Chapter 11- Neurological Disorders Flashcards
Define paralysis
loss of muscle function of one or more muscle groups
Define PAresis
incomplete or partial paralysis
What causes Paralysis?
often trauma to brain or spinal cord, but other major causes include tumors, cascular insults, motor neuron or demyelinating disease, inflammation or infection.
What do you call paralysis or paresis of a single limb?
monoplegia/monopareisus
What do you call paralysis that is bilateral (lower limbs usually) -
Diplegia
- often used when describing cerebral palsy.
What do you call paralysis or paresis affecting one side of the body?
hemiplegia/hemiparesis
What is the most common cause of hemiparesis
cerebrovascular disease
upper limb is usually more affected than lower limb
What do you call paralysis or paresis of the lower trunk and legs?
Paraplegia/paraparesis
What do you call the most severe form of paralysis or paresis that affects both armsd and both legs?
quadriplegia/tatraplegia and tetraparesis.
commonly caused by trauma, in higher cervical spinal cord region.
Paralysis is defined by the area of the body affected and physiologic characteristics often noted as spastic of flaccid. Defione these terms
spastic- associated with motor nerve damange in CNS, is exaggerated deep tendon reflexes and forceful muscle spasms.
Flaccid- associated with cranial or spinal nerve lesions can causes loss of muscle tone.
What do you call limbs that are fixed in bent positions d/t spasms?
contractures
What is Cerebral palsy (CP)
general term that describes a group of disorders characterized by motor deficits that limit activity and often MSK problems, learning disability and behaviour problems.
- arise from brain damage during gestation or time or birth
True or False
Cerebral palsy is the leading cause of chilhood disability
true
CP has 3 major subgroups. Name them
- Dyskinesia syndrome-
- Spastic CP
- Ataxic
define Dyskinesia syndrome
hemiplegic/ demiplegi
involuntary movements, creating abN postures and facial grimaces.
- these movements can be singularly or mixed
a) Athetoid- slow, smothing distal myscles
b) chorea- irregular, unpredictable contractions of single muscles or groups
c) Dystonia- repetitve, patterened twisting of trunk and limbs
Define Spastic CP
result of upper motor neuron disease
- sxs hyperreflexia, spastic hypertonia, and retention of primittive reflexes
Define Ataxic CP
involves gait abnormalities
How is CP diagnosed?
ultrasounds' MRI EECG Sensory evoked potentials -these also evaluate degree of neuro involement.
What is the tx course for CP?
maintaining mobility via physiotherapy
orthopedic device
surgery
speech therapy
The prognosis depends on sevrity of sxs. What does an UW need to review?
- extent and severity of disability
- intellectual impairment
- hx of siezures
- independence?
- mobility abilityt
What are the common causes of spinal cord injuries in the US?
MVA
Falls
Violence
Sports injury
Males 4x > females under 30.
spinal cord damage is associted to what?
the location of the injiry on the vertebral column, an is usually defined by that area.
motor, and sensory function, reflexes and sphincter tone are nromal above lesion, and absent below.
The degree of spinal cord injury impairement is measured using the american spinal cord injurt association (ASIA) scale. What are the 5 grades?
A- complete, no sensory or motor function preserved
B- incomplete, sensory by no motor function is preserved below injury.
C- incomplete, motor is preserved below level of spinal cord and half the key muscles have a muscle grade <3
D- incomplete, motor function preserved below injury and at least half the muscles have a muscle grade >3.
E- normal, motor and sensory function has returned.
What are some favourable features in the prognosis of those paralyzed by a spinal cord injury?
one limb affected and fully ambulatory resp function ok bowel and bladder not affected renal function nromal independent well adjusted to condition no evidence of associated substance abuse
What are some unfavourable features in the prognosis of those paralyzed by a spinal cord injury?
multiple limbs affected artificial ventilation required bowel and bladder funciton impaired impaired renal function requires assistance in DALs poor adjustment/depression evidence of drug/alcohol abuse
What is Peripheral Neuropathy ?
what are some signs and sxs?
sensory issued caused by damage to the peripheral nervous system. Can also involve motor function.
