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Clinical Medicine 4 > Lecture 9 (neuro)-Exam 3 > Flashcards

Lecture 9 (neuro)-Exam 3 Flashcards

1
Q

Seizure disorders:
* What are seizures?
* What are provoked seizures?

A
  • Seizures are episodes of transient neurological changes due to hypersynchronous, hyperexcited neural activity.
  • Provoked seizures: related to identifiable cause (toxins , alcohol withdrawal, metabolic, hypoglycemia, trauma, stroke)
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2
Q

Seizure Disorders
* What is epilepsy?
* What is first time unprovoked seizures?

A
  • Epilepsy: is the syndrome of two or more unprovoked seizures that occur more than 24 hours apart.
  • First Time Unprovoked seizures (Epileptic): occur spontaneously and recur when not treated. Of unknown etiology or when related to a brain lesion or progressive neurological disorder
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3
Q

⭐️

Etiology of Seizures
* What is structural?
* What is genetic? What is a syndrome?

A

Structural
* Cortical or vascular malformations, traumatic brain injuries, tumors, stroke

Genetic
* Chromosomal or molecular, some connection with febrile seizures
* Alpers Syndrome – rare genetic disorder that affects the brain, liver and muscles – causes intractable seizures with status epilepticus with developmental dementia and liver dysfunction

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4
Q

Etiology of Seizures
* What are metabolic sources?
* What are autoimmune sources?

A

Metabolic
* Folate deficiency and creatine disorders
* Inborn errors of metabolism and inherited conditions – typically present during childhood

Immune
* Autoimmune diseases such as Lupus – generally cured with immunotherapy and seizure medication

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5
Q

Etiology of Seizures
* What are the infectious sources?(3)

A
  • Acute infective or inflammatory illness such as bacterial meningitis
  • AIDS – seizures result from central nervous system toxoplasmosis
  • Brain abscess – develop generally in first year of treatment
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6
Q

Clinical Presentation of Seizures
* Seizure presentation in older adults is what? (4)
* What is most common?

A
  • Seizure presentation in older adults is less likely with convulsion and aura and more with nonspecific confusion, dizziness
  • Behavior changes are most common
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7
Q

Clinical Presentation of Seizures
* Sudden what?
* Recurrent what?
* What happens with mental state?

A
  • Sudden falls with no recall or warning
  • Recurrent events in various positions and circumstances
  • Arousal from sleep with confusion or disorientation
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8
Q

Generalized seizures

Absence seizure (nonconvulsive)
* Begins when? (age)
* Characterized by what?
* Begin and ends how? (timing)
* What does the EEG show?
* precipitated by waht?

A
  • begins in childhood (between 2 and 10)
  • characterized by an unresponsive blank stare lasting seconds
  • begins and ends abruptly
  • the EEG shows a characteristic generalized 3Hz spike, normal range for healthy people is 1-30 Hz
  • precipitated by hyperventilation
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9
Q

Generalized seizures

Tonic/Atonic Seizures (nonconvulsive)
* What is it?

A

partial or complete loss of muscle tone that may last less than 15 seconds

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10
Q

Generalized seizures

Myoclonic:
* What does the pt present as?
* What is usually preserved?

A
  • high amplitude, rapid lightning-like jerks that cause elevation/flexion of the upper limbs at the shoulder and at the elbow
  • consciousness is usually preserved
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11
Q

Generalized seizures

Generalized tonic-clonic
* What does the pt present with?
* Associated with what?

A
  • tonic extension or flexion of the limbs followed by larger amplitude clonic movements that get slower
  • associated with bladder loss and followed by post-ictal state
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12
Q

Psychogenic nonepileptic seizure (PNES)
* Referred to as what?
* Formally known as what?

A
  • referred to as PNES – non epileptic events resembling seizures or syncopal attacks.
  • formally known as pseudo seizures or hysterical seizures.
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13
Q

Psychogenic nonepileptic seizure (PNES)
* Episodes of what?
* What is normal?

A
  • episodes of movement, sensation, or behaviors that are like epileptic seizures but do not have a neurologic origin; rather, they are somatic manifestations of psychologic distress
  • EEG is normal during the attacks
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14
Q

Focal seizure ⭐️⭐️
* Not accompanied by what?
* Originates where?
* Broken down into what?

A
  • Not accompanied by an impairment of consciousness.
  • Originates in a specific area of the brain.
  • Broken down into two groups – focal aware and focal impaired awareness seizures.
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15
Q

Focal seizure ⭐️⭐️
* What are the sxs of focal aware group?

A
  • remains fully aware and conscious
  • unusual sensations such as tingling, visual disturbances, involuntary jerking of arm or leg
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16
Q

Focal seizure ⭐️⭐️
* What are the sxs of focal impaired awareness seizures?

A
  • persons LOC is altered or impaired, confused and dazed
  • repetitive movements like lip-smacking, chewing or hand movement
  • after event, memory loss is common
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17
Q

In a patient experiencing a focal motor seizure, what direction would the head turn?

A

opposite side of seizure

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18
Q

Status Epilepticus
* Defined as what? (2)
* Status epilepticus is what?

A

Defined as either:
* a continuous seizure that lasts for 5 minutes or longer, OR
* two or more sequential seizures within 30 minutes without full recovery of consciousness between seizures

Status epilepticus is a medical emergency and should be treated aggressively

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19
Q

Seizure Diagnosis ⭐️⭐️
* There is no what?
* What labs can be done?
* What is preferred?

