Neuropathology Quiz

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Questions and Answers

What medication can be used for an attack of cranial neuralgia and how is it administered?

Sumatriptan; subcutaneously or as a nasal spray

Which of the following are considered preventive treatments for cranial neuralgia?

Verapamil (correct)

Galcanezumab (correct)

Ketorolac

Prednisone (correct)

One characteristic of typical cranial neuralgias is the presence of epicyclic components.

False

Trigeminal neuralgia is characterized by intense, stabbing, electric shock-like pain that follows the ______ distribution.

nerve Signup and view all the answers

Match the following medications with the respective preventive treatment for cranial neuralgia:

Azathioprine, Rituximab, Mycophenolate Mofetil = Old treatments Inebilizumab, Satralizumab, Tocilizumab, Eculizumab = New treatments Signup and view all the answers

What is the most common type of headache?

Tension headache Signup and view all the answers

Which of the following are common characteristics of tension headaches? (Select all that apply)

Pain described as pressing or tightening Signup and view all the answers

Tension headaches can last from 30 minutes to __ days.

7 Signup and view all the answers

Match the following symptoms of cluster headaches to their descriptions:

Ptosis/lid edema = Drooping eyelid/swelling around the eye Miosis, enophtalmos = Constricted pupils, sunken eye Tearing = Excessive tearing from the affected eye Conjunctival injection = Redness of the eye Nasal congestion and/or rhinorrhea = Nasal stuffiness or runny nose Facial sweating = Excessive sweating on the face Signup and view all the answers

Which medication depletes a wider proportion of the B cell repertoire expressing CD19?

Inebilizumab Signup and view all the answers

MOGAD involves the Optic nerve, Spinal cord, and Brain.

True Signup and view all the answers

What is the function of Myelin oligodendrocyte glycoprotein (MOG)?

Function not fully elucidated; it may act as a cell adhesion molecule, regulate microtubule stability, and modulate myelin immune interactions. Signup and view all the answers

MOGAD pathology is characterized by the coexistence of perivenous and conﬂuent primary __________ with partial axonal preservation.

demyelination Signup and view all the answers

Match the following MOGAD disease phenotypes with their descriptions:

Optic neuritis = Severe visual loss, usually good recovery Transverse myelitis = Paraparesis, sensory level, sphincteric symptoms Acute disseminated encephalomyelitis = First polyfocal CNS episode characterized by encephalopathy Cerebral monofocal or polyfocal deﬁcits = Brain and cerebral deficits Brainstem or cerebellar deﬁcits = Deficits related to brainstem or cerebellum Cerebral cortical encephalitis = Cortical inflammation often with seizures Signup and view all the answers

What is multiple sclerosis (MS)?

MS is an autoimmune chronic inflammatory and neurodegenerative disease of the CNS that affects the brain and spinal cord. Signup and view all the answers

What is the main characteristic of multiple sclerosis (MS)?

Demyelination and axonal loss Signup and view all the answers

The incidence of multiple sclerosis (MS) is higher in countries closer to the equator.

False Signup and view all the answers

The mutation in the HLA DRB1*15:01 allele makes individuals _____ times more likely to develop MS.

three Signup and view all the answers

Match the preventive treatments for migraine with their corresponding medications:

Beta-blockers, Calcium channel blockers, Anti epileptic drugs, Anti depressants = Preventive treatments for migraine Valproic acid, Amitriptyline, Topiramate, Metoprolol = Medications used for prophylaxis in migraine Signup and view all the answers

Study Notes

Multiple Sclerosis

Definition: A chronic, autoimmune, inflammatory, and neurodegenerative disease of the CNS that affects the brain and spinal cord, preserving the nerves.
Characteristics: Demyelination and axonal loss, leading to non-traumatic disability in young adults.
Epidemiology:
- Affects 2-3 million people worldwide.
- Most common cause of non-traumatic disability in young adults.
- Incidence: 3/100,000.
- Prevalence: 100/100,000 (50-300) in clinics.
Age: Mean age of onset is 30 years, but can occur in paediatric patients and those over 60 years.
Sex: More common in women, with a female-to-male ratio of 2.5:1.
Global distribution: Increases with distance from the equator, except for some exceptions.

