Medicine Marrow Pg No 647-656 (Neurology)
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Questions and Answers

What percentage of amyotrophic lateral sclerosis (ALS) cases are genetic?

  • 50%
  • 10% (correct)
  • 90%
  • 25%

    • Cognition is affected in patients with amyotrophic lateral sclerosis (ALS).

    False

    What is the primary structure that degenerates first in amyotrophic lateral sclerosis (ALS)?

    Anterior horn cell (AHC)

    The category of ALS that involves both upper and lower motor neurons is known as _____ motor neuron disease.

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

    Match the following clinical features with their descriptions in ALS:

    <p>Fasciculations = Muscle twitches that may occur in affected muscles Brisk reflexes = Increased reflex response often present Atrophy of the forearm = Distal asymmetric wasting in upper limbs Preserved cognition = Higher mental functions remain intact</p> Signup and view all the answers

    Which of the following symptoms are associated with X-linked SMA?

    <p>Proximal wasting with pseudohypertrophy of calf muscles</p> Signup and view all the answers

    Benign Monomelic Amyotrophy primarily affects young females.

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

    What is another name for Benign Monomelic Amyotrophy?

    <p>Hirayama's disease</p> Signup and view all the answers

    X-linked SMA is associated with __________ insensitivity.

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

    Match the following conditions with their associated symptoms:

    <p>X-linked SMA = Tongue wasting + perioral fasciculation Madras MND = Bilateral facial weakness + sensorineural hearing loss Benign Monomelic Amyotrophy = Affects young males primarily</p> Signup and view all the answers

    Which of the following drugs is known to exacerbate Myasthenia Gravis?

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

    The Lambert-Eaton Myasthenic Syndrome (LEMS) is associated with antibodies against postsynaptic membrane receptors.

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

    What is the first-line treatment for a first attack of Myasthenia Gravis?

    <p>Cholinesterase inhibitors</p> Signup and view all the answers

    Symptoms of a cholinergic crisis include DUMBELLS, which stands for Diarrhea, Urination, ______, Bradycardia.

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

    Match the following treatments with their indications:

    <p>IVIg = Myasthenic crisis Plasma exchange = Life-threatening MG Steroids = Maintenance therapy Azathioprine = Refractory MG</p> Signup and view all the answers

    Which tract is primarily responsible for transmitting pain and temperature sensations?

    <p>Lateral spinothalamic tract</p> Signup and view all the answers

    The ventral root is a part of the sensory input pathway.

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

    What type of myelopathy is caused by a tumor such as an ependymoma?

    <p>Compressive myelopathy</p> Signup and view all the answers

    The ________________ tract is responsible for proprioception.

    <p>Dorsal spinocerebellar</p> Signup and view all the answers

    Match the spinal cord pathways to their primary functions:

    <p>Fasciculus cuneatus = Sensory information Ventral reticulospinal tract = Motor control Ventral spinothalamic tract = Pressure and touch sensations Rubrospinal tract = Influencing movement</p> Signup and view all the answers

    What symptom would you expect at the level of a spinal cord lesion?

    <p>Symptoms depend on anterior horn cells</p> Signup and view all the answers

    Syringomyelia is classified as a non-compressive myelopathy.

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

    Describe a characteristic symptom of a L4 spinal segment lesion.

    <p>Absent knee jerk reflex</p> Signup and view all the answers

    Which of the following symptoms is specific for Myasthenia Gravis (MG)?

    <p>Fatigue while chewing</p> Signup and view all the answers

    The Peek sign indicates normal eyelid closure strength.

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

    What test shows the improvement of symptoms after the administration of a cholinesterase inhibitor?

    <p>Edrophonium test</p> Signup and view all the answers

    Myasthenia Gravis symptoms include _____ diplopia and ophthalmoplegia.