A
  • There is no single test to diagnose seizures or epilepsy
  • accurate history and seizure description are most important
  • blood tests: Complete Blood Count (CBC), electrolytes, liver and renal function tests are sometimes utilized
  • Noncontrast CT scan of the head is preferred for evaluation of acute seizure:
    * Remember if the patient comes in with new seizure in the ER – he needs a CT scan to help identify the cause, hemorrhage, tumor, other abnormalities need to be ruled in/out
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20
Q

Seizure Diagnosis ⭐️⭐️
* What is preferred for further evaluation or in non-emergent situations?
* What testing is prefered when wake and sleep?
* when seizures are difficult to diagnose or control, what can be used?

A
  • Magnetic Resonance Imaging (MRI) is preferred for further evaluation or in non-emergent situations
  • Electroencephalograms (EEG), preferably wake and sleep – can be up to 72-hour testing
  • when seizures are difficult to diagnose or control, continuous video-EEG monitoring can be utilized to confirm the diagnosis
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21
Q
A
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22
Q

First Unprovoked Seizure⭐️
* What is required for the dx of epilepsy
* Adults presenting with an unprovoked first seizure should be informed about what?

A
  • at least two unprovoked seizures are required for diagnosis of Epilepsy
  • Adults presenting with an unprovoked first seizure should be informed that the chance for a recurrent seizure is greatest within the first 2 years after a first seizure
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23
Q

First Unprovoked Seizure⭐️
* Clinicians should also advise such patients that clinical factors associated with an increased risk for seizure recurrence include what?

A

Clinicians should also advise such patients that clinical factors associated with an increased risk for seizure recurrence include a prior brain insult such as a stroke or trauma, an EEG with epileptiform abnormalities, a significant brain-imaging abnormality, or a nocturnal seizure.

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24
Q

First Unprovoked Seizure⭐️⭐️⭐️⭐️
* What is likely to reduce the risk for a seizure recurrence in the 2 years after a first seizure?

A

Immediate anti-epileptic drug (AED) therapy, as compared with delay of treatment pending a second seizure, is likely to reduce the risk for a seizure recurrence in the 2 years after a first seizure.
* Better to treat early then wait for another seizure to occur

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25
Q

First Unprovoked Seizure Treatment
* Generally accepted principle that when a patient with a first seizure suffers 1 or more ensuing seizures, what should be initiated?
* For adults with unprovoked first seizure: immediate what needs to be done?

A
  • Generally accepted principle that when a patient with a first seizure suffers 1 or more ensuing seizures, an AED should be initiated because the risk for, yet additional seizures is very high (57% by 1 year and 73% by 2 years)
  • For adults with unprovoked first seizure: immediate AED reduce risk of seizure recurrence to approx. 35% over the next 2 years
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26
Q

Seizure Management ⭐️⭐️
* What do you need do for acute treatment? (test and medications do you need to give)

A

Fingerstick glucose testing – if proven hypoglycemia, IV dextrose should be provided.

First line - IV Benzodiazepam (Lorazepam) initially

Initial treatment failure occurs due to:
* Using inadequate initial doses due to concern for respiratory compromise
* Waiting too long to repeat benzodiazepam doses and advancing to second line agents

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27
Q

Seizure Management: Urgent control therapy⭐️⭐️
* When do you need to move to second line treatment? What are the examples (3)

A

If after 20 minutes they continue, two adequate doses of benzodiazepines and no correctable underlying etiology is found then move to SECOND LINE treatment.

Second line treatment:
* Levetiracetam (Keppra)
* Phenytoin (Dilatin)
* Valproate sodium (Depakene) – used if patient has allergy to phenytoin

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28
Q

Seizure Management
* What are the three goals?
* Clinicians should assist in what?

A

Management is focused on three main goals:
* controlling seizures
* avoiding treatment side effects
* maintaining or restoring quality of life

Clinicians should assist in empowering patients with epilepsy to lead lifestyles consistent with their capabilities.

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29
Q

Seizure Management - Maintenance
* Initial treatment is what?
* Consideration is based upon what?

A
  • Initial treatment is monotherapy
  • Consideration is based upon combination of drug, seizure, patient specific factors (gender, age, cost, availability of the medications)
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30
Q

Seizure Management - Maintenance
* What are the factors to consider? (4)

A
  • considering drug interaction limitations that may make the seizure medication less effective
  • hepatic and renal diseases impact the choice of seizure therapies (metabolized by the liver or excreted by the kidneys)
  • women in childbearing age: teratogenic effects of seizure medications
  • monitor mood and suicidality with AEDs
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31
Q

Patient Counseling - Seizures
* Counseling patients about what?
* Emphasize the importance of what?
* Discuss what?

A

Counseling patients about their risks for seizure recurrence and options for management.
* 70% of patients with proper diagnosis and treatment of epilepsy could live seizure free

Emphasize the importance of compliance with seizure medications

Discuss driving and operating machinery

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32
Q

Review of Correlations to Neurological Exam: Pearls
* previous head injury →
* limb twitch/ incontinence →
* sudden collapse →

A

previous head injury → chronic subdural hematoma

limb twitch/ incontinence → epilepsy

sudden collapse → hemorrhage
* Intracerebral Hemorrhage (ICH)
* Subarachnoid Hemorrhage (SAH)

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33
Q

Review of Correlations to Neurological Exam: Pearls
* Mass lesion →
* Diabetes →
* Alcoholism/ Drug Abuse/Psychiatric illness →
* Viral Infection →

A
  • Mass lesion → Malignancy
  • Diabetes → Hypo / Hyperglycemia
  • Alcoholism/ Drug Abuse/Psychiatric illness → Drug overdose/toxicity
  • Viral Infection → Encephalitis
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34
Q

Multiple Sclerosis – General Characteristics
* Multiple sclerosis is the most common what?
* What is involved?

A
  • Multiple sclerosis is the most common immune mediated inflammatory demyelinating disease of the central nervous system.
  • Inflammation and demyelination primarily involves the white matter of the spinal cord.
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35
Q

Multiple Sclerosis – General Characteristics ⭐️⭐️
* Patients with MS typically follow what?
* Damage is what?
* What may act act as a precipitant?

