CNS Pg No 585 -594
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Questions and Answers

Which of the following drugs is used in rescue therapy?

  • Rotigotine
  • Apomorphine (correct)
  • Ropinirole
  • Pramipexole

    • Pimavanserin is identified as a dopaminergic agonist.

    False

    Name one of the three mechanisms of action of Amantadine.

    Dopaminergic agonist (or Anticholinergic or NMDA antagonist)

    Patients who are ____ years old with significant disease present should be treated with L-dopa + carbidopa.

    <p>60</p> Signup and view all the answers

    Match the following drugs with their primary action or effect:

    <p>Istradefylline = Minimizes motor fluctuations Pramipexole = Antidepressant action also Amantadine = NMDA antagonist Rotigotine = Dopaminergic agonist</p> Signup and view all the answers

    Which of the following structures are pain sensitive? (Select all that apply)

    <p>Dura around vessels</p> Signup and view all the answers

    A tension headache is classified as a secondary headache.

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

    What is a common symptom associated with a dangerous headache related to increased intracranial tension?

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

    A headache that occurs in individuals over 55 years, along with scalp tenderness and thick nodular vessels, may indicate __________.

    <p>giant cell arteritis</p> Signup and view all the answers

    Match the type of headache to its description:

    <p>Tension headache = Most common primary headache Migraine = A recurrent primary headache Cluster headache = Type of trigeminal autonomic cephalgia Brain tumor headache = Rare secondary headache cause</p> Signup and view all the answers

    What is a common feature of Tension-Type Headaches (TTH)?

    <p>Does not affect daily activities</p> Signup and view all the answers

    Migraine headaches are more frequent in males than females.

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

    What medication is commonly used as a prophylactic treatment for chronic Tension-Type Headaches?

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

    Migraine is characterized by an __ onset, with severity increasing with age.

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

    Match the following types of migraines with their descriptions:

    <p>Common = Not associated with aura Classical = Associated with aura</p> Signup and view all the answers

    Which feature is NOT indicative of atypical Parkinson's Disease?

    <p>Presence of tremor</p> Signup and view all the answers

    Corticobasal degeneration (CBD) is classified as a synucleinopathy.

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

    What are glial cytoplasmic inclusions associated with in multiple system atrophy (MSA)?

    <p>A synucleinopathy</p> Signup and view all the answers

    Two examples of taupathies include _________ and _________.

    <p>Progressive supranuclear palsy (PSP), Corticobasal degeneration (CBD)</p> Signup and view all the answers

    Match the symptoms with their associated condition:

    <p>Falls due to postural instability = Progressive supranuclear palsy (PSP) ANS predominant = Multiple system atrophy (MSA) Supranuclear gaze palsy = Multiple system atrophy (MSA) Dementia component = Progressive supranuclear palsy (PSP)</p> Signup and view all the answers

    Which type of rigidity is characterized by both rigidity and tremor at the wrist?

    <p>Cogwheel rigidity</p> Signup and view all the answers

    Flexion hypertonia results in a stooped posture.

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

    What is the clinical sign that indicates activity-induced increase in contralateral rigidity?

    <p>Froment's sign</p> Signup and view all the answers

    Patients with Parkinson's Disease may experience __________, which is characterized by reduced facial expressions.

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

    Match the terms related to Parkinson's Disease with their descriptions:

    <p>Froment's sign = Activity-induced increase in rigidity on the opposite side Micrographia = Decreased size of handwriting Festinant gait = Narrow-based walking with quick steps Hypophonia = Reduced volume of speech</p> Signup and view all the answers

    What type of tremor is characterized by symptoms at rest?

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

    Essential tremor tends to have a family history present.

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

    What is the frequency range of essential tremor?

    <p>5-12 Hz</p> Signup and view all the answers

    Parkinson's disease tremor is primarily characterized by _____ tremor.

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

    Match the symptoms with the corresponding condition:

    <p>Micrographia = Parkinson's disease tremor Postural tremor = Essential tremor Asymmetric distribution = Parkinson's disease tremor Stable course = Essential tremor</p> Signup and view all the answers

    What is a common side effect of L-Dopa?

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

    Amantadine is used primarily to increase synthetic dopamine levels in the brain.

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

    What is the recommended dosing strategy for L-Dopa?

    <p>Small doses multiple times throughout the day</p> Signup and view all the answers

    Dopamine dysregulation syndrome may manifest as ________ control issues.

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

    Match the following side effects with their corresponding description:

    <p>Wearing off = Symptoms return before the next dose is due Peak dose dyskinesia = Choreiform movements due to high dose Off-period dyskinesia = Dystonia due to low drug concentration Postural hypotension = Drop in blood pressure upon standing</p> Signup and view all the answers

    Which clinical feature is associated with a wide-based gait?

