CNS DI-Exam I Flashcards
What type of a disease is cerebral palsy
congenital
a group of congenital abnormalities that occur either in utero, during birthing, or during the first two years of life. These abnormalities are non-progressing in nature and can have multiple etiologies. Motor abnormalities are the most prominent presentation of this disease.
Cerebral Palsy
this disease is also called “brain paralysis” and “static encephalopathy”
Cerebral Palsy
a defining feature of this disease is that the case occurs prior to complete development of the cerebrum
Cerebral Palsy
Risk factors of this disease include preterm birth and maternal or fetal infections.
Cerebral Palsy
Pathophysiology of this depends on the time of onset. Typically, hypoxic-ischemic injury.
Cerebral Palsy
symptoms of this disease typically involve motor disorders, but may include sensory and cognitive defects as well. Clinical signs may be absent at birth and develop slowly as an infant. Milestone delays are the initial clinical symptoms. Major motor abnormalities include: spasticity, dyskinesis, rigidity. 50% of cases experience seizures
Cerebral Palsy
Diagnostic testing for Cerebral Palsy includes:
history and physical to rule out progressive disorders, US of fetus during pregnancy, MRI scan is most useful,
Treatment of Cerebral Palsy includes…
goal is to maximize function, so most treatment is symptomatic. Physical Therapy is very important. Medications include: Botulinum toxin, antispasmatics, anticonvulsants
Complications of Cerebral Palsy include…
hip dislocation due to muscular tone imbalance, difficulty eating and breathing, osteopenia, osteoporosis, chronic MSK pain
Chiari Malformation is what type of disease?
congenital
a dysraphism of the metencephalon.
Dandy-Walker Syndrome
What is a defect in the formation of the midline in an embryo?
Dysraphism
This disease features an enlarged posterior cranial fossa, loss of the cerebellar vermis with an enlarged midline fluid-filled, ependymal-lined cyst representing an expanded fourth ventricle
Dandy-Walker Syndrome
In this disease, on CT you will see a glossy, enlarged fourth ventricle due to the redimentary development of the cerebellum and lack of fusion between the cerebellar hemispheres.
Dandy-Walker Syndrome
A hallmark of this disease is a rudimentary cerebellum and an enlarged cistern in the posterior cranial fossa
Dandy-Walker Syndrome
This is due to a failure of closure or a reopening of the previously closed caudal portions of the neural tube
Spina Bifida
a midline defect in the vertebral posterior arch structures. There is no involvement of neural components
Spina Bifida Occulta
a midline defect of the vertebral posterior arch structures with herniation of the meninges but not the neural structures
Meningocele
a midline defect of the vertebral posterior arch structures with herniation of the meninges and neural structures
myelomeningocele
Prevention mechanisms of spina bifida include
folic acid supplementation during pregnancy
This form of spina bifida is generally asymptomatic and presents as minor changes in skin present over the diaphragm
spina bifida occulta
this form of spina bifida is marked by a fluid-filled, cyst-like sac which extends off the back over the region of the dysraphism
meningocele
this form of spina bifida is marked by numerous organ dysfunctions, cyst-like structures covered by a thin layer of epithelialized material
myelomeningocele
a rare neurocutaneous disorder (also called phakomatoses)
Sturge-Weber Syndrome
Encephalotrigeminal angiomatosis is another term for what disorder
Srurge-Weber Syndrome
Presentation of this disorder includes facial nevus about the eye extending in the opthalmic division of the trigeminal nerve. Glaucoma may also be present in the ipsilateral eye. Patient may also present with developmental delay and learning problems; seizures develop within the first year.