A
  • Patients with MS typically follow either a relapsing-remitting pattern of episodes or a primary progressive course.
  • Damage is random and asymmetric.
  • A viral infection may act as a precipitant.
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36
Q

Multiple Sclerosis – General Characteristics
* When does disease begin?
* Disease is most common in who?

A
  • Disease often begin between 20 and 30 years of age.
  • Disease is most common in women.
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37
Q

Multiple Sclerosis – General Characteristics ⭐️⭐️
* Etiology is what?
* Thought to be what?

A
  • Etiology is unknown, immune or viral factors are suspected but unproven causes.
  • Thought to be multifactorial in origin, with both environmental and genetic factors play a role.
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38
Q

Multiple Sclerosis – Clinical Features
* Onset of MS symptoms usually occurs when? ⭐️
* Initial sxs often relate to what?
* Weakness where? ⭐️
* May complain of what? ⭐️

A
  • Onset of MS symptoms usually occurs over several days and is seldom sudden.
  • Initial symptoms often relate to motor dysfunction.
  • Weakness in the legs, and less commonly, their arms.
  • May complain of foot drop, causing difficulty walking, or causing them to trip on sidewalks or curbs.
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39
Q

Multiple Sclerosis – Clinical Features
* Weakness and stiffness in MS can reflect what?
* Also have what type of sxs?
* May also complain of what? ⭐️

A
  • Weakness and stiffness in MS can reflect compromise of the corticospinal tract.
  • Also have sensory symptoms, such as tingling, pins and needle sensation, numbness, or a sensation of a band around the torso.
  • May also complain of sensations of electricity running down their spine, sometimes extending down into their limbs, aka Lhermitte sign.
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40
Q

Multiple Sclerosis - Types
* What is the clinically isolated syndrome?
* What is relapsing remitting MS? ⭐️

A
  • Clinically isolated syndrome — patient with a first clinical attack, difficult to predict whether a given individual will develop MS following symptom onset. It places the patient at risk for further relapses.
  • Relapsing-remitting MS: the most common type, characterized by sudden onset of neurological symptoms that usually last several weeks and then resolve, often leaving few or no deficits.
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41
Q

Multiple Sclerosis - Types
* What is the secondary progessive syndrome?
* What is primary progessive MS? ⭐️

A
  • Secondary Progressive Syndrome- gradual worsening of an initially remitting relapsing syndrome. Fewer and less complete remissions after each attack.
  • Primary progressive MS —insidious neurological progression of neurological disabilities suggestive of primary progressive MS (no acute relapses)
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42
Q

Diagnosis of Multiple Sclerosis
* One or more of what on imagining is characterisitic of MS?
* Two or more what?

A

One or more hyperintense T2 (type of MRI image) lesions characteristic of MS in one or more of the periventricular, cortical, or infratentorial areas
* Areas of demyelination in the brain/spinal cord that appear on T2 MRI sequence films

Two or more hyperintense T2 lesions in the spinal cord.

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43
Q

Diagnosis of Multiple Sclerosis
* If a patient has symptoms reflecting of what should be suspected of MS? ⭐️

A

If a patient has symptoms reflecting two or more separate brain lesions over TIME, and this is confirmed on MRI brain imaging, SPACE, then MS should be suspected

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44
Q

Diagnosis of Multiple Sclerosis
* “Space and Time” criteria ensures what?

A

“Space and Time” criteria ensure that the diagnosis of MS is based on a pattern of disease activity over time and affecting multiple parts of the CNS, rather than a single event or lesion. This helps distinguish MS from other neurological disorders and confirms the chronic, progressive nature of the disease.

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45
Q

Diagnosis of Multiple Sclerosis
* What is in CSF? ⭐️
* What sign is present on imaging? ⭐️

A

Presence of CSF-specific oligoclonal bands/Myelin Basic Protein
* Oligoclonal bands are present in up to 90% of MS patients.

Dawson Fingers if present confirm diagnosis
* Ovoid lesions extending from lateral ventricles into deep cerebral white matter

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46
Q

Diagnosis of Multiple Sclerosis
* What testing can be done?

A

Autoantibody testing — Testing for the aquaporin-4 (AQP4) IgG serum autoantibody and the myelin oligodendrocyte glycoprotein IgG autoantibody (MOG-IgG) are indicated for patients presenting with acute central nervous system demyelination when clinical, imaging, or laboratory features are atypical of MS.

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47
Q

Pathogenesis of Multiple Sclerosis ⭐️
* Inflammation of what? What can be seen?

A

Inflammation in conjunction with blood-brain-barrier disruption, characterized by gadolinium enhancement on MRI, is seen in the early stages of most demyelinating lesions in patients.
* lesions are bright on MRI and seen in the corpus callosum and periventricular regions.

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48
Q

Pathogenesis of Multiple Sclerosis ⭐️
* What are typically seen on histopathologic samples?
* What is present in CSF?
* MS is a clinical diagnosis that can be supported mainly by what?

A
  • Inflammatory T cells, B cells, and macrophages are typically seen on histopathologic samples
  • The presence of immunoglobulin G (IgG) and immunoglobulin M (IgM) and oligoclonal bands in the cerebrospinal fluid (CSF).
  • MS is a clinical diagnosis that can be supported mainly by MRI of the brain. CSF investigations can be supportive.
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49
Q

Treatment of Multiple Sclerosis
* What do you need to give? ⭐️

A

Corticosteroids may assist maximal recovery from acute exacerbations. High dose IV corticosteroids often are used in the setting of optic neuritis.
* has not been shown to decrease the risk of future attacks or change the course of the disease but are for treating current symptoms

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50
Q

Treatment of Multiple Sclerosis
* Plasma exchange can give benefit to what?
* Sxs therapy for MC can include?