    <p>Postural instability</p> Signup and view all the answers

    There are drugs available that can alter the natural history of Parkinson's disease.

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

    What is the significance of the hummingbird sign in MRI scans related to Parkinson's disease?

    <p>It indicates midbrain and tegmentum atrophy.</p> Signup and view all the answers

    The ______ sign is characterized by fissures on the forehead.

    <p>Procerus/reptile</p> Signup and view all the answers

    Match the following signs with their descriptions:

    <p>Applause sign = Can't stop clapping once started Dirty tie sign = Appearance of the tie that creates an unusual pattern Gunslinger position = Hands held close to the body Rocket sign = A specific posture seen in Parkinson's disease</p> Signup and view all the answers

    What is a primary motor symptom of MSA-P?

    <p>Midline axial rigidity</p> Signup and view all the answers

    Corticobasal degeneration typically presents with bilateral symptoms.

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

    What neurological condition is also known as Steel Richardson syndrome?

    <p>Progressive Supranuclear Palsy</p> Signup and view all the answers

    The presence of the __________ sign is associated with MSA-P.

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

    Match the neurological condition with its corresponding feature:

    <p>MSA-P = Hot cross bun sign in MRI CBD = Apraxia PSP = Cortical atrophy</p> Signup and view all the answers

    What is the primary type of neurons affected in Parkinson's disease?

    <p>Nigrostriatal dopaminergic neurons</p> Signup and view all the answers

    Autonomic nervous system symptoms, including erectile dysfunction, are among the earliest signs of Parkinson's disease.

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

    What protein is primarily associated with the formation of Lewy bodies in Parkinson's disease?

    <p>α-synuclein</p> Signup and view all the answers

    The early symptoms of Parkinson's disease can include REM sleep disorders and __________ dysfunction.

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

    Match the following symptoms with their occurrence related to Parkinson's disease:

    <p>REM sleep disorders = 5-10 years before disease onset Erectile dysfunction = 5-10 years before disease onset Motor symptoms = After disease onset Vision changes = Can occur later in disease progression</p> Signup and view all the answers

    Study Notes

    Active Space

    • Pramipexole, ropinirole, rotigotine, and apomorphine are examples of active space drugs
    • Apomorphine is used in rescue therapy and administered intravenously
    • Amantadine has three mechanisms of action: dopaminergic agonist, anticholinergic, and NMDA antagonist
    • Newer drugs include Istradefylline, an antagonist that minimizes motor fluctuations, and Pimavanserin, an SHT antagonist
    • A treatment protocol for patients over 60 with significant disease involves L-dopa and carbidopa

    Relevant Anatomy

    • Structures sensitive to pain include the dural venous sinuses, dura around vessels, large veins, the circle of Willis, dural arteries, the first few centimeters of medium-sized vessels, pia mater, and small cerebral vessels
    • Pain-insensitive structures include the choroid plexus and ependyma

    Headache

    • Headache classifications include primary and secondary
    • Primary headaches are benign, recurrent, and lack an organic cause
    • The most common types of primary headaches are tension headache and migraine
    • Secondary headaches are caused by systemic infections or brain tumors, which are rare
    • Dangerous headaches include new onset headache after age 55, which can be seen in giant cell arteritis, and increased intracranial tension, which is subacute and rapidly progressive.

    Dangerous Headache

    • New onset headache after age 55 is often associated with scalp tenderness, thick or nodular vessels, jaw claudication, pyrexia of unknown origin (PUO), and increased erythrocyte sedimentation rate (ESR)
    • Increased intracranial tension presents with nocturnal awakening, vomiting that precedes or relieves headache, increased intensity with bending or coughing, and blurring of vision
    • Investigations for dangerous headaches include MRI, MRA, MRV, direct ophthalmoscopy, and imaging if papilledema is present

    Atypical Parkinson's Disease

    • Atypical Parkinson's disease includes taupathy and synucleinopathy
    • Taupathy encompasses progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD)
    • Synucleinopathy includes multiple system atrophy (MSA) and diffuse Lewy body dementia (DLB)

