Flashcards in Week 3 Deck (96):
What inheritance is DMD?
What inheritance is Huntingtons disease?
What age of onset does huntingtons disease usually have?
30 - 50 years
Name two clinical features of huntington disease?
What are these early clinical signs of - clumsiness, agitation, irratability, apathy, anxiety, disinhibition, delusions/hallucinations, abnormal eye movements and depression?
What gene is mutated in HD?
Name three features of the pathology of alzheimer disease?
Loss of cortical neurones
Neurofibrillary tangles (intracellular)
Senile plaques (extracellular)
What is the term for extracellular protein deposits containing amyloid beta protein?
What type of meningitis shows a thick layer of suppurative exudate covering the leptomeninges over the surface of teh brain?
Pyogenic meningitis (exudate in basal and convexity surface, neutrophils in subarachnoid space)
What viruses tend to cause viral meningitis?
Enteroviruses e.g. ECHO
How do you diagnose viral meningitis?
Viral stool culture, throat swab and CSF PCR.
How do you treat herpes simplex viral encephalitis?
Aciclovir IV high doses
What is the meningism triad?
nuchal rigidity (neck stiffness), photophobia (intolerance of bright light) and headache.
In neonates - what are the three common causes of bacterial meningitis?
2. Group B streptococci
In children - what is the commonest cause of bacterial meningitis?
21 onwards - what is the c ommonest cause of bacterial meningiti?s
In the elderly what are the two common causes of bacterial meningitis?
Pneumococcal and listeria
Name a risk factor for getting pneumococcal bacterial meningitis?
Fracture of the cribiform plate
Name a risk factor for getting staphylococcus, gram negative rods, bacterial meningitis?
Name a risk factor for getting listeria bacterial meningitis?
What type of H.influenzae is the most common cause of meningitis in children under 4?
What are the antibiotics of choice for listeria?
(ceftriaxone no value as intrinsically resistant)
How do you treat cryptococcal meningitis?
IV amphotericin B/Flucytosine
Fever, stiff neck and alteration in consciousness?
When should you do a lumbar puncture for bacterial meningitis?
Only if feasible, treat with antibiotics first
What is the Glu (CSF/SERUM) for CSF predictive of bacterial meningitis?
less than 0.23
Aseptic meningitis describes a spinal fluid formula that typically has what?
1. Low number of WBC
2. Minimally elevated protein
3. Normal glucose
In relation to acute adult bacterial meningitis - what should be done on all patients with papilloedema or focal neurological signs?
Should they undergo CT prior to lumbar puncture for meningitis in - immunocompromised state?
Should they undergo CT prior to lumbar puncture for meningitis in - patients with mass lesion or stroke?
Should they undergo CT prior to lumbar puncture for meningitis in - new onset seizures and papilledema?
What is the empiric antibiotic therapy for bacterial meningitis?
1. IV ceftriaxone
2. Add IV ampicillin/amoxicillin if listeria
3. If allergy - chloramphenicol IV with vancomycin IV
4. If allergy - cotrimoxazole
In relation to bacterial meningitis - what do you do with steroids?
Give to all patients suspected of it before or with first dose of antibiotics
What has prominent dopaminergic neuron loss in the substantia nigra pars compacta with alpha-synuclein-containing Lewy bodyies and Lewy neurites?
Rigidity, akinesia/bradykinesia and resting tremor?
Prolonged muscle spasms and abnormal postures
Fragments of movements flow irregularly from one body segment to another causing a dance-like appearance.
What are these non-motor features of - olfactory dysfunction, cognitive impairment, sleep disorders, pain and fatigue?
How do you diagnose rapid eye movement sleep disorder?
How do you treat rapid eye movement sleep behaviour?
Melatonin at bedtime
Where is Lewy body pathology found other than brain?
Spinal cord and peripheral nervous system
What are misfolded alpha-synuclein which is insoluble and aggreagated, forms intracellular inclusions?
What are a neurohistological hallmark of PD?
What is teh greatest PD risk factor?
What is teh dominant PD gene?
