Medicine Marrow Pg No 667-676 (Neurology)
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Questions and Answers

Which of the following is NOT a manifestation of acute disseminated encephalomyelitis (ADEM)?

  • Visual loss
  • Parethisia
  • Seizure
  • Dizziness (correct)

    • Males are more frequently affected by ADEM compared to females in children.

    True

    What is the primary etiology of ADEM in children?

    Post infectious

    The predominant site of acute inflammatory demyelination in ADEM is the ______.

    <p>white matter</p> Signup and view all the answers

    Match the following clinical manifestations of ADEM with their descriptions:

    <p>Seizure = Rapid uncontrolled electrical activity in the brain Visual loss = Impaired vision often leading to blindness Meningismus = Signs of meningeal irritation Choreoathetosis = Involuntary movements featuring a combination of chorea and athetosis</p> Signup and view all the answers

    Which inheritance pattern is more common for Friedreich's Ataxia with an earlier onset?

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

    Friedreich's Ataxia leads to significant abnormalities in the cerebellum.

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

    What is the primary gene defect associated with Friedreich's Ataxia?

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

    Friedreich's Ataxia often leads to ______ symptoms such as myocardial fibrosis and cardiomyopathy.

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

    Match the terms related to Friedreich's Ataxia with their correct descriptions:

    <p>Scoliosis = Deformity of the spine Peripheral neuropathy = Loss of reflexes Dorsal root ganglion = Involvement in sensory ataxia Diabetes mellitus = Endocrine disorder often associated with Friedreich's Ataxia</p> Signup and view all the answers

    Which medication is a sphingosine receptor modulator used in the treatment of Neuromyelitis Optica?

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

    Neuromyelitis Optica is more common in males than females.

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

    What antibody is associated with Neuromyelitis Optica?

    <p>Against aquaporin 4</p> Signup and view all the answers

    In Neuromyelitis Optica, ________ is associated with long segment involvement of the spinal cord.

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

    Match the clinical features with their characteristics in Neuromyelitis Optica:

    <p>Optic neuritis = Bilateral symmetry, Poor response to Rx, Severe visual loss Myelitis = Long segment involvement, Poor prognosis Hearing loss = No involvement in NMO</p> Signup and view all the answers

    Which of the following is NOT an associated condition of multiple sclerosis?

    <p>Cystic fibrosis</p> Signup and view all the answers

    Multiple sclerosis primarily affects the peripheral nervous system.

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

    Name one risk factor associated with multiple sclerosis.

    <p>Vitamin D deficiency</p> Signup and view all the answers

    In multiple sclerosis, the disruption of the ______ is a key component of its pathogenesis.

    <p>blood-brain barrier</p> Signup and view all the answers

    Match the following features with their descriptions:

    <p>Females &gt; Males = Demographic prevalence Astrocytes = Produce VEGF HLA DR2/15 = Genetic association Cortical atrophy = Consequences of disease</p> Signup and view all the answers

    Which area is primarily supplied by the Anterior Cerebral Artery (ACA)?

    <p>Medial surface and paracentral lobule</p> Signup and view all the answers

    The Middle Cerebral Artery (MCA) supplies the inferior surface of the brain.

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

    What is a significant injury feature associated with Anterior Cerebral Artery (ACA) damage?

    <p>Urinary incontinence</p> Signup and view all the answers

    The ______ supplies the most of the superolateral surface, including the primary motor and sensory areas.

    <p>Middle Cerebral Artery (MCA)</p> Signup and view all the answers

    Match each artery with its injury features:

    <p>ACA = Weakness in left side, urinary incontinence MCA = Weakness in right side, aphasia PCA = C/L homonymous hemianopia with macular sparing</p> Signup and view all the answers

    What phenomenon involves blurring of vision during a hot shower?

    <p>Uhthoff's phenomenon</p> Signup and view all the answers

    Oligoclonal bands are absent in the CSF analysis of patients with multiple sclerosis.

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

    Which treatment is commonly used for acute attacks of multiple sclerosis?

    <p>IV steroids</p> Signup and view all the answers

    The presence of hyper-intense lesions in the MRI typically indicates __________ white matter changes.

