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Questions and Answers
What is the primary target of the damage in axonal neuropathies?
What is the primary target of the damage in axonal neuropathies?
Axons
In demyelinating neuropathies, axons are the primary target of damage.
In demyelinating neuropathies, axons are the primary target of damage.
False
What is the primary hallmark of axonal neuropathies in terms of electrophysiology?
What is the primary hallmark of axonal neuropathies in terms of electrophysiology?
A reduction in signal amplitude
What is the electrophysiological hallmark of demyelinating neuropathies?
What is the electrophysiological hallmark of demyelinating neuropathies?
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Which of the following describes a displaced skull fracture?
Which of the following describes a displaced skull fracture?
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What are some of the symptoms that may suggest a basal skull fracture?
What are some of the symptoms that may suggest a basal skull fracture?
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Traumatic brain injury (TBI) can be caused by both penetrating and blunt trauma.
Traumatic brain injury (TBI) can be caused by both penetrating and blunt trauma.
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What is the Glasgow Coma Scale (GCS) used for?
What is the Glasgow Coma Scale (GCS) used for?
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A change in level of consciousness (LOC) is a sensitive indicator of altered brain function.
A change in level of consciousness (LOC) is a sensitive indicator of altered brain function.
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What does the pupillary reflex assess?
What does the pupillary reflex assess?
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Decorticate posturing is a more serious neurological sign than decerebrate posturing.
Decorticate posturing is a more serious neurological sign than decerebrate posturing.
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A concussion is a mild traumatic brain injury with no evidence of brain damage on CT scans.
A concussion is a mild traumatic brain injury with no evidence of brain damage on CT scans.
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What is the most common type of brain injury encountered by military personnel and athletes?
What is the most common type of brain injury encountered by military personnel and athletes?
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What are some of the clinical manifestations associated with a concussion?
What are some of the clinical manifestations associated with a concussion?
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A contusion is a bruise on the brain.
A contusion is a bruise on the brain.
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A laceration is a tear in the brain tissue caused by a penetrating object.
A laceration is a tear in the brain tissue caused by a penetrating object.
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Match the following brain injuries with their descriptions:
Match the following brain injuries with their descriptions:
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Cerebrovascular disease involves altered blood flow to the brain, and can result in ischemic or hemorrhagic stroke.
Cerebrovascular disease involves altered blood flow to the brain, and can result in ischemic or hemorrhagic stroke.
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What are the primary targets of damage in axonal neuropathies?
What are the primary targets of damage in axonal neuropathies?
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What type of pattern of progression is often seen in axonal neuropathies?
What type of pattern of progression is often seen in axonal neuropathies?
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Which of the following are electrophysiologic hallmarks of axonal neuropathies?
Which of the following are electrophysiologic hallmarks of axonal neuropathies?
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What is the primary target of damage in demyelinating neuropathies?
What is the primary target of damage in demyelinating neuropathies?
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What is a displaced skull fracture?
What is a displaced skull fracture?
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What is the most sensitive indicator of altered brain function?
What is the most sensitive indicator of altered brain function?
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Which cranial nerves are involved in the pupillary reflex?
Which cranial nerves are involved in the pupillary reflex?
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What is the most powerful predictor of patient outcome after traumatic brain injury?
What is the most powerful predictor of patient outcome after traumatic brain injury?
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What is the most common type of traumatic brain injury?
What is the most common type of traumatic brain injury?
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Which of the following is NOT a risk factor for concussion?
Which of the following is NOT a risk factor for concussion?
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What are the common clinical manifestations of concussion?
What are the common clinical manifestations of concussion?
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What is the most sensitive diagnostic tool for a concussion?
What is the most sensitive diagnostic tool for a concussion?
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What is a contusion?
What is a contusion?
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What is a laceration?
What is a laceration?
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Where can a contrecoup injury occur?
Where can a contrecoup injury occur?
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What is a key characteristic of Huntington's disease related to its genetic cause?
What is a key characteristic of Huntington's disease related to its genetic cause?
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Which clinical manifestation is typically associated with Huntington's disease?
Which clinical manifestation is typically associated with Huntington's disease?
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Which of the following describes a nonneoplastic lesion found in individuals with NF2?
Which of the following describes a nonneoplastic lesion found in individuals with NF2?
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What is a common initial symptom of Huntington's disease related to cognitive function?
What is a common initial symptom of Huntington's disease related to cognitive function?
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Which physiological alteration is a hallmark of Huntington's disease?
Which physiological alteration is a hallmark of Huntington's disease?
