Clin Med Unit 3: Amyotrophic Lateral Sclerosis (ALS)

The degeneration of which cells is primarily responsible for the motor deficits observed in Amyotrophic Lateral Sclerosis (ALS)?

Which of the following clinical findings is indicative of upper motor neuron damage in a patient with suspected ALS?

A patient presents with progressive muscle weakness, fasciculations, and bulbar symptoms such as dysarthria and dysphagia. An electromyogram (EMG) shows acute and chronic denervation. Which of the following is the most likely diagnosis?

What is the primary mechanism of action of riluzole, a medication commonly used in the treatment of ALS?

Which of the following is a typical early symptom of Guillain-Barre Syndrome (GBS)?

A lumbar puncture in a patient with Guillain-Barre Syndrome (GBS) is most likely to reveal which of the following cerebrospinal fluid (CSF) findings after the first week of symptom onset?

Which of the following antecedent infections is most commonly associated with Guillain-Barre Syndrome (GBS)?

What is the primary treatment for severe, rapidly worsening Guillain-Barre Syndrome (GBS) affecting ventilation?

Bell's Palsy is characterized by paralysis of which cranial nerve?

A patient with Bell's Palsy is unable to wrinkle their forehead on the affected side. This finding helps differentiate Bell's Palsy from:

Which of the following is considered the first-line treatment for Bell's Palsy?

What is the primary pathophysiological mechanism in Myasthenia Gravis (MG)?

A patient with Myasthenia Gravis (MG) presents with ptosis and diplopia that worsen throughout the day. Which term best describes this fluctuating weakness?

The 'ice pack test' is used in the diagnosis of Myasthenia Gravis (MG). A positive test is indicated by:

Which class of medications is used for symptomatic treatment of Myasthenia Gravis (MG) by improving neuromuscular transmission?

Duchenne Muscular Dystrophy (DMD) is caused by a genetic mutation affecting which protein?

Which of the following clinical signs is characteristic of Duchenne Muscular Dystrophy (DMD) and indicates proximal muscle weakness?

What is the primary laboratory finding indicative of muscle damage in a patient with Muscular Dystrophy?

Eteplirsen, used in some cases of Duchenne Muscular Dystrophy (DMD), is what type of therapeutic agent?

Complex Regional Pain Syndrome (CRPS) is often characterized by:

Which of the following is a typical early finding in Complex Regional Pain Syndrome (CRPS)?

A bone scan in a patient with early Complex Regional Pain Syndrome (CRPS) is likely to show:

Which of the following is a non-pharmacological treatment strategy for Complex Regional Pain Syndrome (CRPS) focused on retraining the brain and motor function?

What is the primary difference in the distribution of symptoms between mononeuropathy and peripheral neuropathy?

Carpal Tunnel Syndrome is an example of:

Which of the following diagnostic studies is most useful in localizing the lesion in a patient with mononeuropathy?

A 'stocking-glove' distribution of sensory loss is characteristic of:

What is the most common cause of headache in children?

Which of the following headache characteristics is considered a 'red flag' in pediatric headaches, warranting further investigation?

In pediatric migraine management, which medication class is generally considered first-line for abortive treatment?

What is the characteristic skin finding associated with Sturge-Weber Syndrome?

Buphthalmos, a potential complication of Sturge-Weber Syndrome, refers to:

Intracranial leptomeningeal vascular anomalies and calcifications are commonly found in patients with Sturge-Weber Syndrome. These are typically located in which lobes of the brain?

Prenatal screening for Spina Bifida primarily involves:

Myelomeningocele, the most severe form of Spina Bifida, involves protrusion of:

Which of the following is a common early complication of Myelomeningocele requiring monitoring and potential intervention?

The most common cause of congenital hydrocephalus is:

Which of the following is a surgical management option for hydrocephalus?