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

    Match the following tests with their corresponding characteristics:

    <p>Ice pack test = Cold temperature improves conduction AChR antibody = Most specific test for MG Single nerve fibre electromyography = Most sensitive test for MG Neostigmine test = Cholinesterase inhibitor admin</p> Signup and view all the answers

    Which cranial nerves are primarily involved in Bulbar Palsy?

    <p>Cranial nerves 9, 10, 11, 12</p> Signup and view all the answers

    In Pseudobulbar Palsy, the gag reflex is typically absent.

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

    What type of motor neuron lesion is associated with Bulbar Palsy?

    <p>Lower motor neuron (LMN)</p> Signup and view all the answers

    Patients with ALS may experience ____ due to thenar atrophy.

    <p>hand weakness</p> Signup and view all the answers

    Match the following symptoms with their associated conditions:

    <p>Flaccid tongue with fasciculations = Bulbar Palsy Spastic tongue = Pseudobulbar Palsy Dysarthria with risk of aspiration = Bulbar Palsy Exaggerated gag reflex = Pseudobulbar Palsy</p> Signup and view all the answers

    What is the primary function of the spinal cord?

    <p>Motor output to and sensory output from trunk and limbs</p> Signup and view all the answers

    The spinal cord is 70 cm long.

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

    What is the upper limit of the spinal cord's extent?

    <p>Upper border of the atlas (C1)</p> Signup and view all the answers

    The spinal cord extends from the junction between L1 and L2 in _____ and from L3-L4 in _____ individuals.

    <p>adults, children</p> Signup and view all the answers

    Match the vertebrae with their level of segments:

    <p>Upper cervical = Same Lower cervical = +1 Thoracic = +2 Lumbar = L1, L2</p> Signup and view all the answers

    Which of the following symptoms is NOT associated with Conus Medullaris Syndrome?

    <p>Present motor weakness</p> Signup and view all the answers

    Sensation is consistently completely lost in Cauda Equina Syndrome.

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

    What is the primary pathology associated with Cauda Equina Syndrome?

    <p>Disc abnormality</p> Signup and view all the answers

    The length of the Filum Terminale is approximately __________ cm.

    <p>20</p> Signup and view all the answers

    What sensation is primarily affected by complete hemisection of the spinal cord?

    <p>Posterior column sensations</p> Signup and view all the answers

    Match the spinal roots with their corresponding actions:

    <p>L₁ - L₂ = Hip flexion L₄ - S₁ = Dorsiflexion S₁ = Hip extension L₂ - L₃ = Hip adduction</p> Signup and view all the answers

    Sacral sparing occurs with intradural or extramedullary lesions.

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

    What type of neuropathy is characterized by stocking and glove sensory loss?

    <p>Diabetic neuropathy</p> Signup and view all the answers

    The lateral spinothalamic tract is responsible for transmitting _______ sensations.

    <p>pain and temperature</p> Signup and view all the answers

    Match the following pathologies with their descriptions:

    <p>Complete hemisection of spinal cord = Loss of posterior column sensations on one side Dissociative sensory loss = Sparing of posterior column and involvement of spinothalamic tract Cervical compression myelopathy = Sensory loss in the upper limb Brown-Sequard syndrome = Loss of one side sensations with contralateral symptoms</p> Signup and view all the answers

    What is the first peak age range for Myasthenia Gravis, particularly in females?

    <p>20-40 years</p> Signup and view all the answers

    Thymoma is present in 80% of seropositive Myasthenia Gravis cases.

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

    What is the primary characteristic of the pure motor syndrome in Myasthenia Gravis?

    <p>Fluctuating weakness of proximal muscles</p> Signup and view all the answers

    In Myasthenia Gravis, thymic hyperplasia is present in _____% of seropositive cases.

