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Neurology > Neurodegenerative disorders > Flashcards

Neurodegenerative disorders Flashcards

MLA Content Y3

1
Q

What is Multiple Sclerosis?

A

Cell-mediated autoimmune condition where repeated episodes of inflammation of nervous tissue in the brain and spinal cord lead to loss of the insulating myelin sheath.

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2
Q

Is MS more common in males or females?

A

Females 3:1

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3
Q

At what age does MS usually present?

A

Between 20 and 40.

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4
Q

What cell mediates the Autoimmune disease in MS?

A

T-cells mediate the inflammatory process mainly within the white matter of the brain and spinal cord.

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5
Q

Although plaques of demyelination can occur anywhere within the CNS white matter, where do they have a preference for?

A

Sites such as optic nerves, Brainstem (and its cerebellar connections) and the cervical cord.

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6
Q

What is the definition of a relapse in MS?

A

A relapse is considered any new or acutely worsening neurological symptoms with objective evidence that:
- Is consistent with inflammation and demyelination.
- Lasts for more than 24 hrs.
- Is seperated by at least 30 days from the onset of the last relapse.
- Not related to infection, fever or other stress.

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7
Q

What are the 3 typical Patterns of MS?

A

Relapsing-Remitting MS (RRMS) 85-90%

Secondary progressive MS (RRMS typically develops into this - 75% of cases)

Primary Progressive MS (PPMS)

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8
Q

What is a pseudorelapse in MS?

A

A temporary worsening of symptoms without actual myelin inflammation or damage, brought on by other influences. e.g. fatigue, overexertion, fever, infection.

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9
Q

What does pyramidal dysfunction refer to in the Clinical signs of MS?

A

Pyramidal Dysfunction
- Increased tone
- Spasticity
- Weakness
- Affects extensors of upper and flexors of lower limbs.

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10
Q

Does Multiple Sclerosis affect the Peripheral or Central Nervous system?

A

Central Nervous System.

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11
Q

What Cells are involved in Myelination of the axons in the CNS?

A

Oligodendrocytes

(Schwann cells in the PNS)

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12
Q

What is an MS attack and how could a patient present?

A

The Flare up of acute inflammation and immune cell infiltration which causes damage to the Myelin.

OPTIC NEURITIS.

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13
Q

When can Re-myelination occur?

A

In early disease. Symptoms are able to resolve.

However in later stages the Re-myelination is incomplete and symptoms will gradually become more permanent.

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14
Q

What is Optic Neuritis?

A

Painful visual loss over 1-2 weeks.
- Blurred vision in one eye and loss (or reduction) in colour vision.
- RAPD (relative afferent pupillary defect) will be present on affected side.
- Pale optic disc on affected side.

  • Central Scotoma (enlarged blindspot)
  • pain with eye movement.
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15
Q

What is a unique Characteristic feature of Multiple Sclerosis lesions?

A

“Disseminated in Time and Space”

  • Meaning they vary in location and as a result the affected sites and symptoms change over time.
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16
Q

What are some theorised causes of MS? (NOT CONFIRMED)

A
  • Multiple Genes
  • EBV
  • Low Vit.D
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17
Q

Describe the typical onset of Symptoms in MS

A

Symptom onset usually occurs over more than 24hrs and tend to last days to weeks before improving at first presentation.

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18
Q

How is Optic neuritis usually treated?

A

High dose steroids.

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19
Q

What examples of eye movement abnormalities can patients with MS present with?

A

Lesions affection CN 3, 4 or 6 can cause Diplopia and Nystagmus.

Intranuclear opthalmoplegia is caused by a lesion in the medial longitudinal fasciculus. - responsible for making sure the eyes move together so a default causes impaired adduction on the same side as the lesion and Nystagmus on the contralateral side.

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20
Q

What examples of Focal weakness present in MS?

A
  • Incontinence
  • Horner syndrome
  • Facial nerve palsy
  • Limb paralysis
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21
Q

What Examples of Focal Sensory Symptoms can MS present with?

A
  • Trigeminal Neuralgia
  • Numbness
  • Paraesthesia (pins & Needles)
  • Lhermitte’s signs
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22
Q

What is Lhermitte’s sign?