- neuripathic sxs (numbness, burning, aterd sensation)
- muscle weakness,
- organ/gland dysfunction
- changes on EMG or NCS studies
Define the peripheral nervous system
the communicating network between the CNS and the rest of the body.
Mononeuropathies affect a single nerve. What are some examples of mononeuropathies
- bell’s palsy
- CTS
- Thoracic outlet syndrome- pain/paresthesia of arm caused by compression of the brachial nerve
- mononeuritis multiplex- inflammatory mononeuropathy that involves nerves in at least two area:
What is polyneuropathies?
affects multiple nerves and can occur in acute forms (guillard-Barre syndrome) or chornic forms (CIDP)
Automatic neuropathy is a peripheral nerve dysfunction that affects nerves involved in regulating involuntary muscles involved in what?
- maintaining HR
- BP
- Gastric mobility
- Perspiration
- Pupil dilation
- Glandular funciton
What are the symptoms of autonomic neuropathy .
syncope,
heart cant respond to changing conditions
gastroparesis
urinary retention
erectile dysfunction
decreased Sweating
and inability of the pupil of the eye to adjust to change in light
What are common disease-related causes of peripheral neuropathy?
- metabolic disorders
- generalized vascular disease
- hereditary disease
- toxicity
- connective tissue and chronic inflammatory disorder
- vitamin defficiencies
- infection
- paraneoplastic syndrome.
How do you diagnoses peripheral neuropathy?
extensive hx
clinical examination
tests: blood, nerve conduction, CT and MRI and Electromyography.
Never biopsy possible
what is the treatment for neuropathy?
the control of the underlying disease
rx: gabapentin, duloxetin, pregabelin, and tricyclic antipressents help with pain.
lidocaine patches- local
What is diabetic neuropathy?
Damage to sensory nerve fibres, or automic nerves due to prolonged periods of hyperglycemia
damage results to vibratory sensation and propricerption
- impaired sensation to pain and temperature
How do you test diabetic neuropathy
- exam of feet
- evaluation of reflexes
- vibratory senstation
- monofilament testing or pinprick
How is diabetic neuropathy diagnosed>?
- ruling out the other cause of peripheral neuropathy
- control of hyperglycemia
- education of the individual in protecting the feet and hands from the environement
- periodic food examination by the personal care physician
- treating the autonomic symptoms symptomatically
What is Guillain-Barre
immune-mediated inflammatory condition.
- onset is often noted after mild bacterial or viral infection.
- progressive wekness in legs/upper body
- severe cases have total body apralysis and eresp distress
- ICU admitted d/t CV and resp monitoring
- most recover in weeks to months
- some cases lead to perm disability
How is Guillan-Barre diagnosed? and treated
spinal tap, on top of other neuropathie testing
- tx: IVIG + physio to maintain muscle function
What UW information whould be obtained for cases of Guillan-barre syndrome?1
- confirmation of dx
- course of the disease
- whether a full recovery has been made
- details of any residual deficit.
- whether there has been more than one distinct episode.
What is Chornic inflammatory demyelinating polyneuropathy (CIDP)
?
AKA chronic relapsing polypneuropathy.
immune-mediated disorder
- chronic counterpart of Guillain-Barree syndrome.
What are the signs and sxs of CIDP?
- symmetric proximal and distal motor and/or sensory muscle dysfunction lasting for at least 8 weeks
- diminished tendon reflexes
- alterations in proprioception.
How is CIPD treated?
glucocorticoids
IVIG
plasmaphersis
Gabapenting and tricuclic antidepressents are used for pain.
What is Mutlifocal motor neuropathy? (MMN)
Immune-mediated, demyelinating neuropathy, MMN is characterized by slowly progressive muscle weakness, fasciculations and cramping of muscles.
- sensory involvement is minimal or absent.
- rare, and males>femalres.
- age 40
How is MMN diagnosed, and treated?
Nerve condition studies, and review of Anti-GM! antibodies which are elevated in 50% of pt.
- muscle strength is improved 3-6 weeks after commencement of tx (IVIG and immunosuppresives).
What is Charcot-Marie-Tooth (CMT)?
hereditary autosomal dominant disorder,
- muscle weakness, muscle wasting and sensory loss in the first two decades of life.
