Neurology Diseases and Traumatic Brain Injury
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Questions and Answers

What is the primary target of the damage in axonal neuropathies?

Axons

In demyelinating neuropathies, axons are the primary target of damage.

False

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?

<p>Slowed nerve conduction velocity</p> Signup and view all the answers

Which of the following describes a displaced skull fracture?

<p>A fracture in which bone is displaced into the cranial cavity by a distance greater than the thickness of the bone.</p> Signup and view all the answers

What are some of the symptoms that may suggest a basal skull fracture?

<p>Symptoms referable to the lower cranial nerves or the cervicomedullary region, orbital or mastoid hematomas, CSF leakage from the nose or ear, and infection.</p> Signup and view all the answers

Traumatic brain injury (TBI) can be caused by both penetrating and blunt trauma.

<p>True</p> Signup and view all the answers

What is the Glasgow Coma Scale (GCS) used for?

<p>Assessing the level of consciousness in acutely brain-injured patients.</p> Signup and view all the answers

A change in level of consciousness (LOC) is a sensitive indicator of altered brain function.

<p>True</p> Signup and view all the answers

What does the pupillary reflex assess?

<p>The function of the brainstem and cranial nerves II and III.</p> Signup and view all the answers

Decorticate posturing is a more serious neurological sign than decerebrate posturing.

<p>False</p> Signup and view all the answers

A concussion is a mild traumatic brain injury with no evidence of brain damage on CT scans.

<p>True</p> Signup and view all the answers

What is the most common type of brain injury encountered by military personnel and athletes?

<p>Concussion</p> Signup and view all the answers

What are some of the clinical manifestations associated with a concussion?

<p>Sudden onset of transient neurologic dysfunction, including loss of consciousness, temporary respiratory arrest, loss of reflexes, and postconcussive neuropsychiatric syndromes.</p> Signup and view all the answers

A contusion is a bruise on the brain.

<p>True</p> Signup and view all the answers

A laceration is a tear in the brain tissue caused by a penetrating object.

<p>True</p> Signup and view all the answers

Match the following brain injuries with their descriptions:

<p>Coup injury = Contusion at the point of contact. Contrecoup injury = Contusion on the brain surface diametrically opposite to the point of impact. Direct parenchymal injury = Brain injuries caused by transmission of kinetic energy to the brain. Subdural hematoma = Bleeding that dissects through the two layers of the dura, producing a subdural hematoma.</p> Signup and view all the answers

Cerebrovascular disease involves altered blood flow to the brain, and can result in ischemic or hemorrhagic stroke.

<p>True</p> Signup and view all the answers

What are the primary targets of damage in axonal neuropathies?

<p>Axons</p> Signup and view all the answers

What type of pattern of progression is often seen in axonal neuropathies?

<p>Dying-back pattern</p> Signup and view all the answers

Which of the following are electrophysiologic hallmarks of axonal neuropathies?

<p>Preservation of conduction velocity</p> Signup and view all the answers

What is the primary target of damage in demyelinating neuropathies?

<p>Schwann cells and their myelin sheaths</p> Signup and view all the answers

What is a displaced skull fracture?

<p>A fracture where the bone is displaced into the cranial cavity by a distance greater than the thickness of the bone</p> Signup and view all the answers

What is the most sensitive indicator of altered brain function?

<p>Change in level of consciousness (LOC)</p> Signup and view all the answers

Which cranial nerves are involved in the pupillary reflex?

<p>CNIII</p> Signup and view all the answers

What is the most powerful predictor of patient outcome after traumatic brain injury?

<p>Motor response</p> Signup and view all the answers

What is the most common type of traumatic brain injury?

<p>Concussion</p> Signup and view all the answers

Which of the following is NOT a risk factor for concussion?

<p>Fractured skull</p> Signup and view all the answers

What are the common clinical manifestations of concussion?

<p>All of the above</p> Signup and view all the answers

What is the most sensitive diagnostic tool for a concussion?

