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94 Cards in this Set

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virus that attacks anterior horn cells
-motor neuron only
still have sensation
polio
when most of group is vacinated, the liklihood of the unvacinated to get the disease is unlikely
-only refers to communicable diseases
herd immunity
autoimmune disorder
antibodies against acetylcholine receptors
prevalence: 5-10/100,000 population
two broad categories:
younger: classic
older: thymoma, more common
tumor originating from thymus
Myasthenia Gravis
associated with weakness and fatiguability
-DTRs intact
-No sensory loss
Myasthenia Gravis
tests:
ACH receptor antibody titer specific
electrophysiologic testing confirmatory
Tensilon test
Myasthenia Gravis
What is myasthenia gravis treated with
pyridostigmine (Mestinon)
neostigmine
corticosteroids
azathioprine(immunosuppresive)
thymectomy - remove thymus
thymectomy is curative
Myasthenia Gravis
disorder of the NM junction
caused by exotoxin of Clostridium
botulism
can cause problems with breathing and diplopia
treated with antitoxin
botulism
It acts by blocking nerve function and leads to respiratory and musculoskeletal paralysis
botulism
Blurring of vision (with unreactive dilated pupils) is characteristic, and there may be dryness of the mouth, constipation (paralytic ileus), and postural hypotension. Sensation is preserved, and the tendon reflexes are not affected unless the involved muscles are very weak.
botulism
The clinical manifestations are caused when toxin blocks inhibitory nerve impulses, by preventing the release of inhibitory neurotransmitters GABA and glycine. The result is disinhibition, or increased relative excitation. This leads to unopposed muscle contraction and spasm. Seizures may occur, and the autonomic nervous system may also be affected
tetanus
refers to sustained muscle contraction that is NOT caused by tetanus
tetany
treatment includes immune globulines
tetany
a medication widely used in emergency medicine and anesthesia to induce muscle relaxation, usually to make endotracheal intubation possible. It imitates the action of acetylcholine at the neuromuscular junction, acting on muscle type nicotinic receptors, but it is not degraded by acetylcholinesterase but by pseudocholinesterase, a plasma cholinesterase. This hydrolysis by pseudocholinesterase is much slower than that of acetylcholine by acetylcholinesterase.
Suxamethonium chloride (also known as succinylcholine, scoline, or colloquially as sux
an inherited blood plasma enzyme abnormality. People who have this abnormality may be sensitive to certain anesthetic drugs, including the muscle relaxants succinylcholine and mivacurium as well as other ester local anesthetics.
The patient experiences prolonged paralysis of the respiratory muscles, requiring an extended period of time during which the patient must be mechanically ventilated. Eventually the muscle-paralyzing effects of these drugs will wear off
pseudocholinesterase deficiency
disorders of growth
difficult to differentiate them
poor prognosis and few therapies
refer them out
muscular dystrophy
inherited
usually present in childhood
presents as progressive atrophy and weakness
most survive but Duchenne’s type is fatal in 2nd-3rd decades
genetic counseling
muscular dystrophy
refers to a group of genetic, hereditary muscle diseases that cause progressive muscle weakness.
characterized by progressive skeletal muscle weakness, defects in muscle proteins, and the death of muscle cells and tissue.
muscular dystrophy
current: any age but peak is in 50 and 60s
women twice as often as men
usually young people, but any age can get it?
confirm with muscle biopsy
MRI guided to find "hot spots"
can have false negative
associated with AI disorders
muscle inflammation
muscle weakness, tenderness, achy
Dx: muscle biopsy
Rx: corticosteroids and immunosuppressives
polymyositis
a type of inflammatory myopathy, related to dermatomyositis and inclusion body myositis. means 'many muscle inflammation'.
polymyositis
tends to become evident in adulthood, presenting with bilateral proximal muscle weakness, often noted in the upper legs due to early fatigue while walking. Sometimes the weakness presents itself by the person being unable to rise from a seated position without help, or inability to raise their arms above their head. The weakness is generally progressive, accompanied by lymphocytic inflammation (mainly cytotoxic T8 lymphocytes). The cause is unknown, but seems to be related to autoimmune factors, genetics, and perhaps viruses.
polymyositis
it is polymyositis with associated rash
dusky red, malar distrubution like SLE
underlying malignancy
20% association with internal malignancies
no cause and effect, but there may be association
dermatomyositis
is connective-tissue disease that is characterized by inflammation of the muscles and the skin. Its cause is unknown, but it may result from either a viral infection or an autoimmune reaction. Up to 50% of the cases may be a paraneoplastic phenomenon, indicating the presence of cancer.