- resesive forms usually develop sxs of leg weekness in childhood.
There are 7 types of CMT with CMT1 and CMT2 being the most common. Define these two
CMT1- caused by mutation of PMP22 gene. myelin is unstable and spontaneously breask-down causing demyelination
CMT2- primarily an axonal disorder rather than a demyelinating disorder. Type 2 results in peripheral neuropathy though direct axonal deta.
How is CMT treated?
limited, daily stretchng helps, orthopedic surgery is ofente necessary
its a slowly progressive disorder that can lead to disability lter in life.
Dementia, can occur in those aged 18-65. Its called early onset dementia (EOD). What are the general causes of dementia in younger people?
- neurodegenerative demential: ALZ, dementia with lewy bodies and PKD dementia
- vascular disease: vascular dementia, CADASIL,
- Encephalopathy associated with systemic autoimmune disease
- non-metabolic disorders (mitochondrial disease, leukodystrophies)
- others: HD, or complications from ETOH abuse.
Dementia in younger people can often get mistaked for what other conditions that cause memory loss, behaviour or personally changes?
- psych disorders incl. Depression
- psychosocial stressors
- pathological cause
- severe-obstructive sleep apnea
What are common investigations/tests used in the diagnosis of EOD?
- physical and neurlogical examination
- cognitive testing
- MRI scanning, CT scans
- Laboratory tests (blood, cerebrospinal fluid analysis)
What is Huntingtons disease (HD)?
autosomal dominant condition that causes a progressive neurodegenerative disorder that causes cloreiform movement, psychiatric issues and dementia
HD is genetically confirmed, and if there is presence for the gene, then the pt will develop the disease at some point in their life. What are the first signs of the disease?
- change in usual behaviour.
- unsual jerky, fidgety movements of the upper body and limbs, and face
- unsteadiness of the hands or feet, causing falls and clumsyness
- increasing inability to sustain certain simple voluntary acts. A condition called impersistence.
How is HD treated?
sxs are aliviated via: SSRIs, antispychotics, and moodstabalizers.
Define parkinsonism
group of sxs that resemble those seen in PKD. but are secondary to another diseaser or disorder. The cause is usually primary or secondary.
What are the causes for Primary PArkinsonism?
Parkinson’s disease- sporadic and familial
What are the causes for secondary parkinsonism?
Drug induced hemiatrophy hydrocephalus hypoxia infectious metabolic toxin Trauma tumour Vascular
What are the causes for PArkinsons-Plus syndrome, which are similar to parkinsonisms but have a complex presentation that reflec the involement of multiple neurological systems.
cortical-basal ganglionic degenration
dememential syndromes (ALZ, diffuse lewy body disease)
mutiple system atrophy syndrome (Shy-drafer syndrome, motor neuron disease)
progressive supranuclear palsyt
PKD is a chronic, progressive neurodegenerative disorder characterized by what?
- tremor at rest, improves with movement
- muscle stiffness or rigidity
- bradykinesia
- postural instability (loss of balance)
also: gait disturbances, autonomic dysfunction, loss of facial expression, micrographia, dysphagia, muffled speech and viral changes.
also: cognitive dysfunction, dementia, psychosis, altered sleep, mood disorder, sensory distrubances.
What causes PKD?
deficiency of a dopamine, caused by degeneration of dopamine-producing cells in the substantia nigra (basal ganglia).
this neurotransmitter is required for motor performances and influence the initiation, planing and execution of movements.
How is PKD diagnosed?
clinical examination, confirmed by MRI, brain ultrasound and PET scan.
- Chronic and slowly progressive.
How is PKD treated?
movements/physio/exercise/stretching/
anti-depressives.
Levodopa - reduces idiopathic parkinsons’
but its AE: nausea, hypotension, confision, involuntary movements.
body becomes used to it, and its use diminishes in time.
Can use other treatments: Cardidopa, catechol-o-methyl transferase inhibitors tolcapone, dopamine agnoists, selective MAO-B inhibitor, stalevo
What surgical techniques are currently used to treat PKD?
