PD CH 8-9

Neuropathic pain/ neurogenic pain/ neuralgia/ nerve pain

Caused by damage or disturbance to the CNS or PNS \> see peripheral nerve injuries

symptoms of Neuropathic pain

Burning, stinging, cutting, electric sensation
Radiating pain
Hyperpathia
Allodynia
Hyperalgesia
Paresthesia

Treatment for Neuropathic pain

Difficult to treat
Often persists for weeks to months \> chronic pain
Maybe treated to anti-depressants
Can use self hypnosis to reduce pain complains

Neuromuscular disorders

disorders of the anterior horn, peripheral nerves, muscles or the transition between nerves and muscles; neuromuscular disorder

Motor neuropathy

(Neuromuscular disorder) example:
Amyotrophic lateral sclerosis (ALS)
Spinal Muscular Atrophy (SMA)
‐Type 1 - Wasning-Hoffman
‐Type 2 - Intermediate form
‐Type 3 - Wohlfart-Kugelberg-Welander
‐Type 4 - Adult form
Poliomyelitis and post-polio syndrome

Radiculopathy

(Neuromuscular disorder) diseases of the nerve root example: Discus hernia

Peripheral neuropathy

(Neuromuscular disorder) disorder of the axons example: Mononeuropathy, Multiple mononeuropathy, Polyneuropathy (Guillain-Barré Syndrome (GBS), Charcot-Marie-Tooth (CMT) \= Hereditary Motor Sensory Neuropathy (HMSN))

Neurodegenerative illness

(Neuromuscular disorder) disorder of axon example: Hereditary Spastic Paraparesis (HSP)

Myopathy

(Neuromuscular disorder) disorders of muscle tissue example: Duchenne Muscular Dystrophy (DMD)

PNS disorders: causes

æCaused by:
Trauma, e.g. electrical injury, traction, rupture
Pressure
‐Internal, e.g. oedema, tumour, entrapped
‐External, e.g. crush injury
Disease, e.g. infection, diabetes, auto-immune diseases, shingles or herpes virus, carpal tunnel syndrome,, ...

PNS disorders: consequences

æConsequences depending on:
Which nerve affected: motor or sensory, autonomic or not
Which part of the nerve affected: root, plexus, axon, myelin sheath
Number of nerves affected
Location of the nerve(s) affected
Severity of the injury

Amyotrophic lateral sclerosis (ALS)

Degeneration of the motor anterior horn cells + medulla + pyramidal tract. Onset: 50-60y, more common in men. Progressive \> poor prognosis

Amyotrophic lateral sclerosis (ALS) cause

Cause: unclear \> multifactorial \> about 10%: genetic factor

Amyotrophic lateral sclerosis (ALS)

Muscle weakness and spasms \> in one arm or leg

Amyotrophic lateral sclerosis (ALS)

Usually starts in:
Small hand muscles of one hand \> impact on precision grip and fine finger movement
Stretch muscles of one foot \> foot drop
Muscles of tongue and throat \> articulation problems à later: swallowing disorders

Amyotrophic lateral sclerosis (ALS)

Evolution to extremities, trunk, mouth/pharynx \> breathing problems cause death within 3-5y after first symptoms
Typical:
Tongue paralysis
Fasciculations - small muscle contraction

Spinal Muscular Atrophy

Fault in DNA \> shortage of protein SMN that keeps motor nerve cells alive \> some motor nerve cells in the spinal cord do not function \> no or faulty signals to muscles à muscle paresis and atrophy

Treatment
Available since 2016 \> still very expensive, not widely accessible and strictly limited
Administration: epidural injection several times per year or oral

Collection of illnesses \> classified according to age at onset

Type 1 - Wasning-Hoffmann disease (Spinal Muscular Atrophy)

0-6m
Nearly motionless babies, arms and legs hardly used
Cannot sit up without help
Respiratory muscles also affected \> breathing problems and swallowing disorders
Serious course of the disease and poor life expectancy (90% dies before 5yo)

Type 2 - Intermediate form (Spinal Muscular Atrophy)