X-ray findings include dystrophic calcifications in the muscles.
Tx: corticosteroids
dermatomyositis
Act and behave normal
but can go into total body paralysis within short period of time
may be due to disruptions in electrolytes
hypo or hyperkalemic
can have paralysis of respiratory
association with hyperthyroidism
periodic paralysis
loss of speech, abnormality of language
difficulty with spoken word, written word
generalized language problem
aphasia
same as aphasia but used elsewhere
-may actually be a better description
dysphasia
dysfunction of speech, articulation
-not a language problem
-not a process of dementia
dysarthria
Loss of ability to do something practical that they used to know how to do
apraxia
loss of ability to read when a person used to be able to read
alexia
associated with loss of neurons, plaques, tangles
Alzheimer's
these patients are not sedate
Alzheimer's
#2 cause of dementia
vascular dementia:
multi-infarct dementia
decline is "step-like" loss of function vs progressive even decline
multi-infarct dementia
#3 cause of dementia
mixed vascular dementia and Alzheimer's
is the name given by doctors to a type of depression which appears superficially as a problem with memory and which is relatively common in older people and may be confused with the presence of dementia.
The term applied to illnesses where memory and other higher mental functions are affected and includes illnesses such as Alzheimer's disease.
pseudo-dementia
patients' memory appears to be affected, but closer examination will reveal that they are inattentive to their surroundings and hence unable to retain new information in the form of new memories. Also they are able to precisely date the onset of this impairment and are acutely aware that memory is insufficient. They have slowness and apathy. However, other signs and symptoms of depression are usually present. The significance is that depression in this age group can generally be successfully treated if recognized.
pseudo-dementia
what is associated with "something they hate" and "something they cannot escape"
common factors in depression
drug induced, any combination of tremor, rigidity, bradykinesia, progressive postural instability
Parkinsonism
idiopathic parkinonism =
Parkinson's disease
is any disease or malfunction of the autonomic nervous system. This includes postural orthostatic tachycardia syndrome (POTS), neurocardiogenic syncope, mitral valve prolapse dysautonomia, pure autonomic failure, multiple system atrophy (Shy-Drager syndrome), autonomic instability and a number of lesser-known disorders.
dysautonomia
dysautonomia may be seen in this:
Parkinson's
a combination of Parksinson's and dysautonomia
Shy-Drager Syndrome
"multiple system atrophy"
Shy-Drager syndrome
is a rare, progressively degenerative disease of the autonomic nervous system.
Shy-Drager syndrome
will be passed to next generation, only one gene is required for it to be passed
autosomal dominant
carrier: will not be passed to next generation
autosomal recessive
Huntington's Chorea:
autosomal dominant or autosomal recessive
autosomal dominant
symptoms are abnormal body movements called chorea and a lack of coordination, but it also affects a number of mental abilities and some aspects of personality
Huntington's Chorea
These physical symptoms occur in a large range of ages, with a mean occurrence in a person's late forties/early fifties. As there is currently no proven cure, symptoms are managed with various medications and care methods.
Huntington's Chorea
irreversibly, progressively leads to dementia and institutionalization and death
Huntington's Chorea
Fridreich's Ataxia:
autosomal dominant or autosomal recessive
autosomal recessive
an inherited disease that causes progressive damage to the nervous system resulting in symptoms ranging from gait disturbance and speech problems to heart disease.
Bilateral: feet curling
Friedreich's Ataxia
uncontrolled/involuntary movements that are "snake-like", typically in the hands and feet
athetosis
extra movements that are jerky
characterized by brief, irregular contractions that are not repetitive or rhythmic, but appear to flow from one muscle to the next.