    <p>80</p> Signup and view all the answers

    Match the antibodies involved in Myasthenia Gravis with their descriptions:

    <p>Anti-acetylcholine receptor (AChR) = Specific in 85% of generalized MG cases Anti-musk antibody = Absent in ocular myasthenia Anti-LRP4 = Not specified in the data provided</p> Signup and view all the answers

    What is the typical gender ratio observed in early onset Myasthenia Gravis cases?

    <p>Females &gt; Males</p> Signup and view all the answers

    Neck extensor weakness is a feature associated with Myasthenia Gravis.

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

    The drug commonly used to treat Myasthenia Gravis is called _____

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

    What percentage of Myasthenia Gravis cases are classified as generalized MG?

    <p>85</p> Signup and view all the answers

    What is the prognosis for thymectomy in adult onset generalized Myasthenia Gravis?

    <p>Very good</p> Signup and view all the answers

    Study Notes

    Amyotrophic Lateral Sclerosis (ALS)

    • ALS is a neurodegenerative disease affecting upper and lower motor neurons.
    • 90% of cases are sporadic and 10% are genetic, often due to copper-zinc superoxide dismutase defects.
    • ALS typically affects individuals over 50 years old, with males affected more frequently than females.
    • Average survival after onset is 3 years.
    • Primary structures affected include the anterior horn cells, lower motor neurons in brainstem nuclei, upper motor neurons in the corticospinal tract, and the corticobulbar tract.
    • Clinical features include distal asymmetric upper limb atrophy, fasciculations, cramps, brisk reflexes, and loss of dexterity or tone.
    • Cognition, the autonomic nervous system, and sensory systems remain intact.

    Spinal Cord

    • The spinal cord is a continuation of the medulla, extending from the upper border of the atlas (C1) to the junction between L1 and L2 in adults, or L3-L4 in children.
    • 31 spinal cord segments correspond to the 31 pairs of spinal nerves.
    • Each spinal nerve root is composed of a dorsal and ventral root.
    • The spinal canal is 70 cm long, accommodating the spinal cord and its surrounding meninges (dura mater, arachnoid mater, and pia mater).

    Spinal Cord Tracts

    • Fasciculus cuneatus and fasciculus gracilis: Sensory tracts, involved in proprioception, fine touch, and pressure.
    • Lateral corticospinal tract (pyramidal tract): Major motor pathway for voluntary movements.
    • Dorsal spinocerebellar tract and ventral spinocerebellar tract: Carry sensory information related to proprioception.
    • Lateral spinothalamic tract: Carries pain and temperature sensations.
    • Ventral spinothalamic tract: Carries pressure and touch sensations.
    • Rubrospinal tract: Influences movement.
    • Lateral reticulospinal tract, ventral reticulospinal tract, and vestibulospinal tract: Involved in motor control and balance.
    • Tectospinal tract: Controls reflexes related to eye and head movements.

    Myelopathies

    • Myelopathies are spinal cord diseases causing sensory, autonomic, and motor symptoms.
    • Types of myelopathies:
      • Compressive:
        • Intramedullary: e.g. tumors (ependymoma) and syringomyelia.
        • Extramedullary: e.g. disc compression.
      • Non-compressive: e.g. transverse myelitis.
    • Lesion Presentation:
      • Above the level of the lesion: Normal function.
      • At the level of the lesion: Symptoms depend on the involvement of anterior horn cells.
      • Below the level of the lesion: Motor (UMN-type) and sensory loss.

    Grey Matter

    • Anterior horn cells: Motor neurons responsible for voluntary movement.
    • Level of lesion symptoms:
      • Motor symptoms (LMN-type): Absent reflexes are a key diagnostic feature.
      • Sensory symptoms: Vary based on the location of the lesion.

    X-linked SMA (ALS Variants)

    • Characterized by spinobulbar involvement, proximal muscle wasting, tongue wasting, and perioral fasciculations.
    • May be associated with androgen insensitivity.

    Benign Monomelic Amyotrophy (BMA)

    • Also known as Hirayama disease.
    • Primarily affects young males, causing weakness and atrophy in the upper extremity.
    • Symptoms improve with rest and neck extension.