6-18m
Halt or regression in motor development
Worsening of muscle weakness \> gradually or quickly
Increase in paralysis
Usually sitting up without help is possible, walking not
Respiratory problems too
Life expectancy: 10-40 years

Type 3 - Wohlfart-Kugelberg-Welander disease (Spinal Muscular Atrophy)

18m-4y(-30y)
Until then: normal development with milestones at normal ages
First symptom: muscle weakness in the legs à gradual deterioration
Usually wheelchair bound at 20-40yo
Life expectancy: adulthood \> depending on onset and rate of deterioration

Type 4 - Adult form (Spinal Muscular Atrophy)

Extremely Rare
First symptom: muscle weakness in the legs \> slow and mild deterioration

Poliomyelitis/ polio/ infantile paralysis

Caused by poliovirus \> inflammation of the grey matter in the spinal cord. Often asymptomatic

prodromal phase (Evolution of Poliomyelitis)

flu-like

acute phase (Evolution of Poliomyelitis)

‐Non-paralytic phase: virus in throat and GI tract \> headache, vomiting, meningitis-like, painful limbs
‐Paralytic phase: virus in motor neurons in the anterior horn \> asymmetric flaccid paralysis lower or upper limbs

recovery phase (Evolution of Poliomyelitis)

new connections by axons of remaining motor neurons \> recovery of motor control \> but one motor neuron needs to control more muscle fibers \> decreased muscle strength remains

chronic phase (Evolution of Poliomyelitis)

no cure for paralysis \> leads to permanent muscle weakness or deformities (in growing children) \> high risk of contractures

relapse phase (Evolution of Poliomyelitis)

possible until years after recovery \> \= post-polio syndrome \> other symptoms: fatigue, loss of function, joint and muscle pains

Radiculopathy

Affected nerve root \> myotome affected and possibly dermatome too
Common causes:
‐Compression of lumbar or cervical herniated disc
‐Trauma \> nerve root is severed
‐Infection

Lumbar discus hernia

Excessive rotation, lifting, ... \> pressure in nucleus pulposus increases \> disc prolapse \> presses on nerve root
Symptoms:
‐Back pain \> followed by radiating pain in the leg
‐Depending on the location of the discus hernia
2% of people
67% L4-5 and 28% L5-S1

Mononeuropathy

one peripheral nerve is damaged

Multiple mononeuropathy

several peripheral nerves are damaged \> but in a non-systematic way

Polyneuropathy

several peripheral nerves affected in a systematic way
Common causes:
-Diabetic neuropathy
-Guillain-Barré Syndrome (GBS)
-Charcot-Marie-Tooth (CMT)

Guilain Barre syndrome

polyneuropathy cause by an autoimmune reaction \> system will attack the peripheral nervous system. Starts with deterioration of myelin sheath
Exact cause unknown, but often cross-reaction of antibodies formed in an infectious episode with a bacterium or virus
Rare
More common in men

Guilain-Barre Syndrome symptoms

Start: mild sensory complaints, mostly in lower limbs
Progressive deterioration: progressive muscle weakness and paralysis, also in upper limbs
Afterwards also muscles of face, throat, tongue, respiratory system (20-30%)
If autonomic NS is affected: constipation, incontinence, blood pressure, heart
Stabilization: recovery \> 85% complete recovery in 6-12m

Neuropraxis - Sunderland grade I (Classification by Seddon or Sunderland)

‐Nerve is intact
‐Complete recovery possible fast
‐Up to few hours

Axonotmesis - Sunderland grade II (Classification by Seddon or Sunderland)

‐Axon damaged, myelin sheath intact
‐Regeneration possible but slow: axon grows within myelin sheath
‐1mm/day à up to months
‐During regeneration: prevent muscle atrophy!

Sunderland grade III (Classification by Seddon or Sunderland)

Axon and endoneurium damaged

Sunderland grade IV (Classification by Seddon or Sunderland)

‐Axon, endoneurium and perineurium damaged

Neurotmesis - Sunderland grade V (Classification by Seddon or Sunderland)

‐Axon and myelin sheath damaged
‐Regeneration unlikely
‐Poor prognosis

Miller Fisher Syndrome

variant of guillian barre, patients will experience weakness around the eye muscles which makes eye movements difficult.