- lesioning- selective damage to a certain cell within the areas of the brain, electrode inserted. - used for drug-resitant remor, and regidity.
- deep brain stimulation- implanting electrode into parts of brain, and attaching to IPG to stimulate electric signals by pt using magnet
3, implantation of fetal tissues- replace dying dopamine-producing cells with transplanted brain tissue from fetuses.
What should an UW consider when reviewing a hx of PKD?
1, sxs since time of dx (stable?)
- tremor
- dementia
- treatement and response
- extent of disabilioty
- quality of life
Define the term Muscular dystrophies
group of genetically-determined disorders characterized by progressive muscle weakness (myopathy) and atrophy of skeletal muscle)
What are the 3 most common genetic type of muscular dystrophies?
- X-linked - Duchenne, BEcker, Emery-Dreifuss
- Autosomal recessive - limb-girdle 2A, 2B, 2C, congential Emery-Dreifuss, distal dystrophies
- Autosomal dominant- Fascioscapulohumeral, limb-girdle 1A, 1B, 1C, myotonic type 1 and 2, Distal muscular dystrophoies
What are the characteristics of muscular dystrophies?
electric signals are transmitted by nerves with a corresponding failure of muscles to respond.
How is muscular dystrophy diagnosed?
based on type suspected then:
- genetic testing
- CPK- parallels of the degree of muscle necrosis
- electromyography
- muscle biopsy
- electrocardiogram/ECHO
Which types of Muscular dystrophies are rarely seen at UW and associated with limited life expectance?
Duchenne and Bekcer
Which Muscular dystrophies are more compatible with longer life span and seen during UW?
- Emery-Dreifuss Muscular dystrophy (EDMD)
- Facioscapulohumeral MD
- Limb Girdle MD
- Myotonic MD
5.
What are the characteristics of EDMD?
- contractures in first decade, then muscle weakness
- age 10-20 muscle weakness and wasting occurs in upper and lower limbs
- cardiomyopathy is comon,
- tx is supportive, CV sxs can be tx’ed with pacemaker
Define FSHMD
named after muscles affected: facial muscles, shoulder girdle.
- auto dominant inherrited.
- 2 kinds:
> infantile: early onset, quick progression, face muscle weaknbess, wheelchair age 10. Associated with epilepsy and mental retardation
> Classic: 10-20’s, facial muscles affected early, “scapular singing”, cardiac arrhythmia and condition defects. Retinal vasular disease possible deathness
- dx via DNA testing. muscle biopsies,
- tx’ed: physical therapy, surgery to improve function
Define Limb Girdle Muscular Dystrophy.
- 15 types exist.
- biopsy with immunofluorescence reveals a reduction in muscle proteinds.
- auto dominant is rare and less severe,
- recessive onsets starts age 6-18,
- varying degrees of
a) proxmial muscle weakness/atrophy
b) involvement of calf muscle
c) incidence of cariomyopathy
d) presence of ^ Creatine kinease. - tx aimed at increasing function and correct deformities.
Define Myotonic muscular dystrophy.
- two types (1 and 2)
- onset in teens/20’s
- sxs variable and affect organ systems.
- muscle weakness, and myotonia is often present
What does an UW need to consider when assessing a hx of MD?
- precise diagnosis
- results of investigations/specialist reports
- confirmation of cardiac involvement
- rate of progression
- current level of disability.
What is Motor Neuro Disease? (MND)
progressive degeneration of motor neurons in the spinal cord, brainstem and cortex.
Define ALS (Amyotrophic Lateral Sclerosis)
- most common neuron disease.
- degeneration of upper and lower motor neurons in the brain and spinal cord.
- sxs: weakness, spasticity, hyperreflexia, muscle atrophy and gait.
- clincal dx based on hx and physical findings supported by EEC, MNCS, and MRI.
- progress (2-5 yrs) weakness and loss of voluntary muscle function.
- Riluzole is used to slow progress of ALS and delay need for Vent support.
Define Progressive Bulbar Palsy,
Variant of ALS,
- involves slow progressive weakness and wasiting of muscle innervated by brainstem motor nuclei.
sxs: dysarthria, dysphagia, chewing difficulties, with upper extremity wekaness.
