Podcast
Questions and Answers
Which of the following is considered a primary headache?
Which of the following is considered a primary headache?
All pain-sensitive structures in the head are related to headaches.
All pain-sensitive structures in the head are related to headaches.
False
Name a common type of primary headache.
Name a common type of primary headache.
Tension headache
Increased intracranial tension may cause ________ to be a symptom.
Increased intracranial tension may cause ________ to be a symptom.
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Which of the following is used in rescue therapy for neurological conditions?
Which of the following is used in rescue therapy for neurological conditions?
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Match the headaches with their classification:
Match the headaches with their classification:
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Istradefylline is a dopaminergic agonist.
Istradefylline is a dopaminergic agonist.
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What is the combination of drugs typically prescribed for patients over 60 with significant disease?
What is the combination of drugs typically prescribed for patients over 60 with significant disease?
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Amantadine acts as a dopaminergic agonist, an __________ agent, and an NMDA antagonist.
Amantadine acts as a dopaminergic agonist, an __________ agent, and an NMDA antagonist.
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Match the following drugs with their actions:
Match the following drugs with their actions:
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Which of the following is NOT a feature indicative of Atypical PD?
Which of the following is NOT a feature indicative of Atypical PD?
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Progressive supranuclear palsy (PSP) is classified as a taupathy.
Progressive supranuclear palsy (PSP) is classified as a taupathy.
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What is a common symptom of Multiple System Atrophy (MSA)?
What is a common symptom of Multiple System Atrophy (MSA)?
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Corticobasal degeneration (CBD) is classified under __________.
Corticobasal degeneration (CBD) is classified under __________.
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Match the signs and characteristics with the correct form of atypical Parkinson's disease:
Match the signs and characteristics with the correct form of atypical Parkinson's disease:
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What is the most common type of migraine?
What is the most common type of migraine?
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Tension-Type Headaches are often accompanied by symptoms such as vomiting, photophobia, and phonophobia.
Tension-Type Headaches are often accompanied by symptoms such as vomiting, photophobia, and phonophobia.
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What is the first-line treatment for acute tension-type headache?
What is the first-line treatment for acute tension-type headache?
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Migraine is more common in __________ than in males.
Migraine is more common in __________ than in males.
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Match the following migraine aura subtypes with their descriptions:
Match the following migraine aura subtypes with their descriptions:
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Which treatment is used for moderate to severe migraine attacks?
Which treatment is used for moderate to severe migraine attacks?
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Retinal migraine is characterized by bilateral visual impairment.
Retinal migraine is characterized by bilateral visual impairment.
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Name one disadvantage of triptans in migraine treatment.
Name one disadvantage of triptans in migraine treatment.
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The first-line treatment in migraine prophylaxis is __________.
The first-line treatment in migraine prophylaxis is __________.
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Match the following types of migraine with their characteristics:
Match the following types of migraine with their characteristics:
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Which of the following symptoms is associated with hypertonia in Parkinson's disease?
Which of the following symptoms is associated with hypertonia in Parkinson's disease?
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Bradykinesia refers to the slowness of movement associated with Parkinson's disease.
Bradykinesia refers to the slowness of movement associated with Parkinson's disease.
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What imaging technique is primarily used for the clinical diagnosis of Parkinson's disease?
What imaging technique is primarily used for the clinical diagnosis of Parkinson's disease?
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The test that assesses postural instability in Parkinson's patients is called the __________ test.
The test that assesses postural instability in Parkinson's patients is called the __________ test.
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Match the following symptoms with their descriptions:
Match the following symptoms with their descriptions:
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What is a common clinical feature of Corticobasal Degeneration (CBD)?
What is a common clinical feature of Corticobasal Degeneration (CBD)?
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The hot cross bun sign is associated with Progressive Supranuclear Palsy (PSP).
The hot cross bun sign is associated with Progressive Supranuclear Palsy (PSP).
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What phenomenon involves a patient not using one limb and instead using the opposite limb in CBD?
What phenomenon involves a patient not using one limb and instead using the opposite limb in CBD?
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In Multiple System Atrophy (MSA), a significant finding on MRI is the __________ sign.
In Multiple System Atrophy (MSA), a significant finding on MRI is the __________ sign.
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Match the following conditions with their primary age of onset:
Match the following conditions with their primary age of onset:
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What is commonly considered the initial stage of a migraine?
What is commonly considered the initial stage of a migraine?
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Migraine headaches typically last longer than 72 hours.
Migraine headaches typically last longer than 72 hours.
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What visual symptom is commonly experienced during the aura phase of a migraine?
What visual symptom is commonly experienced during the aura phase of a migraine?
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The most common trigger for migraines is __________.