Chiari Malformation Type 1 is characterized by the displacement of cerebellar tonsils below the:

Cerebral Palsy is best described as:

Spasticity, the most common motor impairment in Cerebral Palsy, is primarily due to damage in:

Charcot-Marie-Tooth disease (CMT) is primarily a disorder affecting:

The 'Champagne-glass deformity' in Charcot-Marie-Tooth disease (CMT) refers to:

Infant botulism is caused by the toxin produced by which bacteria?

Which of the following is a characteristic early symptom of Infant Botulism?

Spinal Muscular Atrophy (SMA) is characterized by the degeneration of:

What is the typical inheritance pattern of Spinal Muscular Atrophy (SMA)?

Poliomyelitis primarily targets which cells in the nervous system?

The primary mode of transmission for poliovirus is:

Tourette Syndrome is characterized by the presence of:

What is the typical age range during which Amyotrophic Lateral Sclerosis (ALS) manifests?

The diagnostic criteria for Amyotrophic Lateral Sclerosis (ALS) as of 2019 (Gold Coast criteria) require:

Which diagnostic finding is commonly observed on an Electromyogram (EMG) in patients with Amyotrophic Lateral Sclerosis (ALS)?

Which of the following complications is a primary concern in the management of Amyotrophic Lateral Sclerosis (ALS)?

What is the typical range for the median survival time after the onset of Amyotrophic Lateral Sclerosis (ALS) symptoms?

Which of the following best describes the function of noninvasive ventilation (NIV) in the management of Amyotrophic Lateral Sclerosis (ALS)?

What is the most common timeframe for the onset of Guillain-Barre Syndrome (GBS) symptoms following an antecedent infection?

In Guillain-Barre Syndrome (GBS), what is the characteristic pattern of weakness progression?

A patient presents with suspected Guillain-Barre Syndrome (GBS). Upon physical examination, which of the following reflex findings is most consistent with this diagnosis?

What is the primary concern when autonomic nerves are affected in a patient with Guillain-Barre Syndrome (GBS)?

What is a crucial evaluation to perform when managing a patient diagnosed with Guillain-Barre Syndrome (GBS)?

Which of the following Nerve Conduction Velocity (NCV) findings in a patient with Guillain-Barre Syndrome (GBS) indicates a poorer prognosis?

In Bell's Palsy, if vesicles are observed in the external ear canal, what additional diagnostic consideration should be undertaken?

What is the expected time course for maximum weakness and resolution in a typical case of Bell's Palsy?

When are antiviral medications typically considered in the treatment of Bell's Palsy?

Which autoimmune process is primarily responsible for the pathology observed in Myasthenia Gravis (MG)?

Which associated finding should prompt suspicion for underlying malignancy in older patients newly diagnosed with Myasthenia Gravis (MG)?

What is the primary purpose of performing a CT scan of the chest in a patient diagnosed with Myasthenia Gravis (MG)?

What adverse effect requires monitoring when initiating pyridostigmine for the treatment of Myasthenia Gravis (MG)?

What is the primary goal of symptomatic treatment with acetylcholinesterase inhibitors in Myasthenia Gravis (MG)?

The progressive muscle weakness seen in Muscular Dystrophies is due to mutations affecting what?

Genetic testing in patients with Muscular Dystrophy is conducted to:

Elevated creatine kinase (CK) levels are indicative of what process in muscular dystrophies?

In Duchenne Muscular Dystrophy, what clinical finding would be most indicative of proximal muscle weakness?

What causes the need for respiratory and cardiac monitoring in patients with muscular dystrophies?

Eteplirsen is a treatment being used in some cases of Muscular Dystrophy, and works by improving what?

Which of the following most accurately represents the underlying mechanism of Complex Regional Pain Syndrome (CRPS)?

After what event does Complex Regional Pain Syndrome (CRPS) usually occur?

What is the typical character of pain associated with Complex Regional Pain Syndrome (CRPS)?

What skin changes are commonly observed in a limb affected by Complex Regional Pain Syndrome (CRPS)?

What radiological finding on a bone scan is suggestive of Complex Regional Pain Syndrome (CRPS)?