Etiology and Pathogenesis

Multifactorial disease involving interaction between genetic substrate and environmental factors.
Environmental factors:
- Pathogens: EBV, CMV, HSV, and others.
- Low vitamin D levels.
- Pediatric obesity.
- Cigarette smoking.
Genetics:
- Association with HLA region of chromosome 6.
- Carriers of HLA-DRB1*15:01 allele are three times more likely to develop MS.
- Genome-wide association studies identified genes associated with immune response.

Gross Anatomy

Multifocal areas of demyelination, called plaques or lesions, occur in the CNS.
Locations: brain, brainstem, and spinal cord.

Migraine

Definition: A complex, chronic, and recurrent disorder characterized by episodic attacks of severe, debilitating headache.
Characteristics:
- Typically unilateral, throbbing, and pulsating.
- Associated with sensitivity to light, sound, and smell.
- Often accompanied by nausea, vomiting, and fatigue.
- Postdrome: a feeling of exhaustion, depression, and anxiety after the attack.

Tension Headache

Definition: A primary headache disorder characterized by pain, usually mild to moderate, and a feeling of pressure or tightness around the head.
Characteristics:
- Pain is non-pulsating, pressing, or tightening.
- Often bilateral.
- Rarely accompanied by photophobia, phonophobia, or nausea.
- Not worsened by physical activity.

Cluster Headache

Definition: A type of trigeminal autonomic cephalalgia characterized by unilateral, severe, and short-lasting pain.
Characteristics:
- Pain is sharp, stabbing, and severe.
- Associated with homolateral autonomic system symptoms.
- Duration: 15-180 minutes.
- Frequency: 1 attack every other day to 8 times/day.
Autonomic system symptoms:
- Lacrimation.
- Conjunctival injection.
- Nasal congestion.
- Facial sweating.
- Ptosis.
- Miosis.
- Enophthalmos.
- Tearing.

Treatments

Symptomatic treatments:
- NSAIDs.
- Analgesic combinations.
- Triptans.
- Ergot alkaloids.
- Gepants.
- Ditans.
Preventive treatments:
- Beta-blockers.
- Calcium channel blockers.
- Anti-epileptic drugs.
- Anti-depressants.
- Botulinum toxin.
- Monoclonal antibodies anti-CGRP ligand or anti-CGRP receptor.### Hypothalamic System and Cluster Headache
The hypothalamic system is involved in the regulation of:
- Circadian rhythm
- Neuroendocrine homeostasis
- Autonomic nervous system
The hypothalamus plays a key role in cluster headache, explaining:
- Circadian pattern (attacks occur at the same time daily)
- Circannual pattern (attacks occur at the same time yearly)
- Autonomic symptoms (e.g. lacrimation, nasal congestion)
The suprachiasmatic nucleus (SCN) is the principal circadian pacemaker and is affected by photoperiodism (changes in sunlight duration)

Cranial Neuralgia

Cranial neuralgias can be typical or atypical
Atypical neuralgias are often secondary to another disease
Typical cranial neuralgias are characterized by:
- Intense, stabbing, electric shock-like pain
- Follows nerve distribution
- Trigeminal neuralgia is the most frequent (90% of cranial neuralgias)
Other possible neuralgias include:
- Glossopharyngeal neuralgia
- Occipital neuralgia
- Nervus intermedius neuralgia

Trigeminal Neuralgia

Trigeminal neuralgia is a disease that affects older people (60s and over 70s)
Incidence: 3-5/100,000
Male:Female ratio: 1:2
95% of cases involve the V2 (maxillary) or V3 (mandibular) branches
5% of cases involve the V1 (ophthalmic) branch
Clinical presentation:
- Severe and sudden facial pain
- Unilateral, short, and unpredictable attacks
- No associated neurological deficits
- No autonomic symptoms
Triggers:
- Touching or washing the face
- Shaving
- Chewing
- Speaking
- Yawning
Pathogenesis:
- Damage to the myelin sheath of the nerve
- Neurovascular conflict
- Compression of the nerve by the superior cerebellar artery (SCA) or antero-inferior cerebellar artery (AICA)