A

Describes and Electric shock sensation that travels down the spine and into the limbs when flexing the neck.
- It indicates disease in the cervical spinal cord and dorsal column.
(caused by stretching the demyelinated dorsal column)

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23
Q

What types of ataxia can be seen in MS?

A

Cerebellar or Sensory.

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24
Q

What is Sensory Ataxia?

A

Occurs due to a loss of proprioception (the ability to sense the position of a joint).
- It results in a positive Romberg’s test and can cause Pseudoathetosis (involuntary writhing movements).
- A lesion in the dorsal colum can cause sensory ataxia.

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25
Q

What is cerebellar ataxia?

A

Results from problems with the cerebellum coordinating movement, indicating a cerebellar lesion.

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26
Q

How is MS diagnosed?

A

Diagnosis is made clinically with symptoms suggesting lesions change location over time.
Support diagnosis:
- MRI can demonstrate typical lesions.
- Lumbar Puncture can detect OLIGOCLONAL BANDS (distinct bands of IgG) in the CSF. (Present in 90+% of cases)

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27
Q

What is the management of an Acute relapse of MS?

A
  • Mild - Symptomatic Tx
  • Moderate - Oral Steroids
  • Severe - Admit / IV Steroids.
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28
Q

What Symptomatic Treatment is used in MS?
- Pyramidal Dysfunction Tx
- Sensory symptom Tx
- Urge Incontinence Tx
- Fatigue Tx
- Oscillopsia Tx

A

Pyramidal Dysfunction
- Physio, Occ health
- Anti-spasmodic e.g Baclofen or tizanidine.

Sensory Symptoms
- Anticonvulsants e.g. Gabapentin
- Antidepressants e.g. SSRIs

Urge Incontinence
- Antimuscarinic meds e.g. solifenacin

Fatigue
- Amantadine
- Modafinil if sleepy
- Hyperbaric oxygen

Oscillopsia
- gabapentin or memantine

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29
Q

What are some Disease modifying therapies for Relapsing-remitting MS induce remission?

A

First line
- Interferon Beta
- Tecfedira, aubagio

Second line
- Monoclonal antibody (tysabri, ocrevus, Lemtrada)

Third line
- Mitoxantrone.

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30
Q

What is Neuromyelitis Optica Spectrum Disorder (NMOSD)?

A

An inflammatory, antibody mediated, immunologic disease of the CNS causing Demyelination of the optic nerve and spinal cord.

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31
Q

Is Neuromyelitis Optica Spectrum Disorder more common in males or females?

A

Females (5:1)
Average onset mid 30s

32
Q

What clinical features are involved in NMOSD?

A
  • Present with Acute flu-like illness (fever, myalgia, headache)
  • More specific signs and Symptoms develop after (optic neuritis or myelitis)
  • Unlike MS related optic neuritis, NMOSD may have worse visual prognosis and patients left with visual disability.
33
Q

What is the Diagnostic Criteria for Neuromyelitis Optica Spectrum Disorder?

A
  • Optic Neuritis
  • Longitudinal Transverse Myelitis
  • Area Prostrema Syndrome.

Diagnosis = 2 core clinical + Aquaporin 4 Antibodies.

34
Q

What Type of antibodies are found in NMOSD?

A

Anti-aquaporin 4 antibodies (99% specific but 75% sensitive)

35
Q

What is the management of Neuromyelitis Optica Spectrum Disorder?

A
  • Steroids - IV methylprednisolone (prolonged oral course)
  • Immunosuppression (Cyclophosphamide, Azathioprine)
  • Plasma exchange
  • Monoclonal Antibodies (Rituximab)
36
Q

What is the definition of Dementia?

A

Clinical syndrome caused by a number of brain disorders which cause memory loss, decline in some other aspects of cognition and difficulties with the activities of daily living.

37
Q

What are the different types of dementias?