A

Plasma exchange can give benefit to patients with acute exacerbations who do not respond to high dose IV corticosteroids.
* must be used on a chronic, ongoing basis.

Symptomatic therapy for MS can include physical, emotional rest, physical therapy as well.

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51
Q

Treatment of MS Exacerbations ⭐️
* What are the options? (3)
* What is the txt for patients with severe neurological deficits?

A

Methyl-prednisolone (Solumedrol): High doses

Oral Prednisone with or without a taper.

ACTH Adrenocorticotropic Hormone IM administration:
* stimulates the adrenal glands to produce corticosteroids which have potent anti-inflammatory properties

Plasma exchange treatments for patients with severe neurological deficits

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52
Q

What are the areas of examination for Neuromuscular Disorders?(6)

A
  • Cranial Nerves
  • Motor Testing
  • Sensory Testing
  • Reflexes
  • Gait and Coordination
  • Skin Examination
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53
Q

Cerebral Palsy ⭐️
* What is it? What is it believed to be the result of ?
* Clinical features are widely varied and include what?

A
  • A chronic impairment of muscle tone, strength, coordination, or movements. It is believed to result from cerebral injury before birth, during delivery, or in the perinatal period.
  • Clinical features are widely varied and include spasticity, lack of coordination, fine motor difficulties, ataxia, lethargy, hypotonia or dystonia. Spasticity being the most prominent.
54
Q

Cerebral Palsy ⭐️
* What does the physical exam reveal?(6)

A

Physical exam reveals hyperreflexia, hypotonia, microcephaly, limb length discrepancies, cataracts and congenital heart disease.

55
Q

Cerebral Palsy⭐️
* What is one of the first signs?
* What is required for imaging?

A
  • One of the first signs that may lead to suspicion of CP is a delay in reaching developmental milestones. This could include delays in rolling over, sitting, standing, or walking. Parents or pediatricians may notice these delays during routine check-ups, which can prompt further investigation.
  • MRI and genetic testing is required to exclude other conditions
56
Q

Cerebral palsy
* What is the txt?
* What is often required for txt?

A
  • Treatment is supportive, with the goal of attaining maximum function and potential in physical, occupational, and speech ability.
  • Pharmacological treatment of spasticity and seizures often required.
57
Q

Myopathy
* Myopathy refers to what? ⭐️

A

Myopathyrefers to a clinical disorder of the skeletal muscles. Abnormalities of muscle cell structure and metabolism that leads to various patterns of weakness and dysfunction.

58
Q

Myopathy
* Cardiomyopathy:
* Inflammatory/autoimmune immune myopathy:

A
  • Cardiomyopathy: involve cardiac muscle fibers, resulting in a hypertrophic or dilated cardiomyopathy
  • Inflammatory/ autoimmune myopathy:Characterized by inflammation of muscles and peri-muscular space (Polymyositis, dermatomyositis, sarcoidosis, lupus, and rheumatoid arthritis)
59
Q

Myopathy
* Toxic myopathy:
* Endocrine myopathy:

A
  • Toxic myopathy:This occurs when a toxin (ETOH, Cocaine. Medications (Antimalaria Chloroquine, Statins, Antipsychotic, Chemotherapy agents..) impair muscle structure and function.
  • Endocrine myopathy:Hormones interferes with muscle function such as Hypo/Hyperthyroid, Diabetes, Cushing’s syndrome.
60
Q

Myopathy
* Infectious myopathy:
* Myopathy secondary to electrolyte imbalance:

A
  • Infectious myopathy:Viral Myositis, HIV, Hepatitis… Necrotizing fasciitis, Polymicrobial related to aerobic and anaerobic bacteria, monomicrobial like Staph. Aureus and hemolytic strep.
  • Myopathy secondary to electrolyte imbalance:Electrolyte problems, such as excessively high or low potassiumlevels ( Hypokalemic Periodic Paralysis) Hypoglycemia, Hypocalcemia, Hypomagnesemia
61
Q

myopathy
* Hereditary/ Congenital: Muscular dystrophies:
* Traumatic Rhabdomyolysis:

A
  • Hereditary/ Congenital: Muscular dystrophies: Limb Girdle muscular dystrophy, muscular dystrophy, Myotonic dystrophy, Occulopharyngeal muscular dystrophy
  • Traumatic Rhabdomyolysis: breakdown of skeletal muscle tissue due to physical trauma.
62
Q

Clinical Presentation for Myopathy ⭐️
* What are the sxs?

A
  • Muscle weakness, impaired function in activities of daily life, muscle pain and tenderness.
  • Symmetric proximal muscle weakness
  • Malaise, fatigue
  • Absence of sensory complaints or paresthesia
  • Deep tendon reflexes (DTRs) may be diminished/absent in hypokalemic paralysis
  • Very late findings: Atrophy and hyporeflexia
63
Q

Diagnostic Tests/ Management of Myopathies
* What are the labs?
* What are teh serologic tests?

A
  • Laboratory Studies – chemistry and urinalysis, elevation of plasma muscle enzymes, Creatinine Kinase (CK), Lactic Dehydrogenase, RBC in the urine is suggestive of myoglobinuria.
  • Serologic Tests – ANA, Antibodies against extractable antinuclear antigen, Antineutrophil Cytoplasmic Antibody (ANCA) for vasculitis and other autoimmune diseases.
64
Q

Diagnostic Tests/ Management of Myopathies
* What are the electophysiologic studies? ⭐️
* What type of biospy?
* Genetic Testing for who?