Charcot-Marie-Tooth

Hereditary Motor Sensory Neuropathy
Hereditary \> abnormality in DNA \> expressed in nerve fibers
Motor and sensory nerves are affected \> peripheral ones
Collection of illnesses with types and subtypes

Charcot-Marie-Tooth symptoms

Muscle weakness
Sensory disturbances
Varied image: more severe symptoms when more neurons are affected

Type 1 (Charcot-Marie-Tooth)

(Charcot-Marie-Tooth)
myelin sheath around the nerve fibers is damaged \> nerve impulses are not transmitted effectively
More males
Onset: 10 yo

Type 2 (Charcot-Marie-Tooth)

(Charcot-Marie-Tooth)
axons are damaged \> number of fibres decreases
More females
Onset: later

X-linked/sex-linked type (Charcot-Marie-Tooth)

(Charcot-Marie-Tooth)
mixed form
Males usually more severely affected
Onset: yet later in women

Hereditary Spastic Paraparesis

Hereditary \> fault in DNA \> spinal nerves degenerate \> work increasingly poorly \> decreases muscle strength and increases stiffness \> can lead to spasticity

Hereditary Spastic Paraparesis Symptoms

Weak lower limbs
Stiff or spastic leg muscles
Sensory issues \> due to affected ascending sensory nerves
Worse in cold weather
Slow deterioration with age \> common evolution: walking stick \> crutches \> wheelchair
Onset: usually adulthood, sometimes childhood
Normal life expectancy

Duchenne Muscular Dystrophy

Hereditary \> through mother
Cause: fault in DNA \> shortage of protein dystrophin in cell walls of muscle cells \> cells loose resilience and strength \> cells are damaged \> eventually die \> dead muscle cells are replaced by connective tissue cells
Onset: < 2yo
Nearly only boys affected
Prevalence: 1/3.5000
Treatment: symptomatic only, no cure

Duchenne Muscular Dystrophy symptoms

-Late milestone to start walking (18m instead of 12m)
-Weak lower limb muscles à difficulty to stand up from squat (need to place hand on upper legs) à progressively worse à affects also respiratory and heart muscles à often oxygen needed as of 20yo
-Difficulty running, climbing stairs
Thickened, hard calves
-Wheelchair bound as of 8-12yo
-Sometimes intellectual disability too

Cerebral palsy (CP)

\= infantile encephalopathy
\= clinical syndrome characterized by a persistent postural or movement disorder due to a non-progressive pathological process that has damaged the brain during development in the first year of life

Risk factors:
Premature
Low birth weight
Multiple pregnancy
Common: 2-3/1.000

Causes (brain) of Cerebral palsy

Lack of oxygen
Infection
Bleeding
Malformation

Symptoms of cerebral palsy

Motor impairment: abnormal muscle tone, strength, control à abnormal posture and movements
Disorder in cognition, communication, perception, behaviour
Keep on changing during growth (even though non-progressive)

Possible consequences:
Contractures
Bone deformities
Growth disorder
Problems with coordination and balance
Learning and speech difficulties
Epilepsy

Spastic paresis (Cerebral Palsy)

-Inability to regulate muscle tonus \> usually hypotonic trunk but hypertonic, spastic extremities \> stiff muscles in the extremities with little variety possible in movement \> scissor and toe walk

Causes problem with movement and posture
Worse during activity, better during rest

Negative symptoms: decrease in normal muscle functions

Paresis: muscle weakness
Decrease in selective movement: in focused, voluntary, independent muscle movement

Hypertonia (Spastic paresis- Positive symptoms)

‐non-speed-dependent muscle stiffness during passive movement (\= rigidity)
‐caused by continuous activation of the muscle or mechanical cause
‐unevenly distributed: more stiffness in flexors in upper limbs, but less in extensors in lower limbs

Spasticity (Spastic paresis- Positive symptoms)