Neuromyelitis Optica Spectrum Disorder (NMOSD)

NMOSD is a demyelinating disease that affects the CNS
Diagnosis:
- Detection of AQP4-IgG in serum (best assessed using live cell-based assays)
- CSF analysis: CSF pleocytosis, CSF-restricted oligoclonal bands
Clinical features:
- Optic neuritis (45% of patients)
- Myelitis (85% of patients)
- Area postrema syndrome (15-40% of patients)
Prognosis:
- 90% of patients have a relapsing disease course
- Clinical deterioration can be irreversible
- Importance of preventive treatments
Treatments:
- Treatment of relapses: similar to MS (i.v. high dose steroids, plasmapheresis)
- Preventive treatments: azathioprine, rituximab, mycophenolate mofetil, inebilizumab, satralizumab, tocilizumab, eculizumab

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD)

MOGAD is a recently described CNS autoimmune disease
Epidemiology:
- 50% of patients have a pediatric onset
- May be monophasic (one shot and never comes back without treatment)
- F:M ratio: 1:1
- Incidence: 1.6/million persons/year
Pathology:
- Characterized by the coexistence of perivenous and confluence primary demyelination with partial axonal preservation and reactive gliosis
- Abundance of intracortical demyelinating lesions
Pathogenesis:
- Outside-in model: where autoantibodies and activated immune cells in the peripheral blood cross the blood-brain barrier
- Infections could cause bystander activation and molecular mimicry
- MOG antibody pathogenicity: opsonization of MOG, complement activation, antibody-dependent cellular cytotoxicity (ADCC), and anti-MOG ab-induced intracellular signaling cascade
MOGAD disease phenotypes:
- Optic neuritis
- Myelitis
- Brainstem syndrome
- Encephalitis
- ADEM-like presentation### Optic Neuritis
Unilateral or bilateral, severe visual loss, usually good recovery
Bilateral simultaneous clinical involvement
Longitudinal optic nerve involvement (>50% length of the optic nerve)
Perineural optic sheath enhancement
Optic disc oedema (radiologically visible optic disc swelling and optic disc oedema on fundoscopy)

Transverse Myelitis

Paraparesis, asymmetrical, sensory level, sphincteric symptoms
Longitudinally extensive myelitis
Central cord lesion or H-sign
Conus lesion

Acute Disseminated Encephalomyelitis (ADEM)

Clinical syndrome characterized by a first polyfocal CNS episode from presumed demyelination
Includes encephalopathy not explained by fever, systemic illness, or postictal features
Requires MRI abnormalities with large poorly demarcated lesions in the white matter with or without gray matter lesions
Characterized by multifocal neurologic symptoms

Disease Phenotypes

Brain, brainstem, or cerebral syndrome: radiological characteristics
- Multiple ill-defined T2 hyperintense lesions in supratentorial and infratentorial white matter
- Deep grey matter involvement
- Ill-defined T2-hyperintensity involving pons, middle cerebellar peduncle, or medulla
- Cortical lesion with or without lesional and overlying meningeal enhancement

Diagnostic Criteria

One of the core declinating events and the positivity of the antibodies are needed for diagnosis
If there is a clear positivity, diagnosis is made
If there is no clear positivity or antibodies are absent, supportive clinical and radiological criteria are needed

Comparison of MOG-Ab, ADEM, MS, and NMOSD

Age: MOG-Ab (0-40), MS (20-40), Apororin (a little bit older)
Inflammatory event: frequent in MOG-Ab, not observed in MS
Relapse: MS is relapsing, MOG-Ab can be monophasic
Radiological features: differences in brain and spinal cord lesions between MOG-Ab, NMOSD, and MS
Optic neuritis: MOG-Ab (bilateral and longitudinally extensive), MS (monolateral)
Spinal cord lesions: MOG-Ab (longitudinally extensive), MS (short lesions)

Prognosis

MOG-Ab can have a better prognosis compared to MS and NMOSD
Lesions can completely disappear in MOG-Ab
Scar formation is less common in MOG-Ab compared to MS and NMOSD

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Description

This quiz covers various topics in neuropathology, including multiple sclerosis, craniofacial pain, and headaches. Test your knowledge of these neurological disorders.