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What condition is classified as neurologic signs and symptoms with acute onset persisting beyond 24 hours?
What condition is classified as neurologic signs and symptoms with acute onset persisting beyond 24 hours?
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What is the role of Thrombotic strokes in cerebrovascular disease?
What is the role of Thrombotic strokes in cerebrovascular disease?
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What best describes focal cerebral ischemia?
What best describes focal cerebral ischemia?
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What neurological sign is commonly associated with stroke?
What neurological sign is commonly associated with stroke?
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Which of the following factors does NOT typically contribute to embolic strokes?
Which of the following factors does NOT typically contribute to embolic strokes?
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Which imaging techniques are most commonly used for diagnosing cerebrovascular disease?
Which imaging techniques are most commonly used for diagnosing cerebrovascular disease?
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What is the main consequence of cerebrovascular disease?
What is the main consequence of cerebrovascular disease?
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What is the primary mechanism by which secondary hemorrhage occurs following cerebral infarction?
What is the primary mechanism by which secondary hemorrhage occurs following cerebral infarction?
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Which of the following factors is most strongly associated with the occurrence of occlusive vascular disease leading to cerebral infarction?
Which of the following factors is most strongly associated with the occurrence of occlusive vascular disease leading to cerebral infarction?
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What distinguishes a nonhemorrhagic infarct from a hemorrhagic infarct in clinical terms?
What distinguishes a nonhemorrhagic infarct from a hemorrhagic infarct in clinical terms?
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What role does oxidative stress play in cerebral infarction?
What role does oxidative stress play in cerebral infarction?
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Which location in the brain is most commonly affected by ischemic strokes?
Which location in the brain is most commonly affected by ischemic strokes?
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What condition may lead to global cerebral hypoxia or ischemia?
What condition may lead to global cerebral hypoxia or ischemia?
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Which statement is true regarding the management of nonhemorrhagic versus hemorrhagic infarcts?
Which statement is true regarding the management of nonhemorrhagic versus hemorrhagic infarcts?
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Which of the following is a common trait of both ischemic and hemorrhagic strokes?
Which of the following is a common trait of both ischemic and hemorrhagic strokes?
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What type of transformation may occur as a result of edema in the brain following ischemia-reperfusion injury?
What type of transformation may occur as a result of edema in the brain following ischemia-reperfusion injury?
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Which of the following best describes the progression of multiple sclerosis (MS) over time?
Which of the following best describes the progression of multiple sclerosis (MS) over time?
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What is a common initial manifestation of multiple sclerosis involving the optic nerve?
What is a common initial manifestation of multiple sclerosis involving the optic nerve?
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Which demographic is most commonly affected by multiple sclerosis?
Which demographic is most commonly affected by multiple sclerosis?
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Which factor is considered a significant contributor to the development of Parkinson's disease?
Which factor is considered a significant contributor to the development of Parkinson's disease?
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What is one of the primary treatment strategies for Parkinson's disease?
What is one of the primary treatment strategies for Parkinson's disease?
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Which of the following symptoms is NOT typically associated with Parkinson's disease?
Which of the following symptoms is NOT typically associated with Parkinson's disease?
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What type of lesions contribute to sensory impairments in multiple sclerosis?
What type of lesions contribute to sensory impairments in multiple sclerosis?
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How does the presence of Lewy bodies relate to Parkinson's disease?
How does the presence of Lewy bodies relate to Parkinson's disease?
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What neurological impact does involvement of the brainstem in multiple sclerosis produce?
What neurological impact does involvement of the brainstem in multiple sclerosis produce?
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Parkinson's disease can lead to dementia in what percentage of individuals, particularly with increasing age?
Parkinson's disease can lead to dementia in what percentage of individuals, particularly with increasing age?
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What is the main complication that patients face in the early days following a subarachnoid hemorrhage?
What is the main complication that patients face in the early days following a subarachnoid hemorrhage?
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Which vascular malformation is most commonly associated with a risk of hemorrhage?
Which vascular malformation is most commonly associated with a risk of hemorrhage?
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What could be a significant outcome of large arteriovenous malformations in newborns?
What could be a significant outcome of large arteriovenous malformations in newborns?
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Cavernous malformations are most often found in which region of the brain?
Cavernous malformations are most often found in which region of the brain?
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How are familial cavernous malformations typically inherited?
How are familial cavernous malformations typically inherited?
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During which age range do cavernous malformations most commonly present symptoms?
During which age range do cavernous malformations most commonly present symptoms?
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Which of the following describing arteriovenous malformations is correct?
Which of the following describing arteriovenous malformations is correct?
