer communicate with the muscles of the body, so the muscles weaken until they can no longer move
What is the average age of onset of ALS?
60-75 years
How long on average do people survive after an ALS diagnosis? What is usually the cause of death?
3-5 years, respiratory failure is commonly the cause of death for people with ALS
Describe some of the signs and symptoms associated with Huntington’s disease
Motor symptoms: Movement inhibition, Excessive involuntary movements, Hypotonia with hyperreflexia (early symptom), chorea (ex. Face, head, neck, tongue, trunk, extremities), athetosis, dystonia, eye movement slows, gait abnormalities (unsteady, irregular -> bradykinesia, rigidity late in disease)
Psychiatric : Apathy, irritability, depression, delusions, aggressions, disinhibition, paranoia
Cognitive: Progressive dementia, inflexibility of thought, decreased insight and concentration, memory loss
Weight loss, cachexia
What type of exams/tests are involved in a diagnosis of Huntington’s disease?
MRI shows atrophy of head of caudate nuclei
Genetic testing for CAG repeats in HTT gene. Normal = 10-35 repeats. >36 repeats = HD
Other: History, physical examination
neurological: motor and mental status exams
Define insomnia
Inability to fall asleep or stay asleep
Most common sleep disorder
What are the diagnostic criteria for chronic insomnia?
Difficulty initiating or maintaining sleep despite adequate opportunity for sleep, with associated distress or impairment of daytime functioning lasting 3 months or longer for at least three nights each week
What are parasomnias
- Unusual behaviours occurring during NRESM sleep
- Sleepwalking, night terrors, rearranging furniture, exhibiting sleep sex or violent behaviour, restless leg syndrome
- Somnambulism and night terrors are common in children and may be related to CNS immaturity
Define and describe somnambulism
Sleep walking
* Primarily a disorder of childhood and will resolve within a few years
* Not associated with dreaming
* Child has no memory when they wake up
* When occurs in adults, commonly associated with sleep disordered breathing
Define and describe night terrors
Night terrors: sudden episodes of apparent arousal with expression of strong fear or emotion
* Most often occurs in children
* Child is not aware, and can be difficult to arouse
* When awake, child has no memory of event
* Not associated with dreams
* Adults also may experience with related daytime anxiety.
Describe the path of the radial nerve through the arm
-BACK OF ARM: Starts at the brachial plexus (C5, C6, C7, C8, T1). Runs from radial groove to radial side of elbow to posterior forearm (runs down middle) to posterior wrist to posterior thumb, digit 1 &2
What does the radial nerve innervate
Motor: Primarily extensors. Muscles that extend the forearm at the elbow (posterior compartment muscles, including the triceps brachii), extensors for the wrist, fingers, thumb.
Sensory: Posterior forearm, radial side of dorsum of hand.
Where is the radial nerve vulnerable to injury?
-Humeral midshaft fractures, shoulder dislocations, or arm compression injuries (trauma, tourniquets)
-Can be injured by improperly fitting crutches
-Also vulnerable to injury where it passes through the supinator muscle (repetitive forearm rotation; fracture)
-Can also be injured near wrist (i.e., tight handcuffs)
What nerve does carpal tunnel affect
Medial nerve
Patho of carpal tunnel
Swelling of tendons in the carpal tunnel causes nerve compression
S&S carpal tunnel
- Tingling and numbness of the thumb, index, middle and ring fingers.
- Can also cause pain in the wrist with numbness/tingling
- Typically, the patient wakes at night with burning or aching pain and with numbness and tingling and shakes the hand to obtain relief and restore sensation.
- Long term compression leads to thenar muscle atrophy and weakness of the thumb and index fingers
Treatment carpal tunnel
- Neutral position splinting
- Treatment of any underlying disorders
- Corticosteroid injection
- Surgical decompression
Describe 2 tests used in dx of carpal tunnel
Tinel and phalens test
Describe tinels test. What is it for?
Dx of carpal tunnel
How it’s done: lighting tapping on wrist over median nerve elicits a sensation of tingling or “pins and needles” in the distribution of the nerve.