The most common trigger for migraines is __________.
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Match the type of migraine with its criteria.
Match the type of migraine with its criteria.
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What is the primary purpose of L-Dopa in patients?
What is the primary purpose of L-Dopa in patients?
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Dopaminergic agonists have no associated neuropsychiatric risks.
Dopaminergic agonists have no associated neuropsychiatric risks.
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Name one side effect associated with L-Dopa administration.
Name one side effect associated with L-Dopa administration.
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L-Dopa is combined with a ________ decarboxylase inhibitor to enhance its effects.
L-Dopa is combined with a ________ decarboxylase inhibitor to enhance its effects.
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Match the following symptoms with their descriptions:
Match the following symptoms with their descriptions:
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Which of the following clinical features is specific to supranuclear palsy?
Which of the following clinical features is specific to supranuclear palsy?
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Neuroprotective drugs are currently used in the treatment of atypical Parkinson's disease.
Neuroprotective drugs are currently used in the treatment of atypical Parkinson's disease.
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What MRI finding is associated with midbrain atrophy in supranuclear palsy?
What MRI finding is associated with midbrain atrophy in supranuclear palsy?
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The __________ sign refers to the inability to stop clapping once initiated.
The __________ sign refers to the inability to stop clapping once initiated.
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Match the following signs to their descriptions:
Match the following signs to their descriptions:
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Study Notes
Active Space
- Pramipexole, ropinirole, rotigotine and apomorphine are commonly used medications.
- Apomorphine is used in rescue therapy and given intravenously.
- Amantadine has three mechanisms of action: Dopaminergic agonist, anticholinergic and NMDA antagonist.
- Newer medications include Istradefylline, an adenosine A2A antagonist, and Pimavanserin, a serotonin 5-HT2A antagonist.
Relevant anatomy
- Pain-sensitive structures include the dural venous sinuses, dura around vessels, large veins, the circle of Willis, dural arteries (meningeal 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
- Classified as either primary or secondary.
- Primary headaches are benign, recurrent and without an organic cause.
- Common primary headaches include tension headaches, migraines and trigeminal autonomic cephalgias (TACS), such as cluster headaches.
- Secondary headaches are caused by systemic infection, brain tumor, or other underlying conditions.
Dangerous headache
- New onset headache after 55 years of age may be a sign of giant cell arteritis.
- Associated symptoms of giant cell arteritis include scalp tenderness, thick/nodular vessels, jaw claudication, pyrexia of unknown origin (PUO) and increased erythrocyte sedimentation rate (ESR).
- Increased intracranial tension can be a sign of dangerous headache, often presenting as a subacute, rapidly progressive headache that worsens at night.
- Vomiting can precede or relieve headaches in cases of increased intracranial tension.
- Headache intensity can increase when bending or coughing, indicating meningeal inflammation.
- Blurred vision is another potential sign.
Atypical Parkinson's Disease
- Atypical Parkinson's disease (PD) includes tauopathies such as progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) and synucleinopathies such as multiple system atrophy (MSA) and diffuse Lewy body dementia (DLB).
- Atypical PD is characterized by absence of tremor, poor response to L-dopa, rapid progression dementia at presentation, visual hallucinations, fluctuating alertness, myoclonus and cortical signs.
- PSP is characterized by falls due to postural instability, dementia and bilateral axial rigidity.
- MSA is characterized by supranuclear gaze palsy, autonomic nervous system (ANS) symptoms and disproportionate cerebellar signs.
- MSA is most common in individuals 45-55 years of age and is characterized by glial cytoplasmic inclusions that stain for alpha-synuclein.
- It is categorized into three subtypes: MSA-P (Parkinson predominant, 80%), MSA-C (Cerebellar predominant) and MSA-A/Shy-Drager syndrome (ANS predominant, 15%).
Tension-Type Headache
- Characterized by middle-aged female prevalence, holocranial band-like or pressure sensation, minimal impact on daily activities and the absence of dangerous signs.
- Acute treatment involves NSAIDs.
- Chronic treatment involves Amitriptyline as a TCA.
- It is not associated with vomiting, photophobia, phonophobia, or aggravation by movement.
Migraine
- Episodic headache, commonly with adolescent onset and increased severity with age.
- It is more frequent in females.
- Migraines are often familial, but not attributable to other diseases.
- Normal systemic examination is present.
- 80% of migraines are common and not associated with aura.
- 20% of migraines are classical, and are associated with aura. Aura symptoms last between 15 minutes and 1 hour.
- Aura subtypes include visual, sensory, auditory and motor.
- Common visual aura is characterized by fortification spectra (zig-zag lines in peripheral vision) and a hazy spot in the center of vision.