‐speed-dependent resistance during (passive) movement
‐caused by imbalance in neurological pathway in activation versus inhibition à overexcitability of the stretch reflex à causes tonic stretch reflexes (muscle tone) and exaggerated tendon reflexes (hyperreflexia)
‐more common in some muscle groups
‐affects growth and development of skeletal muscles à less and shorter muscle and longer tendon (see fig) à weaker muscle
‐causes contractures of joints and muscles

Spastic paresis- Positive symptoms

Abnormal posture reflexes: primitive reflexes continue

Dyskinetic paresis (Cerebral Palsy)

damage to the basal ganglia; involuntary, uncontrolled and repetitive movements, even at rest

Ataxic (Cerebral Palsy)

Cerebellum is infected; loss of normal muscle coordination and movements with abnormal strength, rhythm and precision. Children with this are unsteady with their feet and spread abnormally wide apart when they walk. Experience tremor, peculiar movements and inadequate reactions.

Spasticity (80-90%), Dyskinesia (9%), Ataxia (2%)

3 types of motor disorders

Gross motor function classification system (GMFCS)

-Based on spontaneous movements with focus on sitting, transfers and mobility
-5 groups for 5 age groups (0-18y)
-Mostly differentiated according to functional limitations and use of walking or transport aids \> attempt to focus on potential rather than limitations
-Gives functional prognosis

Manual ability classification system (MACS)

-Based on use of both hands to handle objects in activities in their daily life
-5 levels, 1 age group (4-18y)

0-6y

Cerebral palsy (CP): development of children

increased spasticity (especially 9-24m)
support gross motor skills and coordination and correct posture
if immobile: hip and knee extension

6-12y

Cerebral palsy (CP): development of children

support fine motor skills and ADL
growth spurt à prevent muscle contractures

+12y

Cerebral palsy (CP): development of children

continue to support for ADL: hobbies, education, work

Cerebral palsy (CP): treatment

-Multidisciplinary
-Aimed at functionality
-Occupational and physiotherapy: conservative treatment
Especially important for young children (0-6y) during development of gross motor coordination
Passive mobilization and active exercise
-Aids
Stand aid
Tricycle, ...
Orthoses, serial casting, ...
Communication
-Medical treatment
Muscle relaxants: oral, botulinum injections, baclofen pump
Surgery to muscles, tendons, bones, nerves, ...

Traumatic brain injury (TBI)

\= non-congenital brain injury
\= injury to the brain that occurs after the age of 1y

Traumatic brain injury (TBI) Causes

Trauma: fall, traffic accident (motor), struck with heavy object, skull fracture, ... à called concussion
Pathological process
‐Cerebrovascular accident (CVA)/stroke: infarction or bleeding
‐Infection
›Encephalitis
›Meningitis
›Sepsis (blood)
‐Tumour
‐Poisoning or intoxication
‐Hypoxia or anoxia: lack or absence of oxygen, e.g. cardiac arrest, drowning
‐Drugs, medication, alcohol

Traumatic brain injury (TBI) symptoms

-Depending on nature, location and extent of the brain injury
-Located on contralateral hemisphere
-Increased impact in case of coup-contrecoup injury
-Effect on functioning:
Motor
Sensory
Cognitive
Emotional
Neuropsychological
-Permanent or temporary
-Severity measured by Glasgow Coma Scale (GSC) à gives indication of post-traumatic amnesia (PTA) and disability

Traumatic brain injury (TBI): postconcussion syndrome

-After moderate or severe brain injury
-Symptoms:
Vomiting
Persisting headache
Fatigue
Concentration problems
Memory loss
Depression
Fear
Apathy

Cerebro-vascular accident (CVA)

\= stroke
\= accident in the blood vessels of the brain
Type of non-congenital non-traumatic brain injury
In Belgium: 60/day, of which 11 fatal and about 30% permanent disability and in need of care

Ischemic stroke/infarction

- blood clot stops the flow of blood to an area of the brain
- Blood is suppressed because of a blocking from the inside (e.g. thrombus, cardiac embolus) or compressing from the outside (e.g. swelling)à abrupt decrease in supply of oxygen and glucose à neurons die à permanent damage

Haemorrhagic stroke

Usually due to a burst blood vessel or can be due to trauma
Short term effects worse than long term, as mass of haematoma decreases, function may return to affected area