Define Primary Lateral Sclerosis
Rare, slow progressive disease of primary upper motor neurons affect by mild lower motor neuron sxs can be present.
sxs: arm and leg stiffness and spasticity, muscle weakness without atrophy, dysarthria secondary to involvemnent of facial muscles
- tx: physical and occupation therapy, Baclofen, Tizanidine and clonazepam for spasm,
* survival rate is good
Define the term Spinal Muscular Atrophy (SMA)
syndroms characterized solely by lower motor neuron signs.
- autosomal dominant hereditary disorder, some sporadic mutations.
- sxs: muscle weakness, hypotonia, hyporeflexia, and muscle atrophy * they depend on type of SMA.
- dx: Motor neuron disease testing, Cratine kinease is elevated, biopsy may be necessary
There are 4 main types of SMA. What are they?
- SMA type 1- acute, the bulbar muscles affected. breating often labored. poor prognosis
- SMA type 2- chornic SMA onset 6-18 months, most common type. developemental delay, tremour, and MSK deformaties. Can survie to childhood, but poor life.
- SMA type 3- mildest form, onset >18months. prgnosis is better, mobility is possible, assitance needed later in life
- SMA type 4- adult onset, insidious onset in 3rd decade, slow prognosis. Normal life expectancy.
What is Myasthenia gravis? (MG)>
neuromuscular transmission disorder. Muscle fatigue and weakness increases with repetitive use.
- sxs: ocular, bulbar sxs, weakness of facemuscles, limbs and resp sxs.
- 50% of pt present with ocular myasthenia
- common in F <40, males >60.
- autoimmune disease caused by production of Antibodies against AChR. MuSK antibodies used to calrify diagnosis.
- thymys gland is a factor in the pathogenesis of MG, seen as either thymoma or primary thymic carcinoma.
- dx: hx and neuro exam, serology test, ice pack test, edraphonium choride test, repetitive nerve stimulation study, MRI or CT of chest.
What is Myasthenic crisis?
involves generalized weakness, bulbar signs and resp insufficientcy. Life threatning.
- resp function is monitored by reviwing FVC
Myasthenia gravis can be effectively managed and 30-50% of cases go into remission. What are the treatment modalities?
- anti-cholinesterase agents
- immunotherapy - glucocorticoids,
- IVIG and plasmaphersis (for crisis)
- Thymectomy- (<60 yo)
What are the factors influencing the prognosis in MG?
- age of onset
- occular only vs generalized sxs
- stabaility of disease/ remission
- effectiveness of tx
- complications
- presence/absence of thymoma.
Define Spinocerebella Degenerations
group of disorders with progressive ataxia, spastic paraplegia, peripheral neuropathy, blindness, rigidity and dementia in varying combinations.
- most are herediatry
The prognosis of sporadix ataxias is depending on the udnerlying cause. What is theres no cause?
prognosis is poor, no tx is available.
Herediatry ataxias are a heterogenous group of disorders categorized by the mode of inheritence and causative gene or chromosomal locus. They have 3 common features. What are they?
- Ataxia
- Genetic abnormalities
- pathology involving the cerebellum
What is Freidreich’s Ataxia? (FRDA)
-autosomal recessive slow progressive ataxia with onset before age 25. -2/3 pt have cariomyopathy. - associated with: a) depressed tendon reflexes b) dysarthria c) presence of Babinski reflex d) loss of positional and vibration senses.
Define Spinocerebella Ataxias Types 1-29
- autosomal dominant (SCAs) with progressive cerebella ataxia with varying degrees of bulbar dysfunction, ophthalmoparesis, pyramidal and extrapyramidal signs, optic atrophy, dementia.
- no tx
- profnosis is variable,
- die from cardiomyopathy
Define Transient Global Amnesia (TGA)
- sudden, transient loss of memory.
- unknown cause
- sxs <24 hours
- possible cause from temporary interruption in blood flow to areas in brain involved in memmory.
- episodes need to be differentiated from TIAS, complex migraines, venous congestion and psych disorders.
- ^ risk in people >50 yo with hx of complex migraines.
- prognosis depends on cause.
- tx not required, and unlikely to recur.