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

    Match the following treatment options with their respective descriptions:

    <p>Natalizumab = Anti-α4 integrin with risk of PML Ocrelizumab = Treatment for primary progressive type Glatiramer acetate = Injectable therapy Alemtuzumab = Anti-CD52 therapy</p> Signup and view all the answers

    Which characteristic is typical of Multiple Sclerosis (MS) in comparison to Acute Disseminated Encephalomyelitis (ADEM)?

    <p>Complete myelopathy is common</p> Signup and view all the answers

    In children, males are more frequently affected by Multiple Sclerosis than females.

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

    What is the primary pattern of disease seen in ADEM?

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

    In Multiple Sclerosis, the lesions on MRI are often __________ in size and __________ in shape.

    <p>variable, asymmetric</p> Signup and view all the answers

    Match the following features to either ADEM or MS:

    <p>Age more than 14 years = MS Bilateral optic neuritis = ADEM Common encephalopathy = MS Relapsing remitting pattern = MS</p> Signup and view all the answers

    Which artery supplies blood primarily to the posterior aspect of the brain?

    <p>Right Vertebral Artery</p> Signup and view all the answers

    The anterior cerebral artery supplies blood to the occipital lobe.

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

    What is the purpose of the Circle of Willis?

    <p>To ensure blood supply to the brain in case of occlusion of a certain artery.</p> Signup and view all the answers

    The ______ artery connects the two anterior cerebral arteries.

    <p>Anterior Communicating</p> Signup and view all the answers

    Which artery is known for supplying blood to the eye?

    <p>Ophthalmic Artery</p> Signup and view all the answers

    Match the following arteries with their corresponding brain regions:

    <p>ACA = Frontal and Parietal Lobes MCA = Lateral Aspects of the Brain PCA = Occipital and Temporal Lobes Anterior Choroidal Artery = Serves the Brain</p> Signup and view all the answers

    The Circle of Willis is solely responsible for supplying blood to the cerebellum.

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

    What mnemonic can be used to remember the terminal branches of the internal carotid artery?

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

    What is primarily contained within the posterior limb of the internal capsule?

    <p>Corticospinal tract</p> Signup and view all the answers

    The anterior limb of the internal capsule contains mainly corticospinal fibers.

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

    List one symptom associated with a dense capsule lesion.

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

    The _____ limb of the internal capsule is associated with upper limb more than lower limb impairments.

    <p>Middle cerebral artery (MCA)</p> Signup and view all the answers

    In which part of the internal capsule would you find auditory radiation?

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

    Match the following components of the internal capsule with their associated fibers:

    <p>Anterior Limb = Frontopontine fibers Posterior Limb = Corticospinal tract Genu = Corticobulbar fibers Retrolentiform = Optic radiation</p> Signup and view all the answers

    Corticobulbar injury leads to lower limb impairment in all cases.

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

    What triad of symptoms is commonly associated with a density capsule lesion?

    <p>Hemiplegia, hemisensory loss, homonymous hemianopia</p> Signup and view all the answers

    Which disease pattern in Multiple Sclerosis is characterized by a steady increase in disability from onset without distinct relapses?

    <p>Primary-progressive</p> Signup and view all the answers

    Optic neuritis in Multiple Sclerosis always results in bilateral eye symptoms.

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

    What are the major sensory manifestations observed in Multiple Sclerosis?

    <p>Tingling, numbness, paresthesia, hypaesthesia, and unpleasant sensations.</p> Signup and view all the answers

    Dissemination in time in the McDonald Criteria requires evidence of lesions a minimum of __________ weeks apart.

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

    Which symptom is NOT listed as a minor feature of Multiple Sclerosis?

    <p>Sensory loss</p> Signup and view all the answers

    Match the following disease patterns of Multiple Sclerosis with their descriptions:

    <p>Relapsing-remitting = Periods of noticeable disability followed by remission Secondary progressive = Progression of disability after initial pattern Primary-progressive = Steady increase in disability from onset</p> Signup and view all the answers

    Allodynia is a symptom that can be associated with Multiple Sclerosis.

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

    Name one of the medications mentioned as a treatment for Multiple Sclerosis.