Patho: It is elicited by the tapping (percussion) of an injured nerve trunk at or distal to the site of the lesion.
Sensitivity and Specificity: 25-75% and 70-90%, respectively
What is phalens maneuvre?
How it’s done: The patient places their flexed elbows on a table, allowing the wrists to fall into maximum flexion. The patient is then asked to push the dorsal surface of their hands together and hold the position for 30-60 seconds. It is considered positive when the patient’s symptoms are reproduced (namely paresthesia) in the distribution of the median nerve.
Patho: The pressure on the carpal tunnel increases –> compresses the median nerve between the transverse carpal ligament and the anterior border of the distal end of the radius.
Sensitivity and Specitivity: 51-91% and 33-88%, respectively.
What are early S&S of ALS?
-Early signs and symptoms: tripping, dropping things, slurring or thick speech, difficulty swallowing, weight loss, decreased muscle tone, DOB, increased or decreased reflexes, uncontrollable laughing or crying, feeling weak, fatigue, muscle cramping or twitching, muscle stiffness or rigidity
Describe how symptoms of ALS progress
Over time the muscle weakening will continue to spread throughout the body, eventually causing difficulties with breathing, chewing, swallowing and speaking.
- The senses of sight, touch, hearing, taste and smell are usually not affected, and for many people, muscles of the eyes and bladder remain functional until very late in the disease.
S&S of spinal form of ALS
60% of people have spinal form of disease
* Focal muscle weakness beginning in arms and legs progressing to muscle atrophy, spasticity, and loss of manual dexterity and gait
* No associated mental/ sensory/ autonomic symptoms present
S&S of ALS with progressive bulbar palsy
ALS with progressive bulbar palsy
* Presents with difficulty speaking and swallowing
* Peripheral muscle weakness and atrophy occur withing 1-2 years
* Poorer response to treatment with mech vent
What are 4 classic motor symptoms seen in PD (parkinsons disease)
tremor, rigidity, akinesia/ bradykinesia, postural instability
What accounts for the shuffling gait in PD?
- Postural abnormalities (flexion, leaning forward), difficulty walking, and weakness develop as neurodegeneration progresses. Unable to make postural adjustment; festinating gait (short, accelerating steps) is an attempt to remain upright
Describe some non- motor impairments of PD?
Non-motor Impairments
Psychiatric
* Cognitive impairment
* Depression, anxiety
* hallucinations
* Sleep difficulties → wild dreams, excessive day time sleepinesss
* Mood disturbances
* Decreased motor learning ability
* Dementia (advanced stages)
Micrographia
Autonomic dysfunction
* Orthostatic hypotension
* Constipation, gastric retention
* Urinary retention
* Drooling
* Increased sweating
Decreased sense of smell (olfactory)
* Common first symptom → Hx of decreased or changed sense of taste or smell… prior to motor symptoms
Complications of PD?
COMPLICATIONS OF PARKINSON’S
Freezing phenomenon
* Progressive hypokinesia, bradykinesia → akinetic pause in movement
* Common when walking
* Tend to occur at thresholds (e.g., door frames)
Falls
* Secondary to postural instability, poor movement amplitude
Dystonia → muscles contract involuntarily.
* Abnormal tone across joints → disfiguring, painful, posturing
* Universal flexion of joints → severely kyphotic posturing
* Ultimately results in poor ability to ambulate and ventilate
Dementia
* Common after prolonged, primarily motor disease (in contrast to Lewy body dementia)
* Psychosis, hallucinations (severe)
* Progressive (more common in age >70). Further compromise of mental status may occur by the adverse effects of the medication taken to control symptoms
NOTE: Despite the various motor symptoms of PD, there is NO WEAKNESS of muscles. This differentiates PD from motor cortex or corticospinal pathway disease
What is the diagnostic test for PD?
There is none. It is based on history and clinical presentation. Suspect in anyone with tremor, stiffness, slowness, balance issues, or gait disorders. CT/ MRI should not be routinely used. Trial of dopamine therapy- if it improves symptoms, helps to confirm diagnosis.