- Sensory aura includes tingling and numbness.
Other types of migraine with aura
- Ophthalmoplegic migraine involves transient third nerve palsy and pupil involvement.
- Retinal migraine presents with unilateral visual impairment.
- Basilar type migraine involves occipital involvement, ataxia, tinnitus and vertigo.
- Familial hemiplegic migraine is caused by a calcium channelopathy.
Migraine treatment options
Acute attack
- Antiemetics include metoclopramide (5-10 mg/day) and Domperidone.
- Mild migraine is treated with paracetamol or NSAIDs such as naproxen 550 mg twice daily or ibuprofen 400 mg four times a day.
- Moderate to severe migraine is treated with triptans, which are 5-HT1B/1D agonists.
- Triptan examples include eletriptan, rizatriptan (5-10 mg, max 30 mg), almotriptan and sumatriptan (50-100 mg, max 200 mg).
- Very severe migraines are treated with: sumatriptan 6mg subcutaneously twice or zolmitriptan intranasally.
- Triptan disadvantages include contraindication in cardiovascular or cerebrovascular disease, efficacy dependence on Tmax, and ineffectiveness for migraines with aura.
- Ergotamine is the least likely to cause recurrent migraine attacks.
Prophylaxis
- First line: Beta-blockers, valproate, topiramate.
- Second line: SNRIs such as venlafaxine.
- Third line: Pizotifen (5-HT antagonist), flunarizine (Ca2+ channel blocker), clonidine, candesartan.
- Note: Side effects of topiramate include weight loss and renal calculi. Side effects of valproate include weight gain, liver disease, hyperammonemia and thrombocytopenia.
Newer drugs and therapies
- Erenumab, a CGRP antagonist.
- Supraorbital transcutaneous stimulation.
- Onabotulinum toxin A.
- Greater occipital nerve block.
Stages of Migraine
- Prodrome: Mood disturbance, irritability and depressive symptoms.
- Aura: Zig-zag lines (most common).
- Headache: Unilateral or holocranial, typically frontotemporal, lasting 4-72 hours, described as throbbing or pounding, often associated with nausea, vomiting, photophobia, phonophobia, osmophobia, photopsia (flashing lights) and scintillating scotomas.
- Postdrome: Lethargy and low mood/depression.
Migraine Triggers
- Sleep deprivation (most common), due disrupting the serotonin-melatonin cycle.
- Menstruation, due to hormonal fluctuations.
- Excess stress.
- Other triggers include traffic, climate, mosquitoes, henna, and food.
Criteria for Migraine
Common migraine
- Minimum 5 attacks lasting 4-72 hours, meeting at least 2 of the following characteristics: unilateral, pulsatile, moderate to severe intensity, aggravated by routine physical activity or movement.
- At least one of the following clinical features is present: nausea or vomiting, photophobia or phonophobia.
- It is not attributed to another disorder.
Classical migraine
- At least 2 attacks lasting 4-72 hours, with at least 3 of the following aura characteristics: gradual onset, duration of 5-60 minutes, fully reversible, at least 2 of the aura symptoms occur in succession, do not impair consciousness and are visual, sensory or both.
L-Dopa for symptomatic relief
- L-dopa is a key medication for symptomatic relief of Parkinson's related symptoms.
- L-dopa is quickly metabolized peripherally.
- It is administered in combination with dopa decarboxylase inhibitors such as carbidopa and benserazide, and was previously combined with COMT inhibitors such as tolcapone (hepatotoxic) and entacapone (hepatotoxic, orange colored urine).
- The typical L-dopa to dopa decarboxylase inhibitor ratio is 100:25.
- Patients are initiated on L-dopa in small doses, multiple times a day, and can be crushed and mixed with carbonated water.
- Symptoms that may not respond to L-dopa treatment include freezing, falls. dysphagia, dysarthria and ANS symptoms.
Side effects of L-dopa
- Nausea.
- Vomiting.
- Postural hypotension.
Motor fluctuations
- Wearing-off: A decline in medication effectiveness as the duration between doses increases.
- On/Off: Periods of fluctuating motor function, with periods of good motor function ("on") interspersed with periods of limited movement ("off").
- Delayed onset of action: A delay in the onset of L-dopa's therapeutic effects.
- Drug failure: A gradual loss of L-dopa's effectiveness over time.
-
Dyskinesias: Involuntary, uncontrolled movements.
- Peak dose dyskinesia: Choreiform movements often occur during peak L-dopa concentrations.
- Off period dyskinesia: Dystonia often occurs during low drug concentrations.
Amantadine
- Amantadine is used to manage motor fluctuations.