Srurge-Weber Syndrome
This is caused when the anterior spinal artery infarcts
Spinal Artery Infarction
The presentation of this includes acute onset, back pain, pain radiating into the legs, focal neurologic changes including: weakness below the level of the lesion, incontinence x 2, analgesia below the lesion level, spared vibratory and proprioception senses
Spinal Artery Infarction
Which artery runs midline along the spinal cord and is supplied by 5 or 6 medullary arteries that are derived from spinal arteries
Anterior Spinal Artery
This type of brain injury is almost always non-traumatic in origin, and results in blood entering and contaminating of the CSF
Subarachnoid hemorrhage
Patients with this report having “the worst headache of their life”; this is usually due to meningeal irritation
Subarachnoid Hemorrhage
Treatment for a subarachnoid hemorrhage may include…
surgical repair of aneurysm, drainage and shunt placement, clot removal and triple H therapy (hemodilution, hypertension, and hypervolemia)
This disease is caused by a dysraphism in which defects in the closure of the midline structures related to the metencephalon cause a range of neurological abnormalities involving the posterior cranial fossa
Chiari Malformation
The main abnormality in this disorder involves displacement of the tonsils of the cerebellum, along the medulla into the foramen magnum
Chiari Malformation
The two types of Chiari Malformations are…
Chiari type I and Chiari type II
Chiari type I can also be called
Chronic tonsilar herniation
This disease is characterized by low-lying cerebellar tonsils (NOT the vermis) extending through the foramen magnum. This can cause obstruction of the CSF. It is strongly associated with syringomyelia
Chiari Type I
This disease is characterized by bony and dural abnoralities, a small posterior cranial fossa, abnormal midline cerebellum shape with displacement of the cerebellar vermis (NOT the tonsils). The vermis extends down into the foramen magnum
Chiari Type II
Presentation of this disease is typically asymptomatic in the infant and child with signs and symptoms developing at puberty. The pt. will experience headache, neck pain, urinary frequency, progressive cerebellar ataxia and lower extremity spasticity. Anemic episodes may occur in infants. Older children can present with scoliosis
Chiari Type I
A 14-month-old male is brought to the pediatrician because he is not able to walk. His mother is very concerned because her friend’s daughter began walking around 12 months of age. He was born at 30 weeks gestation to his 28-year-old G1P1 mother. The pregnancy was complicated by eclampsia requiring emergency Cesarean delivery. The mother also reports her son has difficulty with swallowing. Physical exam is notable for hypertonic muscle tone, absence of selective motor control, and motor delay.
Cerebral Palsy
This form of Chiari is almost always associated with pediatric hydrocephalus and myelomeningocele
Chiari type II (Note: chiari type I may cause adult onset hydrocephalus but hydrocephalus is generally uncommon in this form of the disease)
This form of Chiari is strongly associated with syringomyelia, in which patients also have an idiopathic syrinx
Chiari type I
An infant boy presents with trouble swallowing, bilateral arm and leg weakness, and stridor. The infant was born to a 28-year-old woman who did not seek prenatal care. On physical exam, the infant barely moves his arms and legs and has stridor. A meningomyelocele is appreciated in the lumbosacral area. Magnetic resonance imaging of the head shows downward displacement of the cerebellar vermis and tonsils through the foramen magnum.
Chiari Type II malformation
An 1-year-old boy is brought to the emergency department by his parents due to increased irritability and an “enlarging head.” The parents deny any trauma but has noticed their infant appears weaker than his sister when she was his age. They did not receive adequate prenatal care due to the distance and cost to arrive to their closest obstetrician. On physical exam, the infant is inconsolable and has an increased head circumference for his age. Magnetic resonance imaging (MRI) of the head shows hypoplasia of the cerebellar vermis with cystic dilatation of the fourth ventricle leading to a non-communicating hydrocephalus.
Dandy-Walker Syndrome
an abundance of fluid in the ventricular/subarachnoid compartments of the cranium is known as….
hydrocephalus
hydrocephalus can be caused by two general mechanisms:
the overproduction of CSF or impaired drainage of CSF
obstruction of the cerebral aqueduct, secondary to masses or viral infection is one etiology of hydrocephalus. This is called
aqueductal stenosis
in an infant, abnormal enlargement of the head or accelerated rate of head enlargement is likely due to …
hydrocephalus
an older child (whose cranium has already closed) with hydrocephalus may present with
irritability, lethargy, poor appetite, vomiting, headache
a young child (whose cranium is still not closed) with hydrocephaly may present with symptoms that include…
brisk tendon reflexes, spasticity, clonus
Sturge-Weber Syndrome is what type of disorder?
a rare neurocutaneous disorder (aka-phakomatose)
Another term for Sturge-Weber Syndrome is…
encephalotrigeminal angiomatosis
A 5-year-old male presents with an unprovoked convulsive episode that lead to right-sided hemiparesis. On physical exam, a port wine stain is appreciated, affecting the ophthalmic and maxillary division of the trigeminal nerve on the left side of the face. Head CT shows brain calcifications.