<p>CT scan</p> Signup and view all the answers

What is a contusion?

<p>A bruise caused by blunt trauma</p> Signup and view all the answers

What is a laceration?

<p>An injury caused by penetration of an object and tearing of tissue</p> Signup and view all the answers

Where can a contrecoup injury occur?

<p>Both A and B</p> Signup and view all the answers

What is a key characteristic of Huntington's disease related to its genetic cause?

<p>It is caused by an increase in the number of CAG repeats beyond 35 in the HTT gene.</p> Signup and view all the answers

Which clinical manifestation is typically associated with Huntington's disease?

<p>Chorea, which involves jerky and hyperkinetic movements.</p> Signup and view all the answers

Which of the following describes a nonneoplastic lesion found in individuals with NF2?

<p>Menigoangiomatosis, a proliferation of meningeal cells and blood vessels.</p> Signup and view all the answers

What is a common initial symptom of Huntington's disease related to cognitive function?

<p>Forgetfulness and various cognitive dysfunctions.</p> Signup and view all the answers

Which physiological alteration is a hallmark of Huntington's disease?

<p>Progressive degeneration of striatal neurons.</p> Signup and view all the answers

What condition is classified as neurologic signs and symptoms with acute onset persisting beyond 24 hours?

<p>Stroke</p> Signup and view all the answers

What is the role of Thrombotic strokes in cerebrovascular disease?

<p>They result from sudden occlusion due to thrombus formation.</p> Signup and view all the answers

What best describes focal cerebral ischemia?

<p>Reduction of blood flow to a localized brain area.</p> Signup and view all the answers

What neurological sign is commonly associated with stroke?

<p>Contralateral hemiplegia</p> Signup and view all the answers

Which of the following factors does NOT typically contribute to embolic strokes?

<p>Traumatic brain injury</p> Signup and view all the answers

Which imaging techniques are most commonly used for diagnosing cerebrovascular disease?

<p>CT and MRI</p> Signup and view all the answers

What is the main consequence of cerebrovascular disease?

<p>Tissue infarction</p> Signup and view all the answers

What is the primary mechanism by which secondary hemorrhage occurs following cerebral infarction?

<p>Ischemia-reperfusion injury</p> Signup and view all the answers

Which of the following factors is most strongly associated with the occurrence of occlusive vascular disease leading to cerebral infarction?

<p>Chronic hypertension</p> Signup and view all the answers

What distinguishes a nonhemorrhagic infarct from a hemorrhagic infarct in clinical terms?

<p>Nature and extent of secondary injury</p> Signup and view all the answers

What role does oxidative stress play in cerebral infarction?

<p>Contributes to ischemia-reperfusion injury</p> Signup and view all the answers

Which location in the brain is most commonly affected by ischemic strokes?

<p>Basal ganglia</p> Signup and view all the answers

What condition may lead to global cerebral hypoxia or ischemia?

<p>Severe hypotension</p> Signup and view all the answers

Which statement is true regarding the management of nonhemorrhagic versus hemorrhagic infarcts?

<p>Nonhemorrhagic strokes may be treated with thrombolytics</p> Signup and view all the answers

Which of the following is a common trait of both ischemic and hemorrhagic strokes?

<p>Both can cause disruption of the blood-brain barrier</p> Signup and view all the answers

What type of transformation may occur as a result of edema in the brain following ischemia-reperfusion injury?

<p>Hemorrhagic transformation</p> Signup and view all the answers

Which of the following best describes the progression of multiple sclerosis (MS) over time?

<p>Frequency of relapses tends to decrease while neurologic function deteriorates</p> Signup and view all the answers

What is a common initial manifestation of multiple sclerosis involving the optic nerve?

<p>Optic neuritis</p> Signup and view all the answers

Which demographic is most commonly affected by multiple sclerosis?

<p>Young adults</p> Signup and view all the answers

Which factor is considered a significant contributor to the development of Parkinson's disease?