Chorea
jerky movements during intended movements
ataxia
combination of uncontrolled/involuntary movements that are "snake-like", typically in the hands and feet and characterized by brief, irregular contractions that are not repetitive or rhythmic, but appear to flow from one muscle to the next.
chora athetosis
demylination of white matter nerve tracts nerve conduction is slowed down
CNS disease of white matter
causes patches
Multiple sclerosis
prognosis:
better for younger ages
better for females
idiopathic
can present in odd ways with peculiar neurologic losses or complaints
manifests very differently in different patients
common: dizzyness, vertigo, MLF, diplopia, weakness
multiple sclerosis
Hallmark: sx come on and then they go away
multiple sclerosis
What are the two criteria required in multiple sclerosis to make a diagnosis:
1)patterns of neurologic deficit have to be traced to at least two locations
2) episodes must be seperated in time by at least a month
What test results may be seen in patients with multiple sclerosis
CSF has changes
gamma globulins
MRI will show areas of demylination as bright spots
electrical potential evoked by stimuli
can be auditory, pain, visual
used in newborn screening
with MS: subacute optic neuritis
evoked potential
what may be used in the treatment of mulitple sclerosis
steroids
interferons
What are two diseases that may mimic multiple sclerosis:
Acute disseminating encephalitis (ADE) and transverse myelitis
this is often confused with MS and is associated with multiple spots and one episode
acute disseminating encephalitis
can mimick MS and is occurs when one spot in the cord gets demylinated
transverse myelitis
sudden change in behavior traced to corical electric discharge and may be sensory or motor
seizure
requires a history of at least two seizures
epilepsy
symptomatic epilepsy is an odd term that actually means:
secondary epilepsy
if a seizure is not secondary to anything else, if it is primary, it is called:
ideopathic epilepsy
EEG: shows activity all over and stop together
generalized
the two types of partial seizures:
simple and complex
what feature is unique to partial seizures that are not found in generalized seizures
aura
what is the distinguishing difference between simple and complex partial seizures
whether or not the consciousness is impaired
what are these considered:
1 Simple partial seizures evolving to generalized seizures
2 Complex partial seizures evolving to generalized seizures
3 Simple partial seizures evolving to complex partial seizures evolving to generalized seizures
partial seizures evolving to secondarily generalized seizures
a brief, involuntary twitching of a muscle or a group of muscles.
like suddenly jerky movement when one is falling asleep in class
ex: hiccups
myoclunus
total sudden loss of muscle tone
usually occurs in children
atonic
usually begins with cry/groan/noise and then go into a stretching posture and begin rhythmic, symmetrical contractions and extension movements
tonic clonic/grand mal
the altered state of consciousness that a person enters after experiencing an epileptic seizure
postictal state
what are these characteristic of:
loss of consciousness
cortical activity
postictal state
loss of bladder and bowel control
tonic clonic
the person may appear to be staring into space with or without jerking or twitching movements of the eye muscles. These periods last for seconds, or even tens of seconds. Those experiencing these seizures sometimes move from one location to another without any purpose
absence seizures
previously called petit mal seizures
absence seizures
this generalized seizure has no postictal state
absence seizure
may occur during seizure: look purposeful, but they are not conscious
ex: chewing gum
automatism
absence, myoclonic, clonic, tonic-clonic, and atonic are all what kinds of seizures:
generalized
these seizures are myoclonus that are regularly repeating at a rate typically of 2-3 per second.
clonic
involve an initial contraction of the muscles (tonic phase) which may involve tongue biting, urinary incontinence and the absence of breathing. This is followed by rhythmic muscle contractions (clonic phase). This type of seizure is usually what is referred to when the term 'epileptic fit' is used colloquially.
tonic-clonic seizures
a manifestation of thiamine deficiency, or beri-beri. This is usually secondary to alcohol abuse.
Wernicke-Korsakoff syndrome
two common drugs used for seizures:
Valium (diazepam)
Ativan (Lorazepam)
a persistent neurologic deficit after a seizure suggests a:
focal point
also known as a fever fit, it is a generalized convulsion caused by elevated body temperature. They most commonly occur in children below the age of three and should not be diagnosed in children under the age of 6 months or over the age of 6 years. In many cases, the first sign of fever is the onset of the seizure. It has been theorized that the seizure is triggered by the rapidity of the rise in temperature, rather than the actual temperature reached.
febrile seizure
The diagnosis is one that must be arrived at by eliminating more serious causes of seizure: in particular, meningitis and encephalitis must be ruled out. Therefore a doctor's opinion should be sought and in many cases the child would be admitted to hospital overnight for observation and/or tests. As a general rule, if the child returns to a normal state of health soon after the seizure, a nervous system infection is unlikely.
febrile seizure
t or f
hypoxia in brain can cause a brief seizure like activity and/or syncope
T