Sturge-Weber Syndrome
A newborn boy is born to a young mother via a normal spontaneous vaginal delivery. She had not had any prenatal care during her pregnancy. On physical exam, the newborn has a large port-wine stain on the left forehead and eyelid. He is also noted to have some shaking episodes. Concerned, the physician obtains an MRI of the head, which shows a leptomeningeal angioma.
Sturge-Weber Syndrome
A patient with this disorder will present with facial nevus around the eye (and primarily over the V1/V2 area), developmental delay and learning problems, seizures within the first year, glaucoma may be present in ipsilateral eye
Sturge-Weber Syndrome
What is another term for facial nevus?
port-wine stain
This disorder is derived from: A vascular abnormality that develops as the primordial vascular supply to the head, brain and face. The abnormality ultimately causes the leptomeninges (arachnoid and pia) to be over vascularized and the brain to be undervasculated . This causes the brain to calcify and atrophy.
Sturge-Weber Syndrome
A degenerative process of the cerebral cortex resulting in senile dementia is most likely
Alzheimer’s Disease
When does Alzheimer’s Disease usually present?
7th-9th decades of life
Diffuse atrophy of the cerebrocortex with ventriculomegaly, sulcal enlargement, hippocampal atrophy, neuritic plaques, neurofibrillary tangles are common signs of what disease?
Alzheimers disease
Neurofibrillary tangles seen in Alzheimer’s disease contain…
hyperphosphorylated tau proteins
neuritic plaques (senile plaques) seen in Alzheimers disease contain…
A-beta amyloid
A 78-year-old man presents to his primary care clinic and is accompanied by his wife. He feels well, but his wife is concerned that he has become more confused over the past few weeks. He enjoys socializing with family and friends, but sometimes has difficulty finding words and recently forgot his niece’s name. He walks around his neighborhood for exercise, but has the tendency to wander and get lost. He has a history of hypertension and atrial fibrillation. Current medications are warfarin and dronedarone. Physical exam is unremarkable. Recent lab results were within normal limits.
Alzheimers Disease
Common genes involved in Alzheimers Disease are…
Presenilin genes (located on chromosomes 1 and 14)
A 61-year-old male presents with left-sided hand weakness and trouble with walking. He is not sure why these symptoms occur. On physical exam, tongue fasciculations are appreciated. He has slow speech. The left upper extremity shows forearm atrophy and depressed reflexes. The right lower extremity is hypertonic, with 3+ reflexes, and positive Babinski sign.
Amyotrophic Lateral Sclerosis
Lou Gherig’s Disease is another term for…
Amyotrophic Lateral Sclerosis
If astrocytes fail to remove glutamate from the synaptic cleft, a patient will experience continuous motor neuron activity leading to excitotoxicity, oxidative stress, failure of superoxide dismutase enzymes and cell death. This is the pathogenesis of what disease?
Amyotrophic Lateral Sclerosis
The hallmark of this disease is the death of peripheral motor neurons from the ventral horn and brainstem nuclei as well as the bulbospinal and corticospinal neruons in the brainstem and cerebral cortex. (So…neurons in BOTH the peripheral and central motor systems are effected)
Amyotrophic Lateral Sclerosis
Which branches of the nervous system remain intact in a patient with ALS?
peripheral sensory system, central sensory system, autonomic nervous system
Cause of death in an ALS patient is usually due to…
respiratory failure
Presentation of this disease is initially asymmetric involving one hand and then progresses to symmetric involvement. The pt. will experience cramping of muscles, usually early in the morning.
ALS
Flaccid weakness, atrophy and fasciculations will be present in an ALS patient due to what type of neuron involvement?
Spinal motor neurons
In ALS, spastic weakness is due to involvement of what type of neurons?
Corticospinal motor neurons
A patient with ALS will present with dysphagia, dysarthria and dyspnea due to what type of neuron involvement?
Bulbar motor neurons
This disease is the classic form of progressive, hereditary spinocerebellar ataxia. It involves significant loss of myelinated fibers in the posterior columns, spinocerebellar tracts and the corticospinal tracts of the spinal cord.