What component is crucial to treatment for Complex Regional Pain Syndrome (CRPS)?

What is Buphthalmos?

Why give low dose aspirin when treating Sturge-Weber Syndrome?

What symptoms are commonly associated with myelomeningocele?

One feature of cerebral palsy is non-progressive, what is that?

The genetic testing associated with CMT looks for...

What are symptoms of Infant Botulism?

What is the only thing that Infant Botulism Immune Globulin can be used for?

A weak floppy baby has what?

In Amyotrophic Lateral Sclerosis (ALS), the degeneration of anterior horn cells directly results in:

Which of the following electrodiagnostic findings is most consistent with a diagnosis of Amyotrophic Lateral Sclerosis (ALS)?

What is the most common initial presentation of Amyotrophic Lateral Sclerosis (ALS)?

A patient with ALS is experiencing increasing dyspnea. What intervention is most appropriate to improve their quality of life and survival?

Which of the following symptoms is the MOST indicative of bulbar involvement in Amyotrophic Lateral Sclerosis (ALS)?

In a patient diagnosed with Guillain-Barre Syndrome (GBS), what clinical finding would warrant immediate assessment for respiratory support?

Which of the following cerebrospinal fluid (CSF) findings is MOST indicative of Guillain-Barre Syndrome (GBS) after one week of symptom onset?

A patient is diagnosed with Guillain-Barre Syndrome (GBS). What is the MOST important initial step in managing this patient?

What Nerve Conduction Velocity (NCV) finding suggests a poorer prognosis in a patient with Guillain-Barre Syndrome (GBS)?

Which of the following cranial nerves is affected in Bell's Palsy, resulting in facial paralysis?

A patient with Bell's Palsy is unable to close their left eye completely. Which intervention is MOST important to prevent complications?

Which condition should be suspected if a patient presents with Bell's Palsy and also has vesicles in the external ear canal?

In Myasthenia Gravis (MG), autoantibodies primarily target:

What is the purpose of performing a chest CT scan in a newly diagnosed patient with Myasthenia Gravis (MG)?

Why are patients with Myasthenia Gravis encouraged to avoid certain medications such as aminoglycosides and beta-blockers?

In Duchenne Muscular Dystrophy (DMD), the primary cause of muscle fiber instability and degeneration is a mutation affecting:

A young boy with Duchenne Muscular Dystrophy (DMD) uses his hands to 'walk' up his legs when rising from the floor. This maneuver is indicative of:

A patient with Complex Regional Pain Syndrome (CRPS) reports hypersensitivity to light touch in the affected limb. How should this be documented?

What is the MOST likely underlying mechanism contributing to complex regional pain syndrome (CRPS)?

Which therapy has shown the MOST promise in Complex Regional Pain Syndrome (CRPS) by helping to re-train the brain and motor function?

Which of the following is a key characteristic that differentiates tension headaches from migraines in children?

A previously healthy child presents with a sudden onset of severe headache, fever, and neck stiffness. Which of the following is the MOST appropriate next step?

When managing pediatric migraines, which non-pharmacological approach is found to provide the MOST benefit?

A child with Sturge-Weber Syndrome is MOST likely to present with a port-wine stain in which distribution?

A patient with Sturge-Weber Syndrome develops increasing eye pressure and corneal clouding. Which complication is MOST likely occurring?

In Sturge-Weber Syndrome, intracranial leptomeningeal angiomas are MOST commonly located in which areas?

The PRIMARY goal of administering low-dose aspirin to infants with Sturge-Weber Syndrome is to:

Which diagnostic study is MOST appropriate for initial prenatal screening for Spina Bifida?

A newborn presents with a sac-like protrusion on their lower back containing spinal fluid and neural tissue. This is MOST consistent with which neural tube defect?

After surgical repair of a myelomeningocele, what is the MOST critical neurological complication to monitor for in the newborn period?

Myelomeningocele is often associated with other congenital CNS malformations. Which of the following malformations contributes MOST significantly to a poorer prognosis?