A
  • Electrophysiologic Studies – Nerve conduction and Electromyographic (EMG) Studies
  • Muscle Biopsy
  • Genetic Testing for patients with possible heritable myopathies and muscular dystrophies.
65
Q

Treatment of Myopathy
* Treatment of myopathies is what? (general)
* Treatment of Myopathy can be treated with what?

A
  • Treatment of myopathies is multidisciplinary and depends on the type of myopathy.
  • Certain types of myopathies can be treated withsteroid, immune suppressing therapy and IVIG.
66
Q

Treatment of Myopathy
* Most myopathyies require the use of what?
* Surgical treatment of what?

A
  • Most myopathies require the use of supportive services, such as physical and occupational therapy, pulmonary medicine, cardiology, dietary management, and speech/swallowing therapists.
  • Surgical treatment of spine and limb deformities is used in long-standing cases.
67
Q

Myasthenia Gravis ⭐️
* What is it?
* what is the weakness pattern?

A
  • Myasthenia gravis is an autoimmune disease where T-cells, B-cells produce antibodies which block and destroy Acetyl-Choline receptors faster than new ACh receptors can be synthesized.
  • Proximal weakness is weakness predominantly affecting the axial muscles, deltoid and hip flexors.
68
Q

Myasthenia Gravis ⭐️
* How does weakness present throughout the day?
* Fatigability of the muscles with recovery to the baseline strength after what?

A
  • Weakness quickly fluctuates and worsens throughout the day.
  • Fatigability of the muscles with recovery to the baseline strength after a short period of rest.
69
Q

Myasthenia Gravis
* What are some of the ocular symptoms?⭐️
* What is the most common weakness?
* What is the key clinical exam finding? ⭐️

A
  • Will develop ocular symptoms – ptosis can be bilateral or unilateral and can shift quickly from one eye to the other.
  • Limb weakness is most common – presents as having difficulty raising arms above the head, having difficulty climbing up the stairs, and rising from a chair.
  • Key clinical exam finding is fatigability causing increased weakness with use of the muscle.
70
Q

Diagnosis of Myasthenia Gravis ⭐️
* Clinical dx is established by what? What is normal?

A

Clinical diagnosis is established by history and exam.
* reflexes and sensation are NORMAL

71
Q

Diagnosis of Myasthenia Gravis ⭐️
* What is the clinical test? What are the results?

A

Clinical test in patients with ptosis is the ice test – ice is placed over the eyelid for 2 minutes – if ptosis improves after removing the ice, diagnoses can be made.
* cooling improves neuromuscular junction transmission, heat worsens it.
* hence Myasthenia Gravis is worse in the summer

72
Q

Diagnosis of Myasthenia Gravis⭐️
* What are teh serologic test?

A

Serologic tests for autoantibodies, Ach receptor antibodies
* three antibodies – binding, blocking and modulating
* need a positive test for one of these antibodies

73
Q

Diagnosis of Myasthenia Gravis
* What studies should be done?
* What imaging needs to be done?

A

Electrophysiologic studies such as repetitive nerve stimulation and single fiber EMG studies.

CT or MRI of the chest/mediastinum looking for Thymoma (75% will have it)
* Tumor of the thyroid

74
Q

Diagnosis of Myasthenia Gravis
* What test can be done?

A

Tensilon Test – agent given which temporarily inhibits the enzyme Ach – with more present, makes more acetylcholine available to stimulate the receptors on muscle cells and temporarily improve muscle strength in MS.

75
Q

Treatment of Myasthenia Gravis
* Myasthenia Gravis is a chronic but treatable disease, most patients can what?

A

Myasthenia Gravis is a chronic but treatable disease, most patients can achieve sustained remission of symptoms and full functional recovery.

76
Q

Treatment of Myasthenia Gravis
* What are the four different treatment options?

A
  • Pyridostigmine: oral acetylcholinesterase inhibitor as initial therapy for mild to moderate Myasthenia Gravis – only treats symptoms.
  • Glucocorticoids: Prednisone is used.
  • Immunosuppressive therapy: immunosuppressive agents are the mainstay for treatment.
    IVIG, Plasma Exchange for myasthenia crisis
77
Q

Lambert-Eaton Myasthenic Syndrome ⭐️
* Is a disorder of what?
* 50% of cases are associated with what?
* LES can be what type of syndrome?

A
  • Is a disorder of reduced acetylcholine release from the presynaptic nerve terminals.
  • 50% of cases are associated with malignancy, mainly small cell lung cancer.
  • LEMS can be a paraneoplastic syndrome – disorder that occurs because of an immune system response to a cancerous tumor elsewhere in the body
78
Q

Lambert-Eaton Myasthenic Syndrome ⭐️
* It is associated with slowly progressive what?

A

It is associated with slowly progressive proximal muscle weakness mostly in the legs; deep tendon reflexes are typically depressed or absent
* lower limb weakness > upper limb weakness

79
Q

Lambert-Eaton Myasthenic Syndrome⭐️
* It is associated with what?
* What is a key clinical finding?
* What is the tx?

A
  • It is associated with dry mouth and other autonomic symptoms.
  • Key clinical finding is that a patient may regain strength with repeated activation of muscles.
  • Tx with Firdapse (Amifamridine)-> If associated with tumor, LEMS may go away with tumor treatment
80
Q

Neuropathy
* What is Mononeuropathy
* What is Mononeuritis Multiplex?

A
  • Mononeuropathy: focal involvement of a single nerve due to trauma or compression or entrapment (carpal tunnel syndrome, CTS)
  • Mononeuritis Multiplex: simultaneous or sequential involvement of non-contiguous nerve trunks. Compression neuropathies, systemic vasculitis.
81
Q

Neuropathy
* What is peripheral neuropathy?⭐️
* What is radiculopathies? ⭐️

A
  • Peripheral Neuropathy: Disorder of the peripheral nervous system including radiculopathies and mononeuropathies.
  • Radiculopathies: Cervical, thoracic and lumbar
82
Q

Neuropathy
* What is Gillian Barre Syndrome? ⭐️
* What is Complex Regional Pain Syndrome?