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What is a hallmark feature of familial cavernous malformations?
What is a hallmark feature of familial cavernous malformations?
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What is the most common presentation of cavernous malformations?
What is the most common presentation of cavernous malformations?
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Which factor contributes to the increased risk of cavernous malformations in males?
Which factor contributes to the increased risk of cavernous malformations in males?
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What is the primary cause of a hemorrhagic stroke?
What is the primary cause of a hemorrhagic stroke?
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Which neurological sign is most commonly observed during a stroke event?
Which neurological sign is most commonly observed during a stroke event?
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How are transient ischemic attacks (TIAs) primarily characterized?
How are transient ischemic attacks (TIAs) primarily characterized?
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What imaging techniques are considered most reliable for diagnosing cerebrovascular disease?
What imaging techniques are considered most reliable for diagnosing cerebrovascular disease?
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Which factor is typically associated with an increased risk of thrombotic strokes?
Which factor is typically associated with an increased risk of thrombotic strokes?
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What is the leading cause of morbidity and mortality related to cerebrovascular disease?
What is the leading cause of morbidity and mortality related to cerebrovascular disease?
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Which of the following best describes the hallmark of focal cerebral ischemia?
Which of the following best describes the hallmark of focal cerebral ischemia?
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What is the primary consequence of the mutations in the FGF receptor 3 (FGFR3) gene in achondroplasia?
What is the primary consequence of the mutations in the FGF receptor 3 (FGFR3) gene in achondroplasia?
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Which of the following symptoms is NOT typically associated with reactive arthritis?
Which of the following symptoms is NOT typically associated with reactive arthritis?
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In patients diagnosed with achondroplasia, which anatomical feature remains relatively unchanged compared to typical development?
In patients diagnosed with achondroplasia, which anatomical feature remains relatively unchanged compared to typical development?
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Which of the following factors is commonly associated with the genetic mutations causing achondroplasia?
Which of the following factors is commonly associated with the genetic mutations causing achondroplasia?
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What is the typical pattern of arthritis presentation in reactive arthritis following infection?
What is the typical pattern of arthritis presentation in reactive arthritis following infection?
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Which of the following best describes the pathophysiology of cerebral edema?
Which of the following best describes the pathophysiology of cerebral edema?
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Normal pressure hydrocephalus primarily results in which of the following symptoms?
Normal pressure hydrocephalus primarily results in which of the following symptoms?
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What causes increased intracranial pressure (ICP)?
What causes increased intracranial pressure (ICP)?
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In the context of vascular dementia, which treatment option is aimed specifically at improving blood flow to the brain?
In the context of vascular dementia, which treatment option is aimed specifically at improving blood flow to the brain?
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Which factor is NOT a known risk associated with normal pressure hydrocephalus?
Which factor is NOT a known risk associated with normal pressure hydrocephalus?
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What is one effect of generalized cerebral edema on brain structure?
What is one effect of generalized cerebral edema on brain structure?
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What is the normal range for intracranial pressure (ICP)?
What is the normal range for intracranial pressure (ICP)?
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Which type of edema formation is characterized by a localized response to inflammation or neoplasms?
Which type of edema formation is characterized by a localized response to inflammation or neoplasms?
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Which surgical intervention is considered for patients with vascular dementia and impaired blood flow?
Which surgical intervention is considered for patients with vascular dementia and impaired blood flow?
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What is the average age of onset for juvenile dermatomyositis?
What is the average age of onset for juvenile dermatomyositis?
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Which symptom is most commonly associated with dermatomyositis?
Which symptom is most commonly associated with dermatomyositis?
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What characterizes polymyositis when compared to dermatomyositis?
What characterizes polymyositis when compared to dermatomyositis?
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In which areas of the body can telangiectasias be observed in dermatomyositis?
In which areas of the body can telangiectasias be observed in dermatomyositis?
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What is a common early symptom of muscular dystrophies?
What is a common early symptom of muscular dystrophies?
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Which of the following statements best describes congenital muscular dystrophies?
Which of the following statements best describes congenital muscular dystrophies?
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What is the mechanism believed to underlie polymyositis?
What is the mechanism believed to underlie polymyositis?
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Which muscle group is typically affected first in dermatomyositis?
Which muscle group is typically affected first in dermatomyositis?
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What is the primary association of Anti-Mi2 antibodies in dermatomyositis?
What is the primary association of Anti-Mi2 antibodies in dermatomyositis?
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Muscular dystrophies typically come to attention during which stage of life?
Muscular dystrophies typically come to attention during which stage of life?
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What is the primary characteristic of Ewing sarcoma?