A

Alzheimer’s disease (50-70% cases)
Vascular dementia (25%)
Dementia with lewy bodies (15%)
Frontotemporal dementia (<5%)
Potentially treatable dementias (e.g. Metabolic (uraemia), Toxic (alcohol), Vitamin Deficiency B12 and Thiamine.. etc)

38
Q

What is Vascular Dementia?

A

Brain damage due to cerebrovascular disease: either major stroke, multiple smaller unrecognised strokes (multi-infarct) or chronic changes in smaller vessels (subcortical dementia)

39
Q

What is Dementia with lewy bodies?

A

Deposition of abnormal protein (⍺-synuclein) within neurons in the brainstem and neocortex.

40
Q

What is Fronto-temporal Dementia?

A

Specific degeneration / atrophy of the frontal and temporal lobes of the brain.

41
Q

What are some of the Rare causes of Dementia?

A
  • Huntingtons disease
  • Parkinson’s Disease
  • Creutzfeldt–Jakob disease (CJD)
42
Q

What pathological hallmarks occur in Alzheimers disease?

A
  • Loss of cortical neurones
  • Neurofibrillary (tau protein) tangles
  • Senile plaques - Extracellular protein deposits containing amyloid Beta-protein.
43
Q

Which areas of the brain are degenerated in AD?

A

Degeneration of the Medial Hippocampus and later the Parietal Lobes.

Leading to forgetfulness then apraxia / visuospatial disturbance.

44
Q

Which area of the brain is important in the formation and retrieval of memories?

A

The Hippocampus.
(one of the earliest areas affected in Dementia)

45
Q

How much does having a first degree relative with AD contribute to an increased risk?

A

Doubled lifetime risk.

46
Q

Which Genes have been linked to the development of Alzheimers disease?

A
  • E4 allele of the apolipoprotein E gene.
  • Point mutations in the APP gene.
  • Mutations in presenilin (PS) -1 and 2.
47
Q

Which disease has a higher incidence of AD?

A

Downs’ Syndrome.
(3 copies of the APP gene on chromosome 21)
(typical onset in 3rd or 4th decade in these patients)

48
Q

What is the Genetic Mutation that causes Huntington’s disease?

A

A CAG repeat encoding poly-glutamine. It has a toxic effect on cells resulting in neuronal loss.

49
Q

What Areas of the brain are degenerated in Huntington’s?

A

Caudate atrophy - loss of cells in the basal ganglia causing flattening of the normal convex curve of the lateral ventricles.
Cells are also lost from other areas of the brain including the cerebral cortex.

50
Q

What is the name given to a disease where each sequential generation will have symptoms develop sooner than the previous? (e.g. Huntington’s)

A

Anticipation.
(symptoms develop sooner in each successive generation)

51
Q

What are the clinical features of Alzheimer’s disease?

A
  • Gradual onset, decline of particularly short-term memory.
  • Poor concentration, poor sleep, low mood.
  • In end stages - Hallucinations, poor dentition, skin ulcers, loss of verbal communication.
52
Q

What type of memory is often well preserved in AD?

A

Autobiographical and Political.

53
Q

What are two Atypical presentations of AD?

A

Posterior cortical atrophy causing visuospatial disturbance.
Progressive primary aphasia.

54
Q

What are the clinical signs of Vascular dementia?

A

The hallmark of vascular dementia is a progressive, stepwise deterioration in cognition. (months to years)
Stroke like features:
- Visual disturbance
- Sensory or motor symptoms
- Difficulty with attention and concentration
- Seizures
- Memory disturbance
- Gait/speech/emotional disturbance

55
Q

What are the 3 syndromes associated with Fronto-temporal dementia?

A
  • Behavioural Varient. (Behavioural changes, executive dysfunction, disinhibition, impulsivity, loss of social skills, apathy, obsessions, change in diet)
  • Primary progressive aphasia. (Effortful non-fluent speech, articulation errors, lack of grammer or words)
    -Semantic Dementia. (Impaired understanding of meaning of words, Fluent but empty speech, difficulty retrieving names)
56
Q

What are the clinical signs in Dementia with Lewy Bodies?