<p>Dopamine deficiency in the basal ganglia</p> Signup and view all the answers

What is one of the primary treatment strategies for Parkinson's disease?

<p>Restoration of brain dopamine levels</p> Signup and view all the answers

Which of the following symptoms is NOT typically associated with Parkinson's disease?

<p>Excessive energy levels</p> Signup and view all the answers

What type of lesions contribute to sensory impairments in multiple sclerosis?

<p>Spinal cord lesions</p> Signup and view all the answers

How does the presence of Lewy bodies relate to Parkinson's disease?

<p>They are aggregates linked with dopamine deficiency</p> Signup and view all the answers

What neurological impact does involvement of the brainstem in multiple sclerosis produce?

<p>Cranial nerve signs such as ataxia</p> Signup and view all the answers

Parkinson's disease can lead to dementia in what percentage of individuals, particularly with increasing age?

<p>10% to 15%</p> Signup and view all the answers

What is the main complication that patients face in the early days following a subarachnoid hemorrhage?

<p>Increased risk of ischemic injury from vasospasm</p> Signup and view all the answers

Which vascular malformation is most commonly associated with a risk of hemorrhage?

<p>Arteriovenous malformations</p> Signup and view all the answers

What could be a significant outcome of large arteriovenous malformations in newborns?

<p>Congestive heart failure due to shunt effects</p> Signup and view all the answers

Cavernous malformations are most often found in which region of the brain?

<p>Cerebellum</p> Signup and view all the answers

How are familial cavernous malformations typically inherited?

<p>Autosomal dominant</p> Signup and view all the answers

During which age range do cavernous malformations most commonly present symptoms?

<p>10-30 years</p> Signup and view all the answers

Which of the following describing arteriovenous malformations is correct?

<p>They are characterized by high blood flow and arteriovenous shunting.</p> Signup and view all the answers

What is a hallmark feature of familial cavernous malformations?

<p>Multiplicity of lesions</p> Signup and view all the answers

What is the most common presentation of cavernous malformations?

<p>Seizure disorder</p> Signup and view all the answers

Which factor contributes to the increased risk of cavernous malformations in males?

<p>Doubling of cases compared to females</p> Signup and view all the answers

What is the primary cause of a hemorrhagic stroke?

<p>Blood vessel rupture leading to bleeding in the brain</p> Signup and view all the answers

Which neurological sign is most commonly observed during a stroke event?

<p>Contralateral hemiplegia</p> Signup and view all the answers

How are transient ischemic attacks (TIAs) primarily characterized?

<p>Neurologic symptoms that resolve within 24 hours</p> Signup and view all the answers

What imaging techniques are considered most reliable for diagnosing cerebrovascular disease?

<p>CT and MRI</p> Signup and view all the answers

Which factor is typically associated with an increased risk of thrombotic strokes?

<p>Hypertension</p> Signup and view all the answers

What is the leading cause of morbidity and mortality related to cerebrovascular disease?

<p>Ischemic strokes</p> Signup and view all the answers

Which of the following best describes the hallmark of focal cerebral ischemia?

<p>Infarction due to ceased blood flow</p> Signup and view all the answers

What is the primary consequence of the mutations in the FGF receptor 3 (FGFR3) gene in achondroplasia?

<p>Decreased cartilage matrix production</p> Signup and view all the answers

Which of the following symptoms is NOT typically associated with reactive arthritis?

<p>Chronic systemic lupus erythematosus</p> Signup and view all the answers

In patients diagnosed with achondroplasia, which anatomical feature remains relatively unchanged compared to typical development?

<p>Length of the trunk</p> Signup and view all the answers

Which of the following factors is commonly associated with the genetic mutations causing achondroplasia?

<p>Germline mutations predominantly from the paternal allele</p> Signup and view all the answers

What is the typical pattern of arthritis presentation in reactive arthritis following infection?