A
  • Gillian Barre Syndrome: Acute Polyradiculoneuropathy disorder
  • Complex Regional Pain Syndrome: previously called(Reflex Sympathetic Dystrophy, RSD)
83
Q

Polyneuropathy
* What is it?

A

Chronic axonal polyneuropathies which can be due to diabetes or uremia are the most common of polyneuropathies’.

84
Q

Polyneuropathy
* What is acute axonal polyneuropathy?
* What is chronic inflammatory demyelinating polyneuropathy?

A
  • Acute axonal polyneuropathy: affects multiple peripheral nerves and is characterized by rapid onset and damage to axons, the long thread-like parts of nerve cells.
  • Chronic Inflammatory demyelinating polyneuropathy were weakness and numbness often present simultaneously.
85
Q

Polyneuropathy
* What is Acute demyelinating polyneuropathy?

A

Gillian-Barre syndrome which starts predominantly in motor nerve fiber with weakness rather sensory loss, typically the earliest signs of the disease, then dysesthesia in arms or legs with gait difficulty.

86
Q

What are the clinical presentations of neuropathies? (5)

A
  • Symmetric distal sensory loss
  • Burning sensation
  • Weakness is distal
  • Walking difficulties /balance problem
  • Slowly progressive presentation of sensory loss, chronic pain with numbness and burning sensation and pain in the feet with mild gait abnormality this progress is to mild weakness of the lower legs and hand symptoms the resulting in the classic glove and stocking distribution of sensory loss
87
Q

Diagnostic Tests in Polyneuropathies
* In addition to the history of neuropathy itself the patient should be asked about what?
* Diagnostic evaluation with atypical features as what?

A
  • In addition to the history of neuropathy itself the patient should be asked about recent viral illnesses, systemic illnesses, new medications, exposure to solvents or heavy metals or other potential toxins such as alcohol use and family history of neurologic disease.
  • Diagnostic evaluation with atypical features as asymmetry, motor predominance, acute onset prominent autonomic involvement, severe or rapidly progressive symptoms, and sensory ataxia.
88
Q

Diagnostic Tests in Polyneuropathies
* What study can be done? ⭐️
* What are the labs? ⭐️
* What biopsy can be done?

A
  • Electrodiagnostic testing with EMG and or nerve conduction study as the initial diagnostic procedure for patient with suspected polyneuropathy when there is no clear etiology/atypical features.
  • Laboratory testing : blood glucose, serum B12 level, with and without Homocysteine, serum protein electrophoresis SPEP and thyroid function tests.
  • Nerve biopsy is useful for vasculitis or amyloidosis
89
Q

Treatment of Polyneuropathy
* What is the treatment?

A

Treatment with the underlying disease
* tight control of blood sugar to maintain nerve function
* thyroid replacement therapy for hypothyroidism
* eliminating toxins and treating vasculitis which is inflammation of the blood vessels

90
Q

Treatment of Polyneuropathy
* How can you reduce pain?

A

Alleviation of symptoms related to the illness: to reduce pain associated such as with Pregabalin, tricyclic antidepressants.

91
Q

Gillian Barre Syndrome (GBS) ⭐️
* What is it?
* Whatare teh cardinal signs?

A
  • Acute illness causing a rapidly progressive polyneuropathy with weakness or paralysis.
  • Cardinal signs for GBS are progressive, mostly symmetric ascending muscle weakness with depressed or absent tendon reflexes (90%)
92
Q

Gillian Barre Syndrome (GBS) ⭐️
* Primary affects what?
* What is common?
* Sensory fibers are affected, resulting in what?
* How does GBS progress?

A
  • Primarily affects motor roots and produces lower motor neuron symptoms – muscle weakness, flaccid paralysis, and hyporeflexia.
  • Upper cervical root involvement and respiratory paralysis are common.
  • Sensory fibers are affected, resulting in paresthesia.
  • GBS usually progresses over a period of two weeks.
93
Q

Pathogenesis of GBS
* GBS is thought to be the result of what?⭐️
* Symptoms develop how?

A
  • GBS is thought to be the result of immune response to a preceding infection which cross-reacts with peripheral nerves, such as acute influenza.
  • Symptoms develop 1-4 weeks following infection
94
Q

Pathogenesis of GBS
* The immune response can be directed towards what?
* What is the MCC of GBS?

A

The immune response can be directed towards the myelin or axon of peripheral nerves resulting in demyelinating and axonal form of GBS.
* Campylobactor jejuni infection is the most common cause of GBS

95
Q

Pathogenesis of GBS
* What is associated with what?
* What may also trigger it?

A
  • CMV, EBV, HIV, Zika virus have been associated with GBS
  • Immunization, Trauma, surgery may trigger GBS
96
Q

Diagnostic Tests in GBS
* The typical finding with lumbar puncture is what?
* Electrodiagnostic study such as what?
* What antibody is associated with GBS?

A
  • The typical finding with lumbar puncture is elevated cerebrospinal fluid protein with normal white blood cells.
  • Electrodiagnostic study such as EMG and nerve conduction study may show evidence of acute polyneuropathy with predominantly demyelinating features or predominantly axonal features in acute motor or sensory neuropathies.
  • GQ1B antibody associated with Miller Fisher variant of GBS
97
Q

Management of GBS
* What is extremely important in? ⭐️
* What warrant ICU monitoring?