Friedreich Ataxia
What is the genetic inheritance pattern of Friedreich Ataxia?
autosomal recessive mutation
This disease is caused by a mutation in the gene named frataxin, which contains expanded GAA triplet repeats
Friedreich Ataxia
A 23 year old male with a past medical history of diabetes mellitus presents with muscle weakness, staggering gait with frequent falling, dysarthria and a curvature in his upper spine. On physical exam the patient is noted to have marked muscle weakness (2/5 in upper and lower extremitites), a loss of deep tendon reflexes, nystagmus as well as pes cavus and kyphoscoliosis. Patient had a recent ED visit for pneumonia and a bedside ultrasound demonstrated a hypertrophic cardiomyopathy.
Friedreich Ataxia
An 8-year-old boy is brought to the pediatrician because his mother is concerned about recent behavioral changes. His mother states that she has started to notice that he is slurring his speech and seems to be falling more than normal. On exam, the pediatrician observes the boy has pes cavus, hammer toes, and kyposcoliosis. Based on these findings, the pediatrician is concerned the child has a trinucleotide repeat disease. Which of the following trinucleotide repeats is this child most likely to possess?
GAA
This disease is caused by progressive myelin and axon loss from spiocerebellar tracts–>posterior columns –> corticospinal tracts.
Fibrous gliosis is present in these tracks.
There is also loss of Purkinje cells in the cerebellar cortex
Friedreich Ataxia
The initial complaint in this disease is typically difficulty walking. Patient will eventually present with ataxic signs in bilateral legs, wide spread unstable stance and foot slapping.
Within a few months, arms ataxia presents. Speech slows and becomes softer and uncoordinated respiratory muscles cause chocking.
Tendon reflexes will be abolished; plantar reflexes are extensor with flexor spasm. Pes cavus, hammer toes and kyphoscoliosis develops and 1/2 of cases include cardiomegaly
Friedreich Ataxia
This general group of disorders presents with: progressive and symmetric limb ataxia, impaired gait and equilibrium, slow voluntary movements, some sensory impairments, scanning speech and dysarthria, nystagmoid eye movements, tremor of the neck and head
spinocerebellar ataxias
This group of disorders are progressive, degenerative hereditary disorders that may be dominant or recessive. Degeneration is usually seen in the cerebellum, but may include the brainstem and peripheral nerves. There are about 60 forms.
Spinocerebellar ataxias
a cavity that opens in the spinal cord is known as a…
syrinx
This disorder presents as an insidious onset, slowly progressing lesion. It may present as scoliosis. Head, neck or back pain is common and there will be a cape like distribution of analgesia. Flaccid weakness at lesion level with spastic weakness below lesion level. Incontinence may be involved.
Syringomyelia
A syrinx is associated with which form of Chiari Malformation?
Chiari Type I
a syrinx that is a dilation of the central canal of the spinal cord that fills with CSF is termed…
hydromyelia
A syrinx that opens in the white matter of the spinal cord and fills with extracellular fluids is termed…
a true syrinx
an abscess or suppurative infection located between the dural layer and the inner table of the skull is what type of abscess?
Epidural Abscess
This disease presents with a triad of symptoms: midline dorsal pain, fever, progressive limb weakness
Epidural Abscess
The most common bacterium that causes an epidural abscess is…
Staphylococcus aureus
a non-inflammatory demyelination of the pons secondary to the rapid correction of a hyponatremic state or production of a hypernatremic state
Central pontine myelinolysis
Another term for Osmotic Demyelination Syndrome is…
Central Pontine Myelinolysis
This disease is largely associated with severe alcoholism. Other associated diseases include renal dialysis and hepatic failure
Central Pontine Myelinolysis
This disease is caused by rapid correction of hyponatremia to normal or hypernatremic levels.
Central Pontine Myelinolysis
This disease involves lesions that cause severe demyelination beginning in the center of the pons. There will be reactive phagocytes and glial cells present but NO signs of inflammation. Lesions may also appear in the thalamus.
Central Pontine Myelinolysis
A patient with this disease will present acutely-subacutely. They will have flaccid paralysis in all four limbs, inability to chew, swallow or speak. Eye movements and facial expressions can be intact. Spasticity will develop and locked in syndrome can present.