What is the MOST common underlying mechanism leading to congenital hydrocephalus?

A surgical procedure involving the placement of a shunt would be an appropriate intervention for what condition?

In Chiari I malformation, what anatomical structure is displaced?

What is the TYPICAL presentation of Chiari I malformation affecting children?

One of the diagnostic characteristics for definitive diagnosis of Chiari I malformation is:

What prenatal event contributes to the ETIOLOGY of cerebral palsy?

Which condition is a DEFINING characteristic of Cerebral Palsy?

Which of the following is the PRIMARY finding that indicates cerebral palsy?

A child with cerebral palsy exhibits increased muscle tone, especially in the lower extremities, leading to scissoring of the legs. What is the MOST likely type of motor impairment?

What genetic test is MOST likely used to confirm Charcot-Marie-Tooth disease?

A child is suspected of having Charcot-Marie-Tooth. Which of the following would be MOST suspect?

What testing MUST be abnormal in order to make a CHARCOT-MARIE-TOOTH diagnosis?

Which finding on physical exam is MOST suggestive of Charcot-Marie-Tooth disease?

A rehabilitation plan that includes what component is crucial to treatment for Charcot-Marie-Tooth?

In Infant Botulism, how does the toxin lead to muscle weakness?

What is the MOST LIKELY source of C. botulinum spores?

Which of the following is the PREFERRED treatment for infant botulism?

What BEST DESCRIBES the genetic transmission pattern for spinal muscular atrophy (SMA)?

What is the PRIMARY indication for use of the medication Spinraza in Spinal Muscular Atrophy (SMA)?

A 4-month-old infant presents with severe hypotonia, tongue fasciculations, and absent reflexes. Which diagnosis is MOST concerning?

Following recovery from acute paralytic poliomyelitis, a patient reports new onset muscle weakness, fatigue, and joint pain decades later. These signs are MOST suggestive of.

The MOST effective method for preventing poliomyelitis is:

Which clinical symptoms are considered the hallmark diagnostic criteria for Tourette Syndrome, according to DSM-5?

What BEST describes the typical progression of tics in Tourette Syndrome?

In the general approach to treatment, what is the most evidence-based initial intervention for managing tics in Tourette Syndrome?

Which diagnostic result is MOST indicative of mononeuropathy rather than polyneuropathy?

A patient presents with paresthesias and weakness isolated to the ulnar aspect of their hand and forearm. What is the MOST likely diagnosis?

A patient is diagnosed with Carpal Tunnel Syndrome. What conservative measures should be taken first?

A patient presents with bilateral numbness, tingling, and burning pain in their feet, gradually ascending up their legs. This presentation is MOST characteristic of:

What does the TREATMENT STRATEGY require when dealing with a patient exhibiting diabetic neuropathy?

A child presents with a sudden, severe headache along with a fever and stiff neck. What is the MOST appropriate next step in management?

In the diagnostic workup of a child with headaches, which of the following findings is MOST indicative of a secondary headache requiring further investigation?

What is the PRIMARY purpose of the acute management of febrile seizures in children?

Which clinical feature is MOST suggestive of a complex febrile seizure rather than a simple febrile seizure?

What is the inheritance pattern MOST commonly associated with Sturge-Weber Syndrome?

A patient has a facial port-wine stain in Sturge-Weber Syndrome. What is it caused by?

A child with Sturge-Weber Syndrome develops seizures, which is a neurological feature seen in Sturge-Weber Syndrome. What is the cause of their seizures?

In a patient with suspected Sturge-Weber Syndrome, where are the most common locations for intracranial leptomeningeal vascular anomaly and calcifications?

Which complication associated with myelomeningocele would indicate the greatest long term risk?

Following surgical repair of a myelomeningocele, what is the MOST critical neurological complication to monitor for in the newborn period?

What is the MOST common etiology factor in congenital hydrocephalus?

What is THE MOST accurate description of Chiari Malformation type 1?