Haemorrhagic stroke causes

Long-term hypertension \> changes blood vessel wall: more rigid and less able to expand \> especially in medium sized arteries \> increased risk of atherosclerosis at preferred site \> weakens vessel wall \> development of local distension (\= aneurysm) \> risk of aneurysm rupture \> haemorrhage
Trauma \> bleeding

ischemia (CVA: risk factors)

Heart problems
Hereditary coagulation disorders

brain haemorrhage (CVA: risk factors)

Coagulation disorder: small rupture can lead to massive bleeding
Use of "blood thinning" medication

For all types of CVA (CVA: risk factors)

Modifiable:
‐Hypertension
‐Smoking
‐Dyslipidaemia
‐Diabetes
‐Obesity \> abdominal
‐Sedentary lifestyle
Non-modifiable:
‐Previous CVA or TIA in patient or family
‐Gender
‐Ethnicity
‐Age

CVA: symptoms

FAST (face \= other part of the face doesn't have muscle control, arms\= Can they raise both arms and keep them there, speech\= Is their speech slurred , time\= Call ambulance immediately)

Facial paralysis

CVA: possible residual symptoms

difficulty blinking the eyelid
corner of the mouth hangs down

Aphasia

CVA: possible residual symptoms

Broca aphasia: no to poor speech (normal understanding
Wernicke aphasia: impaired language comprehension

Hemianopsia

CVA: possible residual symptoms

Half-sided visual field lost in both eyes

Neglect

CVA: possible residual symptoms

The patient does not pay attention to one part of the body hemisphere.

Agnosia

CVA: possible residual symptoms

Failure to recognize or understand a perceived object.

Psychological dysfunction

CVA: possible residual symptoms

Right hemisphere: insecurity and anxious behaviour
Left hemisphere: disorientation and neglect, often impulsive and know no fear

Cerebro-vascular accident (CVA): treatment

-Emergency CT-scan to determine type of CVA
-Infarction:
Thrombolysis with IV thrombolytics à dissolves clot à reperfusion: blood supply is restored
Within 3h after first symptoms
-Haemorrhage:
Stop blood thinners
If not life-threatening: wait-and-see
Surgery:
‐close aneurysm by clipping or coiling
‐remove blood
-Rehabilitation for residual symptoms
Multidisciplinary
Most recovery in first 6w
Recovery up to 2y

Multiple sclerosis (MS)

-Pathophysiology: inflammatory reactions à plaques/lesions in brain and spinal cord à demyelination around the nerve endings in these plaques à scar tissue (\= sclerosis)
-Cause: genetic sensitivity and auto-immune? à with environmental triggers:
Vit D deficiency
Obesity in early life
Smoking
Hygiene hypothesis
-Risk factors:
White race
Female gender
-Onset: around 30y à diagnosis: around 30-50y
-92/100.000

Multiple sclerosis (MS): symptoms

-Symptoms are not specific \> onset often unnoticed
-Spatial distribution: multiple lesions in multiple locations
-Most frequent:
Fatigue
Paresthesias in limb(s), trunk ("MS hug"), face (one-sided)
Weakness and clumsiness
Visual problems: nystagmus, partial blindness, unilateral eye pain, diplopia, blind spots, blurred vision, ...
Stabbing pain
-Other symptoms:
Temporary optic muscle paralysis
Transient poor muscle strength
Stiffness or fatigue of a limb, spasms of lower limb, tremor
Mild walking disorders
Bladder control problems
Vertigo
Mild emotional disturbances
Loss of coordination and balance
Dysarthria

Multiple sclerosis (MS) Diagnosis

Diagnosis

MRI
Evoked potentials
Lumbar puncture: leukocytes and immunoglobulin

Multiple sclerosis (MS) Evolution of Symptoms

Evolution of Symptoms

Slow: up to 25y
Variable: large variety of neurological symptoms
Unpredictable: with flare-ups and remissions
Worse prognosis: male, older, cerebellum, rapidly changing flare-ups with less recovery in between