Central Pontine Myelinolysis
A 70-year-old hospitalized patient with a serum sodium of 117 mEq/L is corrected to 140 mEq/L in the next 6 hours. In the subsequent days he becomes paralyzed.
Central Pontine Myelinolysis
a medical condition, that damages part of the brainstem, in which the body and most of the facial muscles are paralyzed but consciousness remains and the ability to perform certain eye movements is preserved.
locked-in syndrome
degeneration of the posterior and lateral columns of the spinal cord secondary to a deficiency in vitamin B12. This is a treatable myelopathy
Subacute combined degeneration
vitamin B12 is also called…
cobalamin
Treatment for subacute combined degeneration includes…
prompt B12 supplements.
Note: changes may be reversible but once complete paraplegia has developed, recovery is poor
A 72-year-old man of European ancestry presents for a routine check-up with increased fatigue, a smooth shiney tongue and yellowing of his skin. The man is a strict vegan and refuses to eat anything that casts a shadow. On physical exam you note markedly decreased vibration sensation and proprioception. A peripheral smear is ordered demonstrating large RBC’s as well neutrophils with nuclei that have 7 lobes. This seems to be the start of…
Subacute combined degeneration
Subacute combined degeneration is also known as
Vitamin B12 deficiency
A patient with this disease will present with subacute and progressive development of ataxia, numbness and tingling in the lower extremities, often progressing to weakness and spasticity and increased reflexes. Other symptoms include: pernicious anemia, spinal cord myelopathy, diffuse and symmetric presentation, paresthesias in hands and feet, loss of vibratory sense and proprioception, peripheral neuropathy
Subacute combined degeneration
symptoms of subacute combined degeneration present as:
diffuse and symmetrically
Failure to transfer even very small amounts of B12 across the intestinal wall is the root cause of…
Subacute combined degeneration
A deficiency in vitamin B12 can lead to sponngy vacuolation, intramyelininc and interstitial edema of myelin sheaths and their eventual destruction and astrocytosis. This initially effects the heavily myelinated posterior and lateral columns of the spinal cord. This describes the development of what disease?
Subacute combined degeneration
What are the first spinal cord columns targeted inSubacute combined degeneration?
posterior and lateral columns
Over the course of Subacute combined degeneration which tracts become demyelinated?
large ascending sensory tracts and descending motor tracts
a malignant invasive embryonic tumor of the cerebellum most often seen in children and young adults
medulloblastoma
A 5-year-old child presents with an occipital headache, an ataxic gait, nystagmus, and papilledema
medulloblastoma
this type of tumor occurs mainly in children and is exclussively in the cerebellum, usually near midline (more lateral if in adults). It is highly malignant.
medulloblastoma
Common clinical symptoms for this disease are short in durration (only about 3 months). They present as headaches (due to ICP), persistent vomitting (due to ICP), truncal ataxia and spasticity, papilledema (due to ICP), nystagmus
medulloblastoma
less common symptoms in a patient with medulloblastoma include:
limb ataxia and dysiadokokinesia
tumors arising from ependymal cells lining the ventricular system of the brain; slow growth; well-differentiated
ependymoma
the most common tumor of the fourth ventricle
ependymoma
What are the two most common locations to have an ependymoma?
- fourth ventricle
- supratentorally in the lateral ventricles
(note: it can be anywhere in the ventricular system)
A patient under the age of three with this type of tumor will present with vomiting, ataxxia, headache, lethargy, increased head circumfrence, irritability
ependymoma
An ependymoma usually presents in what stage of life?
0-20 years old
older children with this type of tumor present with ataxia, nystagmus, gaze palsy, hemiparesis, neck pain
ependymoma
A 5-year-old boy presents to pediatrics clinic complaining of headaches. His head circumference is large for his age. He has no other complaints and had been growing and developing normally prior to this visit. Vital signs are stable. Physical examination is benign with the exception of increased head circumference.
ependymoma
slow growing, benign tumors present on the meningeal layers surrounding the brain
meningioma
a meningioma is most common in which age group and gender?