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

    Study Notes

    Anti MOG Antibodies Disease

    • Anti myelin oligodendrocyte glycoprotein (mOG) diseases include Optic neuritis, Myelitis, Aquaporin 4 negative NMO spectrum disorders, and Acute disseminated encephalomyelitis (ADEM).

    ADEM

    • ADEM primarily presents in children under 12 years old, with males being more frequently affected.
    • ADEM etiology is linked to post-infectious states, particularly measles and chickenpox.
    • Incubation period for ADEM is 4-21 days and post-vaccination ADEM also exists.
    • ADEM's underlying pathology is molecular mimicry of myelin basic protein and myelin oligodendrocyte protein.

    Clinical Manifestations

    • ADEM manifests as acute inflammatory demyelination and encephalitis.
    • ADEM lesions are found predominantly in white matter, but can also occur in gray matter, basal ganglia, and thalamus.
    • ADEM Lesions appear as large foci of hyperintensities on MRI and are described as “fluffy lesions.”
    • ADEM is monophasic in nature.
    • Clinical features include seizures, increased intracranial pressure (ICT), loss of consciousness, bilateral hypertonia, hyper-extensive response, meningismus, encephalopathy, visual loss, paresthesia, hemiparesis, ataxia, sphincter disturbance, myoclonus, and choreoathetosis.

    Friedreich's Ataxia

    • Friedreich's ataxia is the most common form of spinocerebellar degeneration.
    • The cerebellum appears normal in Friedreich's ataxia.
    • Inheritance pattern is autosomal recessive, leading to an earlier onset (8-20 years), whereas autosomal dominant is less common with a later onset.

    Involvement in Friedreich's Ataxia

    • Dorsal root ganglion (DRG) involvement leads to sensory ataxia.
    • Involvement of the spinocerebellar tract results in cerebellar findings like cerebellar ataxia, horizontal nystagmus, and dysarthria.
    • Cord involvement in the posterior and lateral columns leads to upper motor neuron (UMN) findings such as extensor plantar.
    • Peripheral nerve involvement causes neuropathy, leading to the loss of reflexes.

    Non-Neurological Manifestations in Friedreich's Ataxia

    • Scoliosis is a common non-neurological manifestation.
    • Cardiac symptoms, including myocardial fibrosis (in 90% of cases) and cardiomyopathy, can occur.
    • Diabetes mellitus is another associated condition.

    Tabes Dorsalis

    • Tabes dorsalis is a manifestation of neurosyphilis.
    • DRG involvement in Tabes Dorsalis leads to sensory ataxia.
    • Bowel and bladder dysfunction can also occur.

    Other Factors in Friedreich's Ataxia

    • The gene defect responsible for Friedreich's ataxia is in the FRATAXIN gene located on chromosome 9, characterized by GAA repetition.
    • The cerebrum and cranial nerves usually remain spared.
    • Males are affected more frequently than females.

    Neuromyelitis Optica (NMO)

    • NMO, also known as Devic's disease, is a chronic inflammatory demyelinating disease.
    • NMO affects females more than males.
    • The typical age of onset is around 40 years.
    • NMO can be associated with other autoimmune diseases such as systemic lupus erythematosus (SLE) in 40% of cases.
    • A characteristic antibody against aquaporin 4 is present in NMO.

    Clinical Features of NMO

    • Both optic neuritis and myelitis are key features.
    • Optic neuritis in NMO often presents with bilateral symmetry, involvement of the chiasm, poor response to treatment, and severe visual loss.
    • Myelitis in NMO is characterized by long segment involvement, long extension of transverse myelitis, poor prognosis, and a monophasic course.
    • Recurrent intractable hiccup may occur due to involvement of the area postrema.

    Investigations for NMO

    • Oligoclonal bands may be present in 30% of cases.
    • MRI of the brain is typically normal.

    Treatment for NMO

    • Treatment for NMO typically involves steroids and plasma exchange.

