Neuro Flashcards
(73 cards)
1
Q
What is the difference between focal and generalised seizures?
A
- Focal - specific neuro deficit within one region (e.g. temporal) with retained/impaired consciousness.
- Generalised - simultaneous involvement of both hemispheres with LoC.
2
Q
What is Jacksonian March?
A
A type of focal seizure of abnormal electrical activity in the frontal lobe where clonic movements (contracting and then relaxing) start in one limb and move proximally through the body.
3
Q
Seizures can be classified by 3 modes - what are they?
A
- Seizure typeLocation (focal/generalised) & motor/non-motor onset
- Level of awareness aware/impaired awareness
- Other features
4
Q
List the 5 main epilepsy childhood syndromes according to onset:
* 6mo
* 6-10mo
* 1-5yrs
* 6-8yrs
* Teenage
A
- West syndrome (full body spasms, poor prognosis, 4-8mo)
- Dravet’s syndrome (prolonged febrile convulsions)
- Lennox-Gastaut syndrome (similar to West syndrome but presents later)
- Benign rolandic epilepsy (focal, facial/tongue/speech paraesthesia often in morning, most common)
- Juvenile myoclonic epilepsy (full myoclonic seizures often following sleep deprivation)
5
Q
What are the EEG changes expected in West’s, Lennox Gastaut’s and Benign Rolandic Epilepsy?
A
West’s - hypsarrhythmia
L-G - slow spikes
BRE - centrotemporal spikes
6
Q
Epilepsy
State the investigations you would do/consider
A
- Bloods - glucose, U&E
- EEG
- ECG
- MRI Brain
- Other - LP, urine cultures, ?septic screen
7
Q
Management for seizures
- community
- hospital
Define status epilepticus
A
-
Community: Rectal Diazepam or Buccal Midazolam
* Hospital: IV Lorazepm (–> repeat after 10 mins if continue) –> IV Phenobarbitone –> I+V. - Prolonged seizure for >5 minutes or 2 seizures without regaining consciousness in between.
8
Q
3 crtiteria for a brain MRI for investigatiing seizures
A
- If seizure happened before age of 2
- If unresponsive to 1st line anti-epilpetics
- Focal seizures
9
Q
Usually anti-epileptics are started after the second seizure. NAme the 4 criteria for reasons to start after 1st sezirue.
A
- Type of seizure:Focal
- EEG findings: unequivocal activity
- Further seizure is perceived an unacceptable risk
- MRI findings: structural abnormality
note after first seizure cannot drive for 6 months (need to inform DVLA)
10
Q
Anti-epileptic 1st and 2nd lines for
- generalised motor
- focal
- Absence
- myoclonic (type of generalised absence)
A
For male (*Sodium Valproate CI for women at reproductive age)
- (1) Sodium valproate (2) Lamotrigine/Levetiracetam
- (1) Lamotrigine/Levetiracetam (2) Carbamazepine
- (1) Ethosuximide (2) Sodium Valproate/Lamotrigine
- (1) Sodum valproate (2) Levetiracetam
Generalised –> generalised absence –> generalised absence myoclonic = C –> E –> L. As the seizure type gets more specific the second line drug is later in the alphabet.
11
Q
Treatement for West Syndrome
A
Prednisolone (reduces spasms) / Vigabatrin (inhibits breakdown of GABA)
12
Q
MOA of
- Sodium Valproate (pleiotropic)
- Carbamazepine/Lamotrigine/Phenytoin
A
- Increased activity of GABA and Na+/Ca2+ channel blockers (stabilsing membrane potential)
- Na2+/Ca2+ channel blockers
Sodium valproate is the boss drug of epilepsy so makes sense to have multiple mechanisms …..and to be for men (not to be sexist)
13
Q
Which drug is teratogenic & SEs include liver damage, hair loss & tremor
A
Sodium Valproate (avoid in woman of reproductive age)
14
Q
Which drug is a P450 inducer & can cause SEs of agranulocytosis and aplastic anaemia
defiency in granulocytes (basophils, neutrophils and eosinophils) -severe form of neutropenia
A
Carbamazepine
15
Q
Phenytoin’s SEs are folate and vitamin D deficiency - what can this lead to?