60s-70s; more common in females
what type of tumor is commonly found in convexities of the cranium, along the falx or at the base of the skull
meningioma
What is the recurrence rate of an ependymoma?
HIGH! 44%
what type of cells is a meningioma most likely derived from?
meningothelial (arachnoid) cells
This type of tumor is usually a solitary lobulated tumor attached to the meninges. It will likely expand and repace CNS tissue without invading it. It may involve skull tissue.
meningioma
presentation of this tumor includes: possibly asymptomatic, focal neurologic defects due to compression of structures, seizures, hemiparesis, gait disturbance
meningioma
the seeding of a distal neoplastic event into the central nervous system is known as
metastatic disease
The most common cause of neoplasm in the CNS is due to….
metastatic disease
Most metastatic tumors are extradural and compress the cord. What vessels do these tumors form in?
Venous Plexus of Batson
What portion of the spin is he most common site for metastasis?
thoracic spine
Metastatic diseases of the spinal cord are classified by location. What are the possible locations?
extradural, intradural extramedullary, intradural intramedullary
pain that persists for an arbitrary time that is longer than normal expected time for healing. This is pain without a peripheral generating source and involves plastic changes in the CNS or PNS
chronic pain
This disease state is characterized by pain of indeterminate duration, refractory to treatment, disrupts sleep and normal living
chronic pain
disruption in the normal nociceptive pathways of peripheral nerves, spinal cord, brainstem or cerebrum is one mechanism that my cause this disease state…
chronic pain
plasticity in normal pathways involving sprouting of axons and loss of inhibitory interneurons is on mechanism that may cause this disease state…
chronic pain
A 28-year-old snow skier strikes a tree and was not wearing a helmet. He loses consciousness for several minutes but later regains consciousness and reports feeling fine. Several hours later his neurological state decompensates acutely. CT showed a lens-shaped, biconvex hyperdense area contained by suture lines
epidural hemorrhage
This type of bleeding event is usually from a traumatic injury (usually the lateral head). This causes bleeding into the potential space between the dural layer and bone.
epidural hemorrhage
which vessel is most commonly causing an epidural hemorrhage?
meningeal artery
In an epidural hemorrhage, between what two layers does blood accumulate?
outer dural later and inner cortical bone
A patient with this type of injury will briefly lose consciousness, then experience a lucid interval. Then there will be a worsening headache, vomiting and change in mental status. Many focal neurologic signs will occur and progress rapidly to bilateral posturing.
epidural hemorrhage
A 69-year-old male with atrial fibrillation on warfarin had a syncopal episode and struck his head on the stairs. Shortly thereafter, he because unconsious.
chronic subdural hemorrhage
an accumulation of extravasated blood located between the inner layer of dura and the subarachnoid membrane.
subdural hemorrhage (may be acute or chronic)
A major pharmaceutical risk factor for an subdural hematoma is…
anticoagulants
patients with this type of hemorrhage will present with impaired consciousness, HA, focal neurologic defects, aphasia, hemiparesis, bradycardia and hypertension, unilateral pupillary dilation and oculomotor palsy
acute subdural hemorrhage
a collection of clotted blood and fibrotic material located between the inner layer of dura and the subarachnoid membrane
chronic subdural hemorrhage
This disease state is slowly progressing and may present days to weeks after minor head trauma. The patient will appear confused and have impaired consciousness, focal neurological deficits, dysphasia, unilateral papillary dilation and oculomotor paresis
chronic subdural hemorrhage
what vessels are likely involved in a subdural hemorrhage?
bridging cortical veins
On CT, a subdural hemorrhage will appear like…
crescent shaped, concave hyperdensity that can extend across suture line
this type of subdural hemorrhage is often associated with additional signs of cerebral trauma
acute
common surgical treatment for hydrocephalus includes…
shunt placement
presentation of this disease state includes increased ICP, unsteady gait, bladder incontinence, confusion
hydrocephalus
a common cause of obstructive hydrocephalus
tumor
a common cause of communicating hydrocephalus
meningeal inflammation due to tumor cells or hemorrhage
hydrocephalus ex vacuo is commonly caused by
brain atrophy
this type of hydrocephalus is due to decreased/impaired reabsorption of CSF. This occurs without CSF-flow obstruction between the ventricles and subarachnoid space
communicating hydrocephalus
this type of hydrocephalus is due to obstructed flow of CSF. This may be due to stenosis at a narrow point of the ventricular system
noncommunicating hydrocephalus
this type of hydrocephalus results from an increase in CSF to compensate for a decrease in brain size.