A
  • Supportive care is extremely important in GBS 30% of patients who develop neuromuscular respiratory failure requiring mechanical ventilation.
  • Autonomic dysfunction warrant ICU monitoring
98
Q

Management of GBS
* What needs to be monitored?
* What do you need to listen to?
* Disease modifying therapies are what?

A
  • Cardiac and blood pressure monitoring
  • Abdominal auscultation to monitor for ileus
  • Disease modifying therapies are plasma exchange and IVIG intravenous immunoglobulin
99
Q

Amyotrophic Lateral Sclerosis (ALS)
* What is it? What is an example? ⭐️⭐️

A

ALS is a relentlessly progressive, presently incurable, neurodegenerative disorder that causes muscle weakness, disability, and eventually death based on the involvement of upper and lower motor neurons.
* Example would be progressive upper extremity weakness, spasms in calves, muscle twitching.

Referred to as Lou Gehrig’s Disease

100
Q

Amyotrophic Lateral Sclerosis (ALS)
* What is the first sign? ⭐️

A

Muscle weakness & atrophy in hands is usually the 1st sign

101
Q

Pathophysiology of ALS
* ALS is characterized by what? What can be preserved? ⭐️
* What break downs and die?

A

ALS is characterized by motor neuron degeneration and death with gliosis (reactive changes) replacing lost neurons.
* Sensory, proprioception and coordination may be preserved

Cortical motor cells (pyramidal and Betz cells) in the motor cortex, anterior horn cells of the spinal cord and lower cranial motor nerves in the brainstem breakdown and die.

102
Q

Pathophysiology of ALS
* This gliosis results in what? What might be unremarkable?
* What happens to the spinal cord?

A

This gliosis results in the bilateral white matter changes sometimes seen in the brain magnetic resonance imaging (MRI) of patients with ALS.
* MRI of the brain and cervical spinal cord may be unremarkable

The spinal cord becomes atrophic.

103
Q

ALS Diagnosis
* Most common form of what? What sign is present? ⭐️

A

ALS is the most common form of motor neuron disease.
* Hoffman sign

104
Q

ALS Diagnosis
* There is no single diagnostic test that can confirm or entirely exclude the diagnosis of motor neuron disease, but a clinical standard includes: (2)
* What is the txt?

A
  • Evidence of upper motor neuron and lower motor neuron signs and spare sensory neurons.
  • Progression of disease (no relapsing or remitting), and the absence of an alternative explanation.
  • Treatment is supportive
105
Q

Hyperkinesia Definitions
* What is tremor?

A

Tremor: rhythmic oscillating movement across a joint, representing contraction of agonist & antagonist muscles

106
Q

Hyperkinesia Definitions
* What is rest tremor? ⭐️
* What os postural temor? ⭐️
* What is action tremor?

A
  • Rest tremor: may involve arms, legs, or jaw (e.g. Parkinson’s disease)
  • Postural tremor: maximal when patient holds extremity suspended against gravity (e.g. Essential Tremor) (stretched out hands)
  • Action tremor: maximal during movement of a limb (e.g. Cerebellar Tremor) finger to nose
107
Q

Hyperkinesia Definitions
* What is chorea? ⭐️
* What is dystonia?
* What is myoclonus?
* What is tic?

A
  • Chorea: A state of excessive, spontaneous movements that are non-repetitive of moderate amplitude & speed with a flowing “dance-like” quality
  • Dystonia: Sustained abnormal muscle contraction producing pain &/or a positional deformity (torticollis)
  • Myoclonus: Lightening-like muscle jerks that may involve any body part, often repetitive
  • Tic: Abnormal repetitive stereotypic movements (motor) or sounds (vocal) that usually begin in childhood or adolescence.
108
Q

Movement Disorders
* One can make a diagnosis with what? ⭐️
* If the tremor is slow, it is either what?
* If the tremor is rapid, it is what?

A
  • One can make a diagnosis with 90% certainty simply by watching the tremor.
  • If the tremor is slow, it is either a cerebellar tremor, which becomes worse with intention, or a parkinsonian tremor, which is worse at rest.
  • If the tremor is rapid, it is primary, chemical.
109
Q

Essential Tremor
* Common or rare?
* slow or fast progressing ? What is the inheritance?
* Postural tremor affects what?

A

Most common cause of tremor.

Slowly progressive disorder
* Autosomal dominant inheritance – primary

Postural tremor affecting hands most often.

110
Q

Essential Tremor
* impairs what?
* Exacerbated by what?

A

Impaired job performance and embarrassment.

Exacerbated by anxiety, emotional stress and caffeine.

111
Q

Essential Tremor
* Dx based on what?
* If the tremor appears how?
* What are the labs?

A

Diagnosis based on history and physical

If the tremor appears when holding a posture, then it is essential tremor

Labs: thyroid function tests – r/o hyperthyroidism

112
Q

Essential Tremor
* What is the txt?
* Most striking and diagnostic feature of essential tremor is said to be what?

A

Treatment:
* Propranolol (Inderal) – sexual and depressive side effects
* Deep brain stimulation
* Botox into muscle that are responsible of tremor

Most striking and diagnostic feature of essential tremor is said to be the dramatic relief with alcohol.

113
Q

Parkinson’s Disease
* Parkinsonism is a clinical syndrome presenting with what?
* What is the most common form?

A
  • Parkinsonism is a clinical syndrome presenting with any combination of bradykinesia, rest tremor, rigidity, and postural instability.
  • The most common form is Parkinson disease (PD), a chronic, progressive disorder caused by degenerative loss of dopaminergic neurons in the brain.
114
Q

Parkinson’s Disease
* PD remains a clinical diagnosis, based on what?
* Parkinson disease is thought of as what?