A
Megaloblastic anaemia and osteomalacia respectively
16
Q
3 side effects of Lamotrigine
A
Stevens Johnson syndrome (rash –> shedding of skin), DRESS syndrome, leucopenia
drug reaction wth eosinophilia and systemic symptoms - rash but with organ invovement (lymphadenopathy, fever)
17
Q
Define status epileptics and its immediate treatment.
What are the 2nd, 3rd and 4th line options if this fails?
A
Seizure lasting >5 mins or 2+ seizures in 1hr.
1) A-E; IM Lorazepam
2) IV Lorazepam (repeat after 10 mins)
3) IV Phenytoin
4) Thiopentate sodium (anaesthesia)
18
Q
How do you treat status epilepticus in the community? (Buccal or rectal)
A
Buccal Midazolam
Rectal Diazepam
19
Q
Clinical features of a cluster headache
Prophylaxis - 1st line
Treatment
A
- Severe onset, localised, severe pain
- repeated episodes (clusters of attacks) –> disappear –> reappear
- acute episodes approx 0.5-2hrs long, often same time each day over a few weeks
- red, swollen, watery, miotic eye
- sweating, nasal discharge
Prophylaxis
- Verapamil
Treatment
- Sumatriptans SC
Oxygen
Differential: SAH, migraine, venous sinus thrombosis, GCA
20
Q
4 types of migraine
A
With aura
Without aura
Hemiplegic migraine (stroke mimic)
Silent migraine (aura, no headache)
21
Q
Prophylactic medical treatments for cluster headaches (3) vs migraine (3)?
A
Cluster - Verapamil (CCB), Lithium, Prednisolone
Migraine - Propanolol, Topiramate (teratogenic), Amitryptiline
22
Q
Medical treatment options for cluster vs migraine (acute & prophylactic options)
A
Cluster - Triptans (S/C); O2
Migraine - Triptan+ NSAID/paracetamol.
Migraine prophylaxis - Topiramate or Propanolol.
23
Q
Class of triptans
A
5HT receptor agonsits; MOA not fully known but may reduce peripheral pain signals
24
Q
Clinical features of meningitis vs meningococcal septicaemia
Most common cause of viral meningitis
A
Meningitis = headache, neck stiffness (in some), photophobia, fever, vomiting, seizure (bacteria/virus in CSF around brain and spinal cord)
Meninogococcal septicaemia (meningococcus in bloodstream) = presence of non-blanching haemorrhagic rash
Viral meningitis - enteroviruses like coxsackie
25
Investigations for meningitis in hospital (3 main ones)
1. **Lumbar puncture**- CSF analysis (culture, **neutrophils,** glucose, protein, PCR)
2. **Blood glucose** (to compare against CSF glucose)
3. **Bloods - Meningococcal PCR** (quicker than culture)
## Footnote
note neutrophils suggestive of bacterial meningitis rather than other causes like HSE.
26
Community vs hospital management for meningitis?
**Community** - suspected meningitis + non blanching rash--> start Benzylpenicillin and immediate transfer to hospital.
**Hospital**
- abx as per trust guidelines and dependent on age
+/- steroids dependent on LP results
- fluids and cerebral monitoring
27
Abx treatment for bacterial meningitis; how does it vary for children <3 months?
**<3 months: Cefutaxime + Amoxicillin** *(to cover for listeria contracted during pregnancy)*
**>3 months: Ceftriaxone** (because Cefutaxime can cause dangerous increase in bilirubin)
| or according to local guidelines
28
Secondary causes of headache - intracranial (list 5), extracranial (list 5)
**Intra** = meningitis, haemorrhage (SAH/ICH) venous sinus thrombosis, temporal arteritis/GCA, raised ICP
**Extra** = acute angle closure glaucoma, pre-eclampsia, encephalitis, carotid artery dissection, sinusitis
29
Raised ICP: presentation & examination findings
**Presentation**: worse in morning with coughing & bending. Nausea & vomiting. Reduced GCS. Visual disturbance/neurological syx/seizures.
**Examination**: Reduced GCS. Pupil dilation. Papilloedema. CN III palsy (late). Cushing's (if herniated). Cheyne Stokes respiration.