    Myasthenia Gravis (MG)

    • Autoimmune neuromuscular disorder affecting the neuromuscular junction.
    • Characterized by fluctuating weakness in skeletal muscles.
    • Primary cause is antibodies against the acetylcholine receptor (AChR) at the neuromuscular junction.
    • 85% of cases are generalized MG.
    • 50% of cases are ocular myasthenia.
    • 10-20% of cases are generalized MG with negative anti-AChR antibodies.
    • May involve thymus hyperplasia or thymoma in a significant proportion of cases.
    • Thymectomy in adult-onset generalized MG has a good prognosis.

    Lambert-Eaton Myasthenic Syndrome (LEMS)

    • Autoimmune disorder affecting the release of acetylcholine from presynaptic motor neurons.
    • Primarily affects proximal lower limbs and autonomic nervous system.
    • Associated with small cell lung cancer in a significant number of cases.
    • Antibodies target voltage-gated calcium channels on the presynaptic membrane.
    • Treatment includes 3,4-diaminopyridine and supportive measures.

    Myasthenia Gravis: Clinical Features

    • Initial weakness, fatigue, and ptosis are characteristic features.
    • Can involve bulbar muscles causing difficulty with speech and swallowing.
    • Diurnal variation with worsening symptoms towards the evening.
    • Muscle weakness increases with repetitive activity and improves with rest.
    • Associated antibodies can be detected.
    • Thymectomy can be beneficial in specific cases.
    • Treatment options include cholinesterase inhibitors, immunosuppressants, and plasmapheresis.

    Myasthenia Gravis: Investigations

    • Clinical tests: Ice pack test, edrophonium test, and neostigmine test.
    • Immunological tests: Anti-AChR antibody, anti-muscle specific kinase antibody, and anti-LRP4 antibody.
    • Electrophysiological studies: Single nerve fiber electromyography, repetitive nerve stimulation test, and nerve conduction studies.

    Bulbar vs. Pseudobulbar Palsy

    • Bulbar palsy: Affects lower motor neurons of cranial nerves 9, 10, 11, and 12, causing dysphagia, dysarthria, and tongue fasciculations.
    • Pseudobulbar palsy: Affects upper motor neurons to cranial nerves 9, 10, 11, and 12, resulting in spastic dysarthria, exaggerated reflexes, and emotional lability.

    Conus Medullaris

    • The terminal portion of the spinal cord located at the level of L1/L2 in adults and L3/L4 in children.
    • Conus medullaris syndrome: Affects the sacral nerve roots and the conus medullaris, causing sacral sensory loss, urinary incontinence, and sphincter dysfunction.
    • Cauda equina syndrome: Affects the lower lumbar and sacral nerve roots, leading to asymmetric radiculopathy, motor weakness, and variable sensory loss.

    Anatomy of Spinal Cord

    • The spinal cord extends from the medulla to the tip of the coccyx.
    • Cervical enlargement houses the nerve roots for the upper limbs.
    • Lumbar enlargement houses the nerve roots for the lower limbs.
    • The filum terminale is a fibrous extension of the pia mater that extends from the conus medullaris to the coccyx.
    • The dorsal tract (posterior column) carries sensory information for proprioception and tactile discrimination.
    • The lateral spinothalamic tract carries pain and temperature sensations.
    • Note:* This is a broad overview of the content. The text you provided does not include comprehensive explanations of specific syndromes and their management, so this summary focuses on highlighting key facts and anatomical features. Further research would be necessary for obtaining a complete understanding of these topics.

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    Description

    This quiz covers essential aspects of Amyotrophic Lateral Sclerosis (ALS) and spinal cord anatomy. Learn about the neurodegenerative effects of ALS, its clinical features, and the structure of the spinal cord. Test your knowledge on key facts related to motor neurons and survival statistics.

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