What is the primary characteristic of Ewing sarcoma?
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Which gene is commonly involved in the majority of Ewing sarcoma tumors?
Which gene is commonly involved in the majority of Ewing sarcoma tumors?
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Which demographic is most commonly affected by Ewing sarcoma?
Which demographic is most commonly affected by Ewing sarcoma?
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What imaging feature is often observed in Ewing sarcoma?
What imaging feature is often observed in Ewing sarcoma?
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What are common symptoms associated with Ewing sarcoma?
What are common symptoms associated with Ewing sarcoma?
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Which of the following is NOT a common location for Ewing sarcoma?
Which of the following is NOT a common location for Ewing sarcoma?
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Which populations are least likely to be affected by Ewing sarcoma?
Which populations are least likely to be affected by Ewing sarcoma?
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Which condition can mimic the symptoms of Ewing sarcoma?
Which condition can mimic the symptoms of Ewing sarcoma?
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What type of periosteal reaction is typically associated with Ewing sarcoma?
What type of periosteal reaction is typically associated with Ewing sarcoma?
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Study Notes
Disease of the Peripheral and Central Nervous System
- Missing slides: Cellular path of CNS, Diffuse axonal injury, spinal cord injury, vascular malformations
- Topics covered in class begining: GB, Trigeminal Neuralgia, Bell palsy (associated nerves), Carpal tunnel/compression neuropathies, MG, Muscular Dystrophies, Schwannomas, Neurofibromas, Neurofibromatosis type 1 and 2, Cerebral Edema (different types), Increased ICP, Neural tube defects and folate, Assessment of brain injury
- Compare/contrast disease etiology, pathophysiology, and clinical manifestations of cranial nerves
Demyelinating/Axonal Neuropathies
- Axons are the primary target in peripheral neuropathies
- Damage is progressive and degeneration outpaces repair
- Electrophysiologic hallmark: reduction in signal amplitude due to axon loss
- Two patterns of damage: acute axonal (distal axon degeneration) and acute demyelinating (random segmental myelin degeneration, sparing axons)
Traumatic Brain Injury
- Patho: Skull fractures, parenchymal injury, and vascular injury can coexist
- Magnitude and distribution of injury depend on object type, force, and head motion
- Diagnosis made at time of injury or within 24 hours
Glasgow Coma Scale
- Tool for assessing level of consciousness in acute brain injury
- Scores given based on eye opening, verbal response, and motor response
Concussion
- Mild traumatic brain injury; most common in military personnel and athletes
- Pathogenesis unknown; likely dysregulation of the reticular activating system (in the brainstem)
- Altered consciousness (< 30 minutes) without evident brain damage
- Common symptoms: headache, nausea, vomiting, dizziness, fatigue, blurred vision, cognitive/emotional disturbances
Contusion
- Brain tissue damage (necrosis, laceration, bruising)
- Similar to a bruise; laceration = penetration and tearing
Coup-Contrecoup Injury
- Coup: contusion at the site of impact
- Contrecoup: contusion on the opposite side of impact (occurs with head movement)
Cerebrovascular Disease and Stroke
- Ischemic: reduced blood supply/oxygen to brain tissues (most common)
- Hemorrhagic: blood vessel rupture inside brain
- Stroke: acute neurological signs/symptoms lasting >24 hours (transient ischemic event: disappears within 24 hours). 3rd leading cause of death, most frequent cause of neurologic morbidity/mortality
Intracranial Hemorrhage
- Subarachnoid hemorrhage: rupture of cerebral aneurysm, most common cause
- Risk factors: hypertension, acute alcohol intoxication, recreational drug use (cocaine)
- Arteriovenous malformations (AVMs): abnormal tangled blood vessels, common cause of hemorrhage
- Cavernous malformations: distended vascular channels; often familial
Vascular Malformations
- Classified into arteriovenous malformations (AVMs), cavernous malformations, capillary telangiectasias, and venous angiomas
- AVMs, cavernous malformations: hemorrhage risk
Stroke Sequence
- Motor/sensory deficits typically contralateral to the side of the stroke
- Rehabilitation crucial, especially in the beginning
Multiple Sclerosis
- Autoimmune demyelinating disorder
- Patchy white matter lesions, progressing over time
- Common symptoms: vision loss, pain, fatigue, impaired coordination, muscle weakness, numbness, thinking/memory problems
Parkinson's Disease
- Dopamine deficiency in basal ganglia (substantia nigra)
- Common symptoms: tremor, mask-like face, arms flexed at elbows and wrists, stooped posture, rigidity, hips and knees flexed, shuffling steps
- No cure but treatment can help restore dopamine levels or activity
Spina Bifida/Neural Tube Defects
- Midline malformations involving neural tissue, meninges, and overlying bone or soft tissue
- Risk factors: folate deficiency during pregnancy
- Manifestations vary (occult defects to severe malformations with exposed spinal cord)
Amyotrophic Lateral Sclerosis (ALS)
- Progressive neurodegenerative disorder
- Loss of upper motor neurons (cerebral cortex) and lower motor neurons (spinal cord/brainstem)
Bell's Palsy
- Idiopathic facial nerve (VII) neuropathy
- Paralysis of facial muscles on one side of face
Trigeminal Neuralgia
- Sharp, recurring facial pain in one or more trigeminal nerve branches (most commonly V3)
Inflammatory Neuropathies
- Acute inflammatory demyelinating polyneuropathy (AIDP): immune-mediated demyelinating peripheral neuropathy, often following an infection.
