Neuro 2 Flashcards
Define Multiple Sclerosis
A chronic, inflammatory, multifocal, demyelinating disease of the CNS. It results in:
Loss of myelin
Oligodendroglial and axonal pathology
What are the most common presenting symptoms of MS?
Optic Neuritis
Motor Weakness
Sensory Disturbances
Fatigue
What are the main risk factors for Multiple Sclerosis?
Nordic origin - living at higher latitudes
Autoimmune Conditions
HLA DLRB1*15
What are the main types of MS?
Relapsing-Remitting (Most common)
Secondary Progressive (Usually develops from RR)
Primary Progressive
How do you disagnose a patient with MS?
Absence of alternative diagnosis
Lesions are disseminated in Time and Space
Brain MRI - Plaques in white matter
CSF Studies
VEPs
Oligoclonal Bands in the CSF NOT the serum (shows location of inflammation to be solely within the CNS)
What are the key presenting symptoms of Myasthenia Gravis?
Muscles fatigue with use
Ptosis
Diplopia
Dysarthria
Dysphagia
SOB
Describe the pathophysiology of Myasthenia Gravis.
Antibodies target ACh receptors at the NMJ. Leads to fatigueability as more ACh is required with prolonged use of muscles.
How would you investigate a suspected case of Myasthenia Gravis?
Associations with Thymus Disorders.
Anti-AChR or Anti-MuSK antibodies
EMG
CT/MRI
What is Lambert Eaton Myasthenic Syndrome?
Weakness where muscles improve with use
Difficulty walking
Weakness in upper arms and shoulders
Dry Mouth, Constipation, Incontinence
Hyporeflexia
How would you diagnose a suspected case of Lambert-Eaton?
Associated with Small-Cell Lung Cancer & Autoimmune Diseases
Bloods - Anti-VGVC Antibodies
EMG
CT/MRI
What is Motor Neurone Disease?
Chronic Neurodegenerative disease causing muscle wasting, paralysis and death from respiratory failure.
How does MND present?
Progressive muscle weakness (spastic)
Dysphagia
SOB
Sparing of oculomotor, sensory and autonomic function
Bulbar Signs (Imparied swallowing and speech)
BOTH UMN and LMN symptoms
How would you investigate a suspected case of MND?
Clinical Diagnosis - investigate to exclude other possible diagnoses.
EMG
CT/MRI
Bloods (Serology, B12, CK)
Muscle Biopsy may be considered
How does Parkinson’s present?
Bradykinesia
Rigidity
Resting Tremor
Monotonous, hypotonic speech
Micrographia
Hypomimesis
March a petit pas (Shuffling Gait)
Misery
Memory Loss
What causes Parkinson’s Disease?
Degredation of dopaminergic neurones in the Substantia Nigra.
How does Parkinson’s Disease progress?
80% of neurones must be destroyed for a patient to become symptomatic.
1) Nigrostriatal Pathway - Motor symptoms
2) Mesolimbic/Mesocortical Pathways - Cognitive Symptoms
Describe the epidemiology of Parkinson’s and its Risk Factors.
Typical patient would be an old man living in the countryside.
FHx increases risk 3x
What is Parkinson’s Disease dementia?
Similar to Lewy Body Dementia. Develops many years after Parkinson’s motor symptoms present.
Presents with:
Amnestic, language difficulties
Visuospatial Dysfunction (Neglect)
Hallucinations (Less Common)
Aggression
What are the main types of Dementia?
Alzheimer’s (Most common)
Lewy Body Dementia (and Parkinson’s)
Vascular Dementia
Frontotemporal Dementia
What are the key symptoms associated with Dementia?
Cognition and memory impairment.
Changes in motivation and attention
Changes in personality and behaviour
What are the key symptoms of Alzheimer’s Dementia?
Amnesia - Remembering
Anomia - Naming
Apraxia - Doing
Agnosia - Recognising
Aphasia - Speaking
In which area of the brain would you expect to see degeneration in a patient experiencing deficits in episodic memory?
Hippocampus
Describe the pathophysiology of Alzheimer’s.
Amyloid plaques form from abnormally degraded amyloid precursor proteins. they form plaques, leading to degradation and inflammation.
Inside - Tau proteins tangle, causing atrophy and decreased ACh
What are the main Risk Factors for developing Alzheimer’s?
Age
female
Vascular (DM, HTN, Dyslipidaemia, Diet)
Trauma
What is Chronic Traumatic Encepalopathy?
‘Punch Drunk Syndrome’
Acute head injury seems to increase inflammation and amyloid levels within the brain. Associated with late-life risk of Alzheimer’s.
How can you investigate a possible case of Alzheimer’s Disease?
CSF - High Tau Proteins, Low Amyloid.
CT/MRI show atrophy in late stages.
Tissue required for definitive diagnosis.
PET/SPECT are possible future diagnostic tools.
MMSE, MOCA
What is Vascular Dementia?
Neuronal death occurs due to infarction; multiple strokes damage the small and medium sized vessels of the brain.
As such, risk factors closely match those of CVS disease.
Often displays stepwise progression.
What is Pick’s Disease?
Form of dementia only affecting the frontal and temporal lobes.
Only caused by tau proteins, not amyloid plaques.
How does Pick’s Disease present?
Personality Changes
Disinhibition
Overeating (sweet foods)
Emotional Blunting
Relative preservation of memory v other dementias
Presents earlier (40-60)
What is Wernicke’s Encepalopathy?
Thiamine (B1) Deficiency leading to acute neurological signs.
Triad = Ataxia, confusion, eye signs
Typically occurs in malnourished alcoholics.
What are the eye signs seen in Wernicke’s Encepalopathy?
Opthalmoplegia
Nystagmus
Diplopia
Ptosis
What are the main causes of Wernicke’s Encepalopathy?
Alcoholism
Malnutrition
Bariatric Surgery
Dialysis
How would you investigate a patient with suspected Wernicke’s Encepalopathy?
Serum Albumin and Vit B1
ECG
CT
Neuropsychology
How would you manage a patient with confirmed Wernicke’s Encepalopathy?
Immediately give Pabrinex (B1)
What is Huntington’s Disease?
Autosomal Dominant neurodegenerative disorder causing both motor and cognitive symptoms.
How does Huntington’s present?
Motor
Chorea - Rapid jerky movements
Athetosis - Writhing hand movements
Ataxia
Dysphagia
Cognitive
Lack of concentration
Depression
Dementia
Personality changes
What causes the symptoms of Huntington’s?
Atrophy of the caudate and putamen.
Global atrophy seen in advanced disease.
How would you investigate a suspected case of Huntington’s Disease?
CAG codon repeat testing.
The more CAG repeats, the more likely the patient to develop the disease.
MRI/CT - Caudate/Striatal Atrophy in advanced disease.