Multiple sclerosis (MS) Treatment

Treatment

No cure
Immunotherapy to slow down progression
Avoid heat

Dementia

-Syndrome
-Clogs of proteins \> loss of brain cells
-Cognitive and non-cognitive symptoms:
Impaired memory and comprehension
Behavioural problems: agitation, aggression
Psychological problems: delusions, hallucinations, depression
-Risk factors: age and female gender
-Symptoms:
Non-specific at onset \> become more specific when problems with ADL
Forgetfulness \> other than normal age-appropriate forgetfulness \> sensitive signs:
‐Dementia as diffuse process: other cognitive problems: word-finding problems, failure to recognize objects or persons, loss of initiative
‐Head turning sign: check others for confirmation
-Evolution: varies, 5-10y
-Treatment: no cure

Spina bifida

\= neural tube defect
-Developmental disorder: during first weeks of pregnancy
-Vertebral arches remain open \> spinal cord, meninges, ... are not protected \> can be damaged and/or come out
-Location: usually lumbosacral \> sometimes higher up

Spina bifida aperta

Spina bifida aperta
‐2 types
›Meningocele: meninges have come out, in a cyst filled with brain fluid à nerve tissue not affected à nearly no neurological problems
›Myelomeningocele: meninges + myelum/spinal cord have come out à nerve tissue is deformed à significant neurological problems
‐Covered by skin or not

Spina bifida occulta

‐Hidden: not visible externally, closed skin
‐Sometimes swelling palpable à sometimes lipoma
‐Sometimes tuft of hair, birthmark, dimple, ...
‐Nearly no neurological problems
‐Sometimes tethered cord: fatty tissue fixates the end of the spinal cord

Spina bifida: symptoms

1. Walking difficulties
2. Reduced sensation in legs and feet
3. Increased risk of burns and pressure sores
4. Sexual dysfunction
5. Deformities of spine (scoliosis)

Spina bifida: treatment

Treatment

-Multidisciplinary
-Surgery:
In first days after birth
Closing meningocele
Followed by liquordrainage during 3-4w to treat hydrocephalus
æFor muscle imbalance and secondary contractures and luxations:
Conservative treatment:
‐Passive mobilization and active exercise
‐Cast
‐Verticalizing the patient with standing device
Medical
‐Surgery to muscles, tendons, bones, nerves, ...
‐Muscle relaxants: oral or injection with botulinum

Spinal cord injury: etiology

Etiology

-15-20/100.000/year
-75% under 40y

-Causes:
Traumatic 35%
‐Falls \> especially elderly
‐Traffic accidents
‐Violence
‐Work accidents
‐Sport accidents: horses and football
Non-traumatic 65%
‐Tumor
‐Degeneration of vertebrae
‐Vascular abnormalities
Inflammation

Spinal cord injury: symptoms and evolution

-Varies between extremes:
Complete: full paralysis and full loss of sensibility
Incomplete: minor to severe loss of strength and sensation
-Not only the injury in itself causes loss of function, but also secondarily the swelling \> spinal shock \> flaccid paralysis \> temporary up to several days-weeks \> evolves to spastic paralysis with hyperreflexia \> is chronic with possible recovery
-Possible functions affected:
Motor functions
Sensory functions
Autonomous functions

Spinal cord injury: AIS classification

-ASIA Impairment Scale (AIS) by American Spinal Injury Association (ASIA)
-Based on most caudal spinal cord segment that scores normal for motor skills and sensibility
-Test: dermatomes and myotomes left and right in supine position à scored for presence and level of strength and feeling
-Complete lesion:
Anal region (S3-5) is unable to contract randomly (motor) or is not sensible (sensory) à \= no sacral sparing/activity
Zone of partial preservation (ZPP): no sacral sparing, but sensation and/or motor activity present below the lesion level
-Incomplete lesion: all other lesions à some information passes through the lesion

Tetraplegia \= quadriplegia

Cervical lesion
Function of legs, arms and trunk affected
High tetraplegia \> C1-4
Low tetraplegia \> C5-8

Paraplegia

Thoracic, lumbar or sacral lesion
Function of lower limbs affected
Depending on level of lesion: trunk affected
Function of upper limbs not affected