    Clinical Features of Atypical PD

    • Atypical PD lacks tremor, is unresponsive to L-dopa, rapidly progresses, presents with dementia, visual hallucinations, fluctuating alertness, myoclonus, and cortical signs
    • PSP is characterized by falls due to postural instability, dementia, and bilateral axial rigidity
    • MSA presents with supranuclear gaze palsy, autonomic nervous system symptoms, and disproportionate cerebellar signs
    • MSA is a synucleinopathy that typically affects individuals between 45 and 55 years of age
    • MSA is characterized by glial cytoplasmic inclusions that stain for alpha synuclein, REM sleep disorders years before diagnosis, and autonomic nervous system dominance
    • MSA is categorized into MSA-P (80%): Parkinson predominant, MSA-C: Cerebellar predominant, and MSA-A/Shy-Drager syndrome (15%): Autonomic nervous system predominant

    Tension-Type Headache

    • Tension-type headache is most common in middle-aged females
    • One-third of TTH cases are associated with depression
    • TTH presents with holocranial band-like or pressure sensation
    • TTH doesn't affect daily activities and lacks dangerous signs
    • Acute TTH is treated with NSAIDs, while chronic TTH is managed with amitriptyline, a TCA
      • Amitriptyline is used prophylactically for associated depression
    • TTH doesn't involve vomiting, photophobia, phonophobia, or aggravation by movement

    Migraine

    • Migraine is episodic and typically begins in adolescence
    • Its severity tends to increase with age
    • Migraine occurs more frequently in females than males
    • There's a positive family history of migraine
    • Migraine doesn't demonstrate systemic abnormalities
    • There are two migraine types: common (80%) and classical (20%)
    • The classical type is associated with aura, while the common type is not
    • Aura subtypes include visual, sensory, auditory, and motor

    Migraine Aura Subtypes

    • Visual aura subtypes include fortification spectra (zig-zag lines in peripheral vision), hazy spot in the center of vision
    • Sensory aura involves tingling and numbness
    • Auditory and motor auras also occur

    Parkinson's Disease

    • Parkinson's disease is a progressive neurological disorder that affects movement due to the degeneration of dopamine-producing cells in the substantia nigra
    • It typically presents with tremor, rigidity, bradykinesia, and postural instability
    • Tremor is a rhythmic oscillatory movement of a body part
    • Rest tremor occurs when the affected limb is at rest
    • Tremor can be accentuated by stress and fatigue
    • Rigidity is resistance to passive movement
    • Cogwheel rigidity is a type of rigidity that involves intermittent catches or jerks during passive movement
    • Lead-pipe rigidity is a type of rigidity that is constant and unwavering
    • Bradykinesia is a slowness of movement
    • Postural instability makes it difficult to maintain body position
    • Additional symptoms of Parkinson's disease include freezing, falls, dysphagia, dysarthria, and autonomic nervous system symptoms

    ### Clinical Features of Parkinson's Disease

    • Parkinson's disease can be diagnosed based on clinical features
    • Common clinical features include bradykinesia, rigidity, tremor, and postural instability
    • Bradykinesia can manifest as bradyphrenia, reduced arm swinging, flexion hypertonia, short quick steps with freezing, festinant gait, micrographia, hypomimia, hypophonia, and reduced blinking
    • Rigidity can present as cogwheel rigidity, lead pipe rigidity, Froment's sign (activity-induced increase in contralateral rigidity), and flexion hypertonia
    • Postural instability can lead to unsteadiness and falls
    • The pull test can be used to assess postural instability: a patient with postural instability will fall backward

    Investigation of Parkinson's Disease

    • MRI can be used to investigate Parkinson's disease and differentiate it from atypical forms
    • MRI findings in Parkinson's disease include hyperintensities in the grey matter of the midbrain level, surrounded by hypointense red nuclei and crural fibers
    • The absence of the swallow tail sign in the substantia nigra is another characteristic finding on MRI
    • The swallow tail sign is a normal structure observed in the substantia nigra on MRI

    Drugs for Symptomatic Relief of Parkinson's Disease

    • L-dopa is a dopamine precursor used to treat Parkinson's disease
    • L-dopa is administered in combination with a dopa decarboxylase inhibitor, such as carbidopa or benserazide, to reduce its peripheral metabolism
    • The optimal L-dopa to dopa decarboxylase inhibitor ratio is 100:25
    • Patients are typically started on low doses of L-dopa, administered multiple times daily
    • L-dopa can cause side effects, including nausea, vomiting, postural hypotension, and motor fluctuations
    • Motor fluctuations include wearing off (space drug without much gap), on/off phenomenon, delayed onset of action, drug failure, and dyskinesias
    • Dyskinesias include peak dose dyskinesia (choreiform movements due to high dose) and off-period dyskinesia (dystonia due to low drug concentration)
    • Amantadine is used to address dyskinesias
    • Central anticholinergics like trihexyphenidyl are utilized in drug- induced Parkinson's disease
    • Dopaminergic agonists can be used as a replacement for L-dopa
    • Dopaminergic agonists can cause dopamine dysregulation syndrome, characterized by neuropsychiatric manifestations like visual hallucinations, impulse control issues, and addiction