    Oral Medications for NMO

    • Fingolimod: Sphingosine receptor modulator
    • Teriflunomide: Dihydroorotate dehydrogenase inhibitor
    • Dimethyl fumarate

    Multiple Sclerosis (MS)

    • MS is a chronic inflammatory demyelinating disorder of the central nervous system (CNS).
    • MS is an autoimmune disease.
    • Females are affected more frequently than males.
    • MS has a significant family history.
    • HLA DR2/15 is a genetic marker associated with MS.

    Risk Factors for MS

    • Epstein-Barr virus infection (EBV)
    • Smoking
    • Vitamin D deficiency
    • High socio-economic status

    Pathogenesis of MS

    • The pathogenesis of MS involves inflammation, demyelination, gliosis, and secondary neuronal injury.
    • Disruption of the blood-brain barrier plays a role.
    • Astrocytes release vascular endothelial growth factor (VEGF), leading to exudation from capillaries.
    • On MRI, MS lesions appear hyperintense on T2-weighted images and contrast-enhanced on T1-weighted images.

    Target Antigens in MS

    • Myelin basic protein (MBP)
    • Myelin oligodendrocyte glycoprotein (MOGP)

    Consequences of MS

    • Demyelination
    • Loss of saltatory conduction
    • Cumulative axonal destruction
    • Cortical atrophy
    • Progressive neuronal damage

    Blood Supply of Brain

    • The right internal carotid artery is a principal artery.
    • The right common carotid artery supplies blood to the right side of the neck and head.
    • The brachiocephalic trunk supplies blood to the right arm and head.
    • The right vertebral artery supplies blood to the posterior aspect of the brain.
    • The terminal branches of the internal carotid artery include the ophthalmic artery, posterior communicating artery (PCA), anterior choroidal artery, anterior cerebral artery (ACA), and middle cerebral artery (MCA).
    • The circle of Willis is a circulatory anastomosis that ensures blood supply to the brain in case of occlusion of a specific artery.

    Arterial Supply to the Brain

    • The ACA supplies blood to the frontal and parietal lobes.
    • The MCA supplies blood to the lateral aspects of the brain.
    • The PCA supplies blood to the occipital and temporal lobes.
    • The anterior communicating artery connects the two ACAs.
    • The posterior communicating artery connects the PCA to the internal carotid artery.

    Multiple Sclerosis Disease Patterns

    • Relapsing-remitting MS is characterized by periods of noticeable disability followed by remission.
    • Secondary progressive MS involves progression of disability after an initial relapsing-remitting pattern.
    • Primary-progressive MS is characterized by progressive disability from onset without distinct relapses.

    Clinical Features of Multiple Sclerosis

    Major Features

    • Sensory manifestations, such as tingling, numbness, paresthesia, hypaesthesia, and unpleasant sensations, are common.
    • Allodynia (pain from normally non-painful stimuli) is also reported.
    • Motor manifestations include upper motor neuron (UMN) weakness, loss of strength, speed, tone, dexterity, spasticity, and exercise-induced fatigue.
    • Optic neuritis characteristically involves the anterior pathway and can be unilateral or asymmetric, presenting with retrobulbar pain.

    Minor Features

    • Ataxia
    • Vertigo
    • Bladder dysfunction

    Other Presentations of Multiple Sclerosis

    • Bilateral Internuclear Ophthalmoplegia (B/L INO)
    • Bilateral Trigeminal Neuralgia

    McDonald Criteria for MS Diagnosis

    • Dissemination in time: Requires evidence of two separate lesions at least four weeks apart or a single hyperintense lesion on T2-weighted images or a single lesion enhancing on contrast.
    • Dissemination in space: Evidence of lesions in two or more distinct locations.

    Blood Supply to the Surface of the Brain

    • The ACA supplies the medial and superolateral surfaces of the brain.
    • The MCA supplies most of the superolateral surface, including areas like the primary motor and sensory areas.
    • The PCA supplies the inferior surface of the brain.

    ACA vs. MCA vs. PCA: Areas Supplied

    • ACA supplies the majority of the medial surface, paracentral lobule/cingulate gyrus, and a narrow strip of the cerebral cortex bordering the superomedial area.
    • MCA supplies the majority of the superolateral surface, including frontal eye fields, Broca's and Wernicke's areas (in the dominant hemisphere), and the temporal pole.
    • PCA supplies the majority of the inferior surface excluding the temporal pole.