<p>Acute-onset asymmetric oligoarthritis</p> Signup and view all the answers

Which of the following best describes the pathophysiology of cerebral edema?

<p>Increased fluid leakage from blood vessels leads to brain swelling.</p> Signup and view all the answers

Normal pressure hydrocephalus primarily results in which of the following symptoms?

<p>Progressive mental impairment, walking difficulties, and bladder control issues.</p> Signup and view all the answers

What causes increased intracranial pressure (ICP)?

<p>Space-occupying lesions and obstruction of CSF flow.</p> Signup and view all the answers

In the context of vascular dementia, which treatment option is aimed specifically at improving blood flow to the brain?

<p>Surgery to improve blood flow.</p> Signup and view all the answers

Which factor is NOT a known risk associated with normal pressure hydrocephalus?

<p>Genetic predisposition for hydrocephalus.</p> Signup and view all the answers

What is one effect of generalized cerebral edema on brain structure?

<p>Flattening of gyri and narrowing of sulci.</p> Signup and view all the answers

What is the normal range for intracranial pressure (ICP)?

<p>0 to 15 mm Hg</p> Signup and view all the answers

Which type of edema formation is characterized by a localized response to inflammation or neoplasms?

<p>Vasogenic edema</p> Signup and view all the answers

Which surgical intervention is considered for patients with vascular dementia and impaired blood flow?

<p>Bypass surgery</p> Signup and view all the answers

What is the average age of onset for juvenile dermatomyositis?

<p>7 years</p> Signup and view all the answers

Which symptom is most commonly associated with dermatomyositis?

<p>Prominent Gottron papules</p> Signup and view all the answers

What characterizes polymyositis when compared to dermatomyositis?

<p>It occurs only in adults</p> Signup and view all the answers

In which areas of the body can telangiectasias be observed in dermatomyositis?

<p>Nail folds, eyelids, and gums</p> Signup and view all the answers

What is a common early symptom of muscular dystrophies?

<p>Progressive muscle damage</p> Signup and view all the answers

Which of the following statements best describes congenital muscular dystrophies?

<p>They are associated with CNS developmental abnormalities</p> Signup and view all the answers

What is the mechanism believed to underlie polymyositis?

<p>An immunologic basis</p> Signup and view all the answers

Which muscle group is typically affected first in dermatomyositis?

<p>Proximal muscles of the body</p> Signup and view all the answers

What is the primary association of Anti-Mi2 antibodies in dermatomyositis?

<p>Prominent Gottron papules and heliotrope rash</p> Signup and view all the answers

Muscular dystrophies typically come to attention during which stage of life?

<p>Childhood to adulthood</p> Signup and view all the answers

What is the primary characteristic of Ewing sarcoma?

<p>It is characterized by primitive round cells without obvious differentiation.</p> Signup and view all the answers

Which gene is commonly involved in the majority of Ewing sarcoma tumors?

<p>EWSR1</p> Signup and view all the answers

Which demographic is most commonly affected by Ewing sarcoma?

<p>Teenagers and young adults aged 5 to 25 years.</p> Signup and view all the answers

What imaging feature is often observed in Ewing sarcoma?

<p>Lytic tumor with moth-eaten margins and layers of reactive bone.</p> Signup and view all the answers

What are common symptoms associated with Ewing sarcoma?

<p>Pain, fever, weight loss, and leukocytosis.</p> Signup and view all the answers

Which of the following is NOT a common location for Ewing sarcoma?

<p>Proximal phalanx of the hand.</p> Signup and view all the answers

Which populations are least likely to be affected by Ewing sarcoma?

<p>Individuals of African or Asian descent.</p> Signup and view all the answers

Which condition can mimic the symptoms of Ewing sarcoma?

<p>Osteomyelitis.</p> Signup and view all the answers

What type of periosteal reaction is typically associated with Ewing sarcoma?

<p>Onion-skin fashion.</p> Signup and view all the answers

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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Disease of the Peripheral and Central Nervous System PDF

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.

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