A
  • Fluctuating cognition: Changes in attention and alertness may occur.
  • Parkinsonism: Rigidity, bradykinesia, and postural instability are common.
  • Visual hallucinations: Patients often experience complex and recurrent visual hallucinations.
  • High sensitivity to neuroleptics: These drugs can induce or worsen parkinsonism.

if cognitive impairment and parkinsonism develop <1 year of each other, it is likely LBD.

57
Q

What percentage of patients with Parkinsons disease develop Dementia?

A

80% after 15-20 yrs of PD.

58
Q

What is needed to make a diagnosis of Dementia in PD?

A

Must have had Parkinsonism for at least 1 yr prior to onset of Dementia.
- Clinical presentation similar to Dementia with Lewy Bodies.

59
Q

What are the clinical features seen in Huntington’s?

A
  • Involuntary movements
  • Dementia
  • Decline in executive function.
  • Short and long term memory deficits.
  • Anxiety, Psychosis, Compulsions, Suicidality, aggression, blunted affect.
    Late clinical sings: Rigidity, Bradykinesia, Severe Chorea, Serious weight loss, Inability to walk or speak.
60
Q

What imaging is Typically used in Dementia?

A

CT is standard,
- Not always necessary e.g. 80 yr old w typical Hx.

Others:
- MRI if young, fast progressing or other atypical features.

61
Q

What Imaging is most useful in Frontotemporal Dementia?

A

SPECT Scan (Single-photon emission computed tomography)

62
Q

What imaging modality is most useful for suspected DLB/DPD, (esp when patient hasn’t enough supporting features to be diagnostic)?

A

DAT scan. (Dopamine Active Transpoter)

63
Q

What is the Mainstay of Tx in Dementia?

A

Supportive, unless something you can fix like metabolic disturbance or that you can cut out - then theres no Tx.

64
Q

What can be used to treat AD?

A

Cholinesterase Inhibitors (Donepezil, Rivastigmine, Galantamine)
- Contraindicated in Long QT syndrome, 2nd or 3rd degree Heart block and Bradycardia <50bpm.

Mematine - NMDA receptor blocker. Used in Moderate to severe AD or when Cholinesterase Inhibitors are not tolerated.

65
Q

How is Vascular dementia Treated?

A
  • Manage vascular risk factors +/- Cholinesterase inhibitor.
66
Q

How is Fronto-temporal Dementia treated?

A
  • Trial of Trazadone / antipsychotics (SSRIs) to help behavioural features.
  • Safety management.
  • Power of Attorney.
67
Q

How is DLB treated?

A
  • Small dose Levodopa
  • Cholinesterase inhibitors - rivastigmine.
68
Q

How is Huntington’s managed?

A
  • Mood stabilisers.
  • Manage Chorea.
69
Q

What is Creutzfeldt-Jakob disease?

A

Rapidly progressive, invariably fatal neurodegenerative disorder believed to be caused by an abnormal isoform of a cellular glycoprotein known as a prion.

70
Q

What are prions and how do they cause Creutzfeldt-jakob disease?

A

Prions are misfolded proteins that induce normal proteins within the brain to misfold and aggregate.
Leading to neuronal loss.

71
Q

Which procedures are Prions resistant too?

A

Procedures that modify nucleic acids as they lack this.

72
Q

What are the clinical features of CJD?

A
  • Rapidly progressive dementia
  • Neurological symptoms including ataxia, weakness, and visual disturbances
  • Psychiatric impairment, often preceding the neurological symptoms
  • Myoclonus, particularly triggered by startle
73
Q

What investigations are done in CJD?

A

Tissue biopsy.
- Tonsil or Olfactory mucosa are typically used as its less invasive than brain biopsy.
- Supportive Ix include EEG, MRI and LP.

74
Q

What is the Management of CJD?

A

Symptom control:
- Medications to control Psychiatric, neurological or myoclonus symptoms.
- Palliative care.
(prognosis varies depending on type of prion disease)

75
Q

What are the different Types of Prion disease in CJD? (4 types)

A
  • Sporadic - patients ~ 60 years
  • Variant - patients ~ 20 years, due to BSE exposure
  • Iatrogenic - patients ~ 30 years
  • Genetic - affects any age group

Neurology (13 decks)

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