    ACA vs MCA vs PCA: Injury Features

    • ACA injury leads to weaker left-sided weakness (more than right side) and paracentral lobule involvement can cause urinary incontinence.
    • Cingulate gyrus involvement due to ACA damage can present with apathy, abulia, amotivation, and akinetic mutism (in severe cases).
    • MCA injury leads to stronger right-sided weakness (more than left side) and temporal pole involvement can cause macular involvement.
    • Cortical lesions in MCA can affect Broca's area (inferior frontal gyrus), Wernicke's area (superior temporal gyrus), and lead to aphasia.
    • PCA injury results in visual area damage, typically causing contralateral homonymous hemianopia with macular sparing.

    Internal Capsule

    • The internal capsule is a band of white matter connecting the cerebral cortex with the brainstem.
    • Parts of the internal capsule include the anterior limb, posterior limb, genu, retrolentiform, and sublentiform parts.

    Clinical Significance of Internal Capsule Lesions

    • Dense capsule lesions often lead to a contralateral facial palsy and hemiplegia.
    • A dense capsule lesion is associated with the triad of hemiplegia, hemisensory loss, and homonymous hemianopia.
    • The anterior limb contains frontopontine fibers and corticobulbar fibers.
    • The posterior limb contains the corticospinal tract (anterior 2/3), corticorubral fibers, and corticospinal fibers (in the genu).
    • The retrolentiform part contains the posterior thalamic radiation (optic), and the sublentiform part contains the inferior thalamic radiation (auditory).

    Neurology

    • Uhthoff's phenomenon: Blurring of vision during a hot shower.
    • Lhermitte's symptom: Flexion of the neck produces pain in the spine.
    • Pulfrich phenomenon: Altered visual perception, perceived as three-dimensional.

    MRI in Neurologic Conditions

    • Periventricular white matter hyperintensities are common in MS.
    • Dawson's fingers, hyperintensities in the corpus callosum, are typical of MS.

    Cerebrospinal Fluid (CSF) Analysis

    • Oligoclonal bands are present in CSF analysis for MS.
    • IgG is increased in approximately 90% of MS cases.

    Visually Evoked Response (VER)

    • VER has a sensitivity of 80-85% in diagnosing MS.

    Treatment for Multiple Sclerosis

    Acute Attacks

    • Intravenous steroids are used.

    Relapse/Remission

    • Intravenous steroids are used.

    Prevention

    Infusable
    • Natalizumab: Anti-α4 integrin. A potential adverse effect is progressive multifocal leukoencephalopathy (PML).
    • Ocrelizumab: Used for primary-progressive MS.
    • Alemtuzumab: Anti-CD52.
    Injectable
    • Glatiramer acetate
    • Interferon β (IFN β)

    ADEM vs MS: Hyperintensities on MRI

    • ADEM is more common in children under 14 years old, with males being more commonly affected.
    • MS is more common in females (2:1 ratio).
    • Both ADEM and MS can be triggered by infections.
    • Constitutional symptoms are more common in ADEM compared to MS.
    • Optic neuritis is typically bilateral in ADEM, while unilateral in MS.
    • Complete myelopathy is rare in ADEM but common in MS.
    • Encephalopathy is rare in ADEM but common in MS.
    • ADEM is generally monophasic, whereas MS is relapsing-remitting.
    • ADEM lesions on MRI are typically large, symmetrical, centripetal, and often involve gray matter with a mass effect.
    • MS lesions vary in size, are asymmetrically located in periventricular regions ,and are less likely to have a mass effect or gray matter involvement.

    Additional Information in the Image Descriptions

    • The text describes MRI images but lacks labeled figures or numerical values.
    • The description mentions 'periventricular lesions', but it's difficult to provide more details without a clearer image.
    • A table is presented for CSF analysis, but it is not possible to determine precise values from the provided description.

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    Description

    This quiz examines Anti MOG antibodies diseases, focusing on Acute Disseminated Encephalomyelitis (ADEM). It covers the etiology, clinical manifestations, and pathology associated with ADEM, particularly in children. Test your knowledge on symptoms, MRI findings, and treatment options related to these conditions.

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