Central anticholinergics
- Anticholinergics, such as trihexyphenidyl, are used in cases of drug-induced PD.
Dopaminergic agonists
- Can be used as an alternative to L-dopa.
- Associated with dopamine dysregulation syndrome, which encompasses neuropsychiatric symptoms such as visual hallucinations, impulse control issues and addiction.
Supra Nuclear Palsy (PSP)
Clinical Features
- Bilateral symmetrical axial rigidity → Extension hypertonia.
- Postural instability leading to frequent falls.
- Supranuclear gaze palsy → Downgaze palsy (eyelid apraxia).
- Dementia.
- Wide-based gait.
- Square-wave jerks (Saccades intrude fixation).
- 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.
- Pseudobulbar palsy.
Note
- In contrast to supranuclear palsy, a nuclear gaze palsy affects the brainstem and results in the loss of the vestibulo-ocular reflex.
Investigation
- MRI:
- Midbrain and tegmentum atrophy.
- Sagittal section: Hummingbird sign/penguin silhouette sign.
- Axial section: Morning glory sign.
- Midbrain and tegmentum atrophy.
Treatment of PD
- No treatment exists for atypical PD.
- Medications are used for symptomatic relief only.
- Neuroprotective drugs such as MAO-B inhibitors (Selegiline) and glutamic acid release inhibitors (Riluzole) are no longer used for PD, although Riluzole is still used for amyotrophic lateral sclerosis (ALS).
Parkinson's Disease (PD)
Symptoms
-
Hypertonia:
- Cogwheel rigidity: At wrist, rigidity combined with tremor.
- Lead pipe rigidity: At elbow.
- Froment's sign : Activity-induced increase in contralateral rigidity.
-
Akinesia/Bradykinesia:
- Bradyphrenia: Slowed thinking.
- Reduced arm swing: Difficulty with limb movements.
- Flexion hypertonia. Stooped posture.
- Short, quick steps and freezing between steps.
- Festinant gait: Narrow-based gait.
- Micrographia: Small handwriting.
- Hypomimia: Minimal facial expression.
- Hypophonia: Decreased voice volume.
- Reduced blinking. Glabellar tap sign positive (absence of blinking after repeated taps to the forehead).
-
Note:
- In atypical PD, axial rigidity is prominent, particularly difficulty turning in bed and rising from a chair (Wheel chair sign).
- Hypokinesia, a symptom of reduced movement, can also be caused by hypothyroidism.
- Postural instability: Unsteadiness & falls. Pull test: Patient falls backward (Normal: Takes 2-3 steps backwards to correct posture).
Investigation
- Clinical diagnosis is typically made based on symptoms.
- MRI findings:
- Hyperintensities bilaterally in the midbrain grey matter surrounded by hypointense red nuclei and crural fibers.
- Absence of swallow tail sign [Indicative of substantia nigra (SN)].
- Note: PD patients can also exhibit sensory symptoms (pain, anosmia), neuropsychiatric symptoms (depression more common than dementia), and gastrointestinal symptoms such as dysphagia.
Multiple System Atrophy (MSA)
MSA-P (Parkinson predominant)
- Most common subtype.
- Includes bilateral axial rigidity and craniocervical dystonia.
- Stridor may be present.
- Falls are less frequent.
- Pisa sign (Leans to one side).
- Rocket sign (Getting up from a chair upright).
Investigation
- MRI findings:
- Hot cross bun sign (in the pons).
- Putaminal rim sign.
Corticobasal Degeneration (CBD)
- Typically affects individuals aged 65-75 years.
Involvement
- Clinical features include cortical involvement (superior parietal lobule), apraxia, myoclonus, cortical dementia progression, alien limb phenomenon.
Note
- Alien limb phenomenon is commonly unilateral, causing patients to stop using the affected limb and begin using the other limb instead.
Investigation
- MRI finding: Cortical atrophy.
Progressive Supranuclear Palsy (PSP)
- Also known as Steel Richardson syndrome.
- Typically affects individuals aged 55-65 years.
- The most common subtype of atypical PD.
Imaging Descriptions
- MRI images showing different anatomical features including the "hot cross bun" sign, and "leaning tower of Pisa" sign.
- Other images may be blurry and difficult to interpret.
Additional Notes
- This document provides a summary of various neurological diseases, highlighting the differentiating symptoms, signs, and investigative findings based on MRI imaging.
- It also identifies the age ranges commonly associated with each disease.
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Description
This quiz covers essential information on headache management, including various medications such as Pramipexole, Ropinirole, and Apomorphine. It also delves into the relevant anatomy associated with pain-sensitive and pain-insensitive structures, along with classifications of headaches. Test your knowledge on these important health topics!