    Comparison of Parkinson's Disease Tremor and Essential Tremor

    • Parkinson's disease tremor is typically at rest and sometimes reemergent, while essential tremor is postural
    • Parkinson's disease tremor has a frequency of 4-6 Hz, while essential tremor has a frequency of 5-12 Hz
    • Parkinson's disease tremor is asymmetric, while essential tremor is mostly symmetric
    • Parkinson's disease tremor affects hands and sometimes legs, while essential tremor affects hands, head, and voice
    • Parkinson's disease tremor causes small writing (micrographia), while essential tremor causes large and tremulous writing
    • Parkinson's disease tremor progresses, while essential tremor is stable or slowly progressive
    • Parkinson's disease tremor has an uncommon family history (1%), while essential tremor has a strong family history
    • Extrapyramidal signs, such as bradykinesia, rigidity, and loss of postural reflex, are present in Parkinson's disease tremor but absent in essential tremor
    • Parkinson's disease tremor is relieved by levodopa, dopamine agonists, and anticholinergics, while essential tremor is relieved by alcohol, propranolol, primidone, topiramate, gabapentin, clonazepam
    • The usual site for surgical deep brain stimulation in Parkinson's disease tremor is the subthalamic nucleus or globus pallidus interna, while the ventral intermediate thalamus is targeted in essential tremor

    MSA-P

    • MSA-P is characterized by midline (most common) axial rigidity and craniocervical dystonia
    • Other observed features may include stridor and falls (less frequent)
    • Signs include the Pisa sign and rocket sign (getting up from a chair upright)
    • MRI findings for MSA-P include a hot cross bun sign in the pons and a putaminal rim sign

    Corticobasal Degeneration (CBD)

    • CBD typically affects individuals between 65 and 75 years of age
    • CBD involves both the cortex (superior parietal lobule) and basal ganglia
    • Clinical symptoms include apraxia, myoclonus, and progression to cortical dementia
    • The alien limb phenomenon is a characteristic feature of CBD, where symptoms are unilateral and the patient stops using the affected limb and starts using the opposite limb
    • MRI reveals cortical atrophy in CBD

    Progressive Supranuclear Palsy (PSP)

    • PSP, also known as Steel Richardson syndrome, affects individuals between 55 and 65 years of age
    • PSP presents with atypical parkinsonian symptoms
    • MRI findings for PSP include cortical atrophy

    Parkinson's Disease Pathology

    • Parkinson's disease involves the degeneration of nigrostriatal dopaminergic neurons
    • Intracytoplasmic Lewy bodies, composed of α-synuclein, are a hallmark of the disease

    Pre-Clinical Symptoms of Parkinson's Disease

    • Some symptoms, such as REM sleep disorders and autonomic nervous system (ANS) symptoms like erectile dysfunction, can occur 5-10 years before the onset of Parkinson's disease

    Genetic Defect in Young Onset PD

    • A genetic defect can contribute to young-onset Parkinson's disease

    Clinical Features of a Neurological Condition

    • The document describes clinical features, investigations, and treatment of a neurological condition, likely Parkinson's disease or a similar disorder
    • The clinical features include symmetrical axial rigidity (extension hypertonia), postural instability (frequent falls), supranuclear gaze palsy (downgaze palsy, eyelid apraxia), dementia, wide-based gait, square wave gait, procerus/reptile sign (fissures on forehead), dirty tie sign, rocket sign, hemifacial spasms, gunslinger position (hands close to the body), applause sign (inability to stop clapping once started), spastic dysarthria, frontal release signs, and pseudobulbar palsy
    • Nuclear gaze palsy, where the brainstem is affected and the vestibulo-ocular reflex is lost, helps differentiate from supranuclear palsy
    • Investigations for the neurological condition include MRI, which reveals atrophy in the midbrain and tegmentum
    • On sagittal sections, MRI displays the hummingbird sign or penguin silhouette sign, while axial sections show the morning glory sign
    • There's no treatment for atypical Parkinson's disease or drugs available to alter its natural history
    • Treatment is limited to providing symptomatic relief
    • Neuroprotective drugs, such as MAO-B inhibitors (selegiline) and glutamic acid release inhibitors (riluzole), are not currently used but riluzole remains in use for ALS
    • The document appears to be clinical notes or a medical report, including photographs or scans of patient presentations
    • A proper interpretation would require qualified clinicians.

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