What is the recessive PD gene?
What is teh greatest genetic risk factor fort PD?
Mutations in GBA
Name a symptomatic treating drug for PD?
1. Levodopa - dopamine agonist
What can be effective for tremor in PD?
Anticholinergic agent - trihexyphenidyl or clozapine
Naem three side effects of dopamine agonist levodopa?
Nausea, daytime somnolence and oedema
What drug causes gamblong, hypersexuality, binge eating and compulsive spending?
How is psychosis in PD managed?
What is late stage demeintia in PD trated with?
What type of tremor is pill rolling?
Where is cog wheel rigidity normally felt?
What type of parkinsons affects predominantly lower limbs, rest tremor uncomon, brain lesions might be present, poor levodopa response?
What type of parkinsonism tends to be symmetrical, often coarse postural tremor?
Name a common cause of degenerative parkinonism?
Multi system atrophy
Dysautonomia, cerebelalr features, parkinsonism?
Multi system atrophy
What sign is seen on MRI in multi system atrophy?
Hot cross bun sign
What is symmetric akinetic rigid syndrome with predominantly axial involvement?
Progressive supranuclear palsty
What type of injury causes relase of excitatory amino acids, binding to receptors, relwease of intracellular calcium, activation of phospholipases, cell swelling and apoptosis?
Primary head injury
What does MAP - ICP =
CPP (cerebral perfusion pressure)
Raccoon or panda eyes?
Anterior cranial fossa skill base fracture
Battle sign over mastoid area?
Middle cranial fossa skull base fracture
What three categories make up GCS?
Best motor response
4 eye opening stages in GCS?
2. To command
3. To pain
5 verbal response stages in GCS?
3. Inappropriate words
4. Incomprehensible sounds
6 best motor response stages in GCS?
1. Obeys command
2. Localises pain
3. Flexes to pain
4. Abnormal flexion
Do not open eys
Do not obey commands
Do not speak
GCS less than 8
When should you do CT scan in head trauma?
Any patient with skull fracture
Not orientated GCS less than 15
Focal neurological signs
Taking anti-coagulants (warfarin)
During intensive care management of head injury - what is done to reduce cerebral metabolic rate, reduce cerebral blood flow and reduce ICP?
During intensive care management of head injury - what is done to maintain adequate oxygenation and maintain normocapnia?
During intensive care management of head injury - what is done to manipulate to maintain CPP of more than 60?
During intensive care management of head injury - what is done to maintain normoglycaemia?
During intensive care management of head injury - what is done to maintain euthermia?
Give three late effects of head injury?
Rhythmic sinusoidal oscillation of body part?
Involuntary sterotyped movements or vocalixations?
Brief irregular purposeless movements which fit and flow from one body part to another ?
Brief electric shock like jerks?
Abnormal posture of the affected body part?
Give two causes of a kinetic tremor?
1. Cerebellar disease
2. Wilsons disease
What investigation might you do for tremor in a young patient?
Thyroid function test and copper + coeruloplasmin
Give two first line treatment options for dystonic tremor?
What three main physiological abnormalities have been found in patients with dystonia?
1. Loss in reciprocal inhibition
2. Alterations in brain plasticity
3. Alterations in sensory function
What condition starts before age of 28, usually childhood, starts in limb, usually legs and often family history present?
DYT1 - torsion dystonia
What surgery is now procedure of choice in dystonia?
Deep brain stimulation - but after botox trial
Name an inherited cause of chorea?
Name an autoimmune cause of chorea?
Autosomal dominant inherited neurodegenerative disorder characterized by progressive behavioural disturbance, dementia, and movement disorder, usually chorea.
What is age of onset of tourette syndrome?
Less than 18
What is symptomatic treatment for tourette syndrome?
Clonidine and tetrabenazine
What conditions has typical precipitatns of the myoclonic jerks and seizures being alcohol and sleep deprivation?
Juvenile Myoclonus Epilespy
What does EEG show for Juvenile Myoclonus Epilepsy?
3-5 Hz polyspike and wave pattern