- Chronic inflammatory demyelinating polyneuropathy (CIDP): mixed sensorimotor polyneuropathy, usually of gradual onset (different time course from AIDP)
Infectious Neuropathies
- Neuron destruction, damaging both proximal and distal parts of the body
Varicella Zoster
- Reactivation of the varicella-zoster virus (VZV) can cause shingles
- Manifestations usually confined to the dermatome innervated by the affected ganglia
- Often accompanied by significant pain
Compression Neuropathies
- Carpal tunnel syndrome: median nerve compression at the wrist
- Common in women; can also occur with edema, pregnancy, certain medical conditions, and repetitive motions
Myasthenia Gravis
- Autoimmune disease directed against acetylcholine receptors
Muscular Dystrophies
- Group of inherited disorders characterized by progressive muscle damage
Schwannomas
- Benign nerve sheath tumors
- Often arise from peripheral nerves
Neurofibromatosis Type 1 and 2
- Neurofibromas (benign)
- Associated with other tumors and systemic abnormalities
Huntington's Disease
- Autosomal dominant neurological disorder, with movement and dementia abnormalities
- Caused by CAG trinucleotide repeat expansions
Osteomalacia / Rickets
- Bone demineralization due to vitamin D deficiency
- Rickets is in children, osteomalacia in adults. Symptoms include skeletal pain, skeletal deformities, increased fractures
Osteopetrosis
- Rare genetic condition in which bone resorption is defective
- Results in thickened, brittle, and easily fractured bones
Osteogenesis Imperfecta
- Inherited disorder affecting connective tissue
- Genetic abnormality leads to problems in type I collagen synthesis
- Clinical features: blue sclerae, hearing loss, dental imperfections
Achondroplasia
- Skeletal dysplasia; results in dwarfism.
Ewing Sarcoma
- Difficult to diagnose cancer in bones
- Often in children/young adults; primarily affects long bones
Gout
- Hyperuricemia leads to urate crystal deposition in joints
- Characterized by acute inflammatory attacks
Pseudogout
- Calcium pyrophosphate dihydrate crystal deposition disease (CPPD).
Paget's Disease (Osteitis Deformans)
- Chronic disorder characterized by excessive bone resorption and formation.
Ankylosing Spondylitis
- Inflammatory disease affecting the spine and sacroiliac joints
- Characterized by low back pain, limited range of motion.
Ehlers-Danlos Syndromes (EDS)
- Rare genetic disorders affecting connective tissue
- Features: hypermobile joints, stretchy skin, wounds heal poorly
Juvenile Idiopathic Arthritis (JIA)
- Arthritis in individuals younger than 16
- Can involve various joints and have systemic manifestations
Fractures
- Stages of fracture healing: Hematoma formation, fibrocartilage formation, callus formation, ossification formation, consolidation/remodeling.
Complications of Fractures
- Delayed union, malunion, nonunion, osteonecrosis, and osteomyelitis.
Calcium Homeostasis
- Regulation of calcium balance in the body involving parathyroid hormone, vitamin D, and calcitonin.
Ganglion Cysts and Synovial Cysts
- Benign, fluid-filled masses arising near joints and tendons
- Ganglion cysts are commonly found around the wrist
- Synovial cysts are associated with inflammation (e.g., rheumatoid arthritis).
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Description
Test your knowledge on various diseases affecting the peripheral and central nervous systems, including demyelinating and axonal neuropathies. This quiz also explores traumatic brain injury, focusing on etiology, pathophysiology, and clinical manifestations. Challenge yourself with questions on GB, trigeminal neuralgia, and other relevant conditions covered in class.