A
  • PD remains a clinical diagnosis, based on the clinician’s ability to recognize its characteristic signs and associated symptoms, especially in the early stages.
  • Parkinson disease is thought of as a motor disorder, sensory systems are also affected. Loss of sense of smell is almost universal.
115
Q

Parkinson’s Disease – Signs and Symptoms ⭐️
* What is bradykinesia?
* What is rigidity?

A
  • Bradykinesia is defined as slowness of movement plus a decrease in amplitude/speed or progressive hesitations/halts as movements are continued.
  • Rigidity is a velocity-independent resistance (sometimes referred to as “cogwheel” or “lead-pipe resistance”) to passive movement of the major joints while the patient is in a relaxed position.
116
Q

Parkinson’s Disease – Signs and Symptoms ⭐️
* What is a resting tremor?
* What are other signs?
* Patients with parkinson have a difficulty time doing what?

A

Resting tremor is a 4 to 6 Hz tremor that is observed in a fully resting limb and is suppressed when initiating movement (pill-rolling).
* Is an early manifestation of Parkinson disease

Other signs: Masked faces (Hypomimia), Decreased Blink rate, Speech impairment (hypophonia).

Patients with Parkinson disease will have difficulty performing two tasks as the same time - “tap the top of your head and stick out your tongue to me”

117
Q

Parkinson’s Disease
* What are the red flag findings?

A
  • Early onset of, or rapidly progressing, dementia
  • Rapidly progressive course
  • Upper motor neuron signs
  • Cerebellar signs – ataxia
  • Urinary incontinence
  • Early symptomatic postural hypotension
  • Early falls
  • If the tremor is persistent only at rest, this almost guarantees the diagnosis.

Majority of cases of Parkinson disease are unknown.

118
Q

Treatment of Parkinson’s Disease
* When do you start with treatment? What is the medication?
* No treatment affects what?
* What are the physical measures?

A

Treatment is initiated when the patient’s quality of life is affected and usually consists of either:
* Carbidopa/Levodopa can help to alleviate the symptoms.

No treatment affects the degenerative process, symptomatic treatment is the mainstay of therapy.

Physical measures such as physical therapy, speech therapy, and exercise are important and have a major impact on their lives

119
Q

Restless Leg Syndrome
* What is it also called as?
* What is the pathophysio?

A

AKA Willis-Ekbom disease which affects up to 15% of population

Genetic predisposition has been described and occurs earlier in life
* related to disturbance of dopaminergic receptors and diminished brain iron stores

120
Q

Restless Leg Syndrome
* Chronic movement disorder in which patients have what?
* There is usually no what?
* When are sxs worst?

A

Chronic movement disorder in which patients have an irresistible urge to move their legs
* there is usually no pain, and urge is relieved by movement
* symptoms are usually worse at night causing sleep disturbance

121
Q

Restless Leg Syndrome
* What is the clinical presentation?

A
  • sensations such as crawling, creeping, pulling, itching, drawing, or stretching, all localized to deep structures rather than the skin
  • periodic leg movements (foot dorsiflexion) lasting 1-5 seconds and occurring every 20-40 seconds during sleep
122
Q

Restless Leg Syndrome
* how do you dx it?

A

Diagnosis: exam is usually normal, therefore historical points are essential
* EMG/blood work can be done to r/o other causes
* Polysomnography can quantify the frequency of leg movements
* iron studies should be done (typically low)

123
Q

Restless Leg Syndrome
* What is the txt?

A
  • vibration devices for feet/lower legs
  • avoid caffeine, dopamine blocking agents and antidepressants and replace iron
  • medications reserved for moderate symptoms (dopamine agonists)
124
Q

Tardive Dyskinesia
* What is it?

A

Involuntary and uncontrolled movement disorders stemming from dopamine receptor blockade (medication induced)

“Tardive – means late or after a long period of treatment”

125
Q

Tardive Dyskinesia
* Feelings of what?
* Involuntary muscle contractions such as what?
* Typically, in patients who are chronically on what?

A

Feeling of restlessness, accompanied by a constant desire to move.

Involuntary muscle contractions such as eye spasms, twisting of the head.

Typically, in patients who are chronically on typical antipsychotics (schizophrenia/bipolar)
* symptoms may persist even if medications are stopped

126
Q

Tardive Dyskinesia
* How do you dx it?

A

TD symptoms improve with movement (like parkinsonism)

Abnormal Involuntary Movement Scale (AIMS)
* baseline should be obtained prior to initiation of medication treatment

Get a CT or MRI to r/o other causes as they are usually normal in drug induced TD

127
Q

Tardive Dyskinesia
* What is the treatment?

A

avoid dopamine antagonists
* continued use of these maintain an increased or heightened state of dopamine receptors.
* persistent blockage and resulting neurochemical imbalance can make symptoms of TD worse
* Valbenazine approved in 2017
* Benztropine (Cogentin) has been used off-label

128
Q

Huntington’s Disease ⭐️
* When is the onset (age)
* What is the genetic pattern?

A
  • Age of onset ranges from childhood to the eighth decade but is most common in mid-life.
  • Autosomal dominant disorder, characterized by psychiatric disease.
129
Q

Huntington’s Disease ⭐️
* What are the three major symptoms?
* Variable flowing movements of what?
* What is the major manifestation of hyntington’s disease?

A
  • Three major symptoms – involuntary movements, changes in behavior and personality, and cognitive impairments.
  • Variable flowing movements of the limbs and trunk, reflexes that are brisk, gait that is rapid, wide based and uncoordinated.
  • Chorea is a major manifestation of Huntington’s Disease.
130
Q

Huntington’s Disease
* What is the patho?
* What is the txt (general)

A

The pathophysiology of HD is not fully understood, although it is thought to be related to toxicity of the mutant huntington protein involving putamen and caudate atrophy.

No known cure, treatment is symptomatic and remains supportive.

Clinical Medicine 4 (13 decks)

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