hydrocephalus ex vacuo
loss of perfusion or oxygenated blood to part or all of the nervous system is the end stage of what disease state?
cerebrovascular disease?
an occlusive process that includes the plugging of a vessel. This results in stopping blood flow to tissue past the block.
infarctive stroke
what is the difference between an embolic or thrombotic stroke? (both are infarctive strokes)
embolic-an embolus from another vessel or from the heart passes upward into cerebral vasculature causing an occlusion
thrombotic-this type of strokeoccurs when vessels gradually occlude due to atherosclerosis or lipohyalinization.
this type of stroke is usually abrupt in onset and does not progress
infarctive stroke
3 types of strokes:
infarctive, hemorrhagic, diffuse ischemia
this type of stroke is caused by failure of a vessel wall leading to vessel rupture and bleeding into surrounding tissue.
hemorrhagic stroke
this type of stroke can be abrupt and progressive
hemorrhagic stroke
this type of stroke is due to failure of the heart to keep up sufficient blood flow to the brain
diffuse ischemia
bleeding within the CNS parenchyma and into the ventricular system results in…
intraparenchymal hemorrhage
the majority of intraparenchymal hemorrhages are due to this pre-existing condition
hypertension
The main cause of an intraparenchymal hemorrhage in young people is
sympathomimetic drugs (like cocaine)
typical locations for spontaneous hypertensive intraparenchymal hemorrhages are:
1-basal ganglia
2-thalamus
3-cerebellum
4-pons
A vascular abnormality that is highly associated with developing an intraparenchymal hemorrhage is…
carotid stenosis
These are degenerative disease of the alpha motorneurons in the ventral horn of the cord. These diseases cause extensive motorneuron death with muscle dennervation and atrophy
Spinal Muscular Atrophies
SMA type 1 is also called
Werdnig-Hoffmann disease
The severe infantile form of SMA type 1, which usually causes death in early infancy is called…
Non-sitters form
SMA type 2 in late infantile form is called…
Sitters
How long do children with SMA type 2 usually live for?
Survival into the school years
SMA type 3 is also called…
Kugelberg-Welander disease
The chronic juvenile phase of SMA type 3, in which patients usually survive into the middle ages of life is called
Walkers
When is the time of onset of SMA type 4?
adult-onset
What is the genetic inheritance pattern for most types of SMA?
autosomal recessive inheritance
The gene involved in SMA is the ______.Two copies of this gene are located on chromosome number____.
- Survival of Motor Neurons gene (SMN)
- chromosome 5
What are the two copies of the SMN genes involved in SMA?
SMN 1 and SMN2
Loss or mutation in ____(gene) correlates with SMA.
SMN1 gene
The number of additional copies of ____(gene) can blunt the expression of SMA.
SMN2
The following are clinical signs of what disease?
Severe hypotonia
Generalized weakness
Muscle atrophy
Loss of tendon reflexes
Involvement of tongue face and jaw muscles (bulbar muscles)
Intercostal muscle involvement produces bell-shaped thorax with belly breathing
Diaphragm involvement brings respiratory distress
Congenital joint contractures can occur
Sensory nerve involvement has also been noted
Comorbid cardiovascular disorders noted
Cognition is often normal
SMA type 1
The following are clinical signs of what disease?
Onset between 6 and 18 months
Progressive hypotonic, areflexive weakness, involving legs more so than arms with minimal involvement of the bulbar muscles
Joint contractures
Can sit unsupported bu cannot stand or ambulate unassisted
Scoliosis develops in the longer term patients
Secondary lung restriction
Cognition is normal
Generally patients survive into their third decade
SMA type 2
The following are clinical signs of what form of SMA?
Progressive weakness involving legs more so than arms
Patients can sit, stand and ambulate alone
Lack bulbar symptoms
Life expectency is normal
SMA type 3
The following are clinical signs of what form of SMA?Adult-onset mild form
Stands and walks
Normal life span
SMA type 4