After receiving a diagnosis of Charcot-Marie-Tooth, the patient should receive genetic testing for which gene duplication?

When assessing a child for Charcot-Marie-Tooth, what is the MOST common way that the disease presents?

Which diagnostic test is ABNORMAL, making the diagnosis of Charcot-Marie-Tooth?

A baby presents with poor head control, constipation, and a weak cry. What diagnosis should be considered?

How does the botulinum toxin affect function in Infant Botulism?

What is the best preventative strategy, and BEST reduces the risk of Infant Botulism?

What clinical signs differentiate Spinal Muscular Atrophy from other motor neuron diseases in infants?

What factor increases the probability that someone will contract poliomyelitis?

What is the MOST effective preventative measure for poliomyelitis?

A young patient is diagnosed with Tourette’s Syndrome. What has to have occurred in the patient’s clinical history to make the diagnosis?

What is the MOST common pattern of signs and symptoms for Tourette's in patients?

Which factor is a key in successful controlling or decreasing patient's symptoms due to Tourette Syndrome?

Mononeuropathy involves which distribution?

Mononeuropathy is often associated with which of the following factors?

Which of the following nerve studies help in identifying the local region of compression in Monoeuropathy?

Which strategy is the initial treatment plan in Mononeuropathy?

Why is it important to determine causative events in both classifications of neuropathies?

A 6-year-old child presents with a history of recurrent headaches, primarily located in the front of their forehead, and that they report are brought on by stress and lack of sleep. What is the MOST appropriate first-line abortive treatment for their headaches?

A pregnant mother recently had an ultrasound that showed her child has spina bifida. She asks you if there was any preventative measure she could have taken to prevent the condition, what is the MOST important thing to advise this parient?

Infant botulism is treated using botulism immune globulin intravenous (BIG-IV or BabyBIG). What is the goal of the treatment when treating infant botulism?

A 3-year-old presents with gait abnormalities, muscle weakness, and abnormal posture. What is the PRIMARY cause for patients presenting with this cerebral palsy symptom?

You are advising a group of expecting parents about steps that can help support their health, and in turn the health of their future baby. What type of exposure should they do their best to reduce, in hopes of preventing brain injury?

When educating parents of a child with cerebral palsy what should they know about course and prognosis?

What is the treatment plan to reduce paralysis associated with poliomyelitis?

A 7-year-old patient presents with a new onset of motor tics and vocal tics for at least 1 year. The nurse practitioner suspects the patient has Tourette Syndrome. What must also be ruled out?

In distinguishing between primary and secondary headaches in children, what aspect of the clinical presentation is most critical?

Which of the following headache characteristics should prompt immediate neuroimaging in a child?

What non-pharmacologic intervention is typically recommended as a first-line approach for managing tension-type headaches in children?

Which of the following clinical findings is most suggestive of Sturge-Weber Syndrome?

A child with Sturge-Weber Syndrome presents with increased intraocular pressure. This is most indicative of which complication?

In Sturge-Weber Syndrome, where are intracranial leptomeningeal vascular anomalies and calcifications most frequently located?

What is the rationale for using low-dose aspirin in infants with Sturge-Weber Syndrome?

Which prenatal screening method is typically used to detect Spina Bifida?

A newborn presents with a sac-like protrusion containing spinal fluid and neural tissue on their lower back. Which condition is most likely?

After surgical repair of a myelomeningocele, what potential complication requires close monitoring in the newborn period?

Myelomeningocele is often associated with what?

What is the most common cause of congenital hydrocephalus?

Which of the following represents a surgical intervention for hydrocephalus?

In Chiari malformation type 1, which anatomical structure is displaced?

What diagnostic findings are indicative of Chiari 1 malformation?

What characteristics should be expected from patients diagnosed with Cerebral Palsy?

What is the common presentation of cerebral palsy?

In the genetic testing associated with CMT, what is the most common subtype?

What clinical presentation would be most expected with Infant botulism?

Which action best reduces the risk of Infant Botulism?