30
Herpes simplex encephalitis is most common cause of sporadic encephalitis.
The virus typically affects **which lobes**, causing which typical symptoms?
Ix: CT head & LB. Findings?
Mx?
* Temporal lobes
* Headache, fever, seizure
* Focal sx - aphasia
* CT - Hypodensities/ptetchial haemorrhages in temporal lobes (aka **bitemporal lobe changes**)
* LP - raised WCC & proteins.
* IV Aciclovir
31
Risk factors for venous sinus thrombosis?
Hypercoaguable state (pregnancy)
32
Venous sinus thrombosis - presentation, investigations & management
**Presentation**: headache, N&V, seizures, features of raised ICP, focal neurology.
**Investigations**: CT venography
**Management**: LMWH + antibiotics (high risk of infection)
33
GCA presentation, investigations(3) and managementy - how does this vary for if there is visual involvement?
**Presentation**: over 55, temporal headache, scalp tenderness & jaw claudication, ipsilateral blindness, papilloedema.
**Investigations**:
1. Bloods - raised ESR,
2. Duplex temporal artery - (assess speed and volume of blood) - "halo" sign, stenosis
3. Biopsy temporal artery - multinucleated cells/skiplesions
**Management**: high dose glucocorticoids
Evolving visual defect: Methylpred then Pred
No visual defect: Pred
34
Primary causes of headache
Tension headache
Cluster
Migraine
35
Typical presentation of neurogenic thoracic outlet syndrome?
Pressure on bracchial plexus --> wasting of thenar eminence, hand weakness (worsened by raising above head), +/- paraesthesia +/- autonomic symptoms
36
Typical presentation of vascular TOS?
vascular: pressure on subclavian artery or subclavian vein.
Artery - claudication.
Vein - arm swelling.
37
**Features of Tremor**
* Parkinsonism
* Benign Essential Tremor
* Cerebellar disease
* Anxiety
* Thyrotoxicosis
* Hepatic encephalopathy
* CO2 retention
* Renal failure (uraemia)
* Asymmetrical, resting tremor ("pill rolling"), relieved by voluntary movement. Associated with bradykinesia / problems with dexterity/fine movement
* Symmetrical, alleviated by alcohol and rest, worse if arms outstretched.
* Intention tremor, impaired finger-nose test.
* Symmetrical, worse under stress. Hx of MH.
* Associated with thyrotoxic signs
* Asterixis (flapping tremor) - bilateral, postural. Hx of CLD.
* Asterixis (flapping treor) - bilateral, postural. Hx of COPD.
* Same
38
**Motor Neurone Disease**
* brief definition
* subtypes, relative prognosis and whether UMN/LMN signs are affected (4)
* risk factors (3)
* sx presentation and important negatives (3)
* only licensed treatment available:
* MND is a neurodegenerative condition twhich ultimately results in paralysis.
* ALS (50% of cases, both UMN/LMN sx, 2-5 yr prog)
* PMA (7-8yrs, LMN)
* PLS (10 yrs, UMN)
* PBP (<1 yr, both)
* Genetics (10%), idiopathic, physical activity, male, increasing age
* Progressive muscle weakness or bulbar symptoms. Negatives - spares ocular & pelvic floor muscles - x oculomotor disturbance, x incontinence - until late stage.
* Riluzole (glutamate antagonist)
39
Distinguish **bulbar palsy** from **pseudobulbar palsy**
Both affect the cranial nerves 9,10,11,12 found on the medulla of brainstem - involved in function of muscles of face, mouth and tongue. Both cause **tongue muscle wasting +**
* Bulbar palsy- LMN signs - fasciculations
* Pseudobulbar palsy - UMN - exaggerated jaw jerk relfex, tongue spasticity
40
**Multiple scerlosis**
* Definition
* Disease patterns (4)
* Risk factors (3)
* General presentation, and common types (3)
* Diagnosis (2) and investigations (2)
* Management - MDT & Neuro rehab team. Long term treatment class and medx for relapse
* First line treatment options for spasticity (2)
* A chronic progressive condition caused by demyelination and plaque formation of the CNS neurons. Often autoimmune attack against the myelin sheath.
* Clinically isolated episode (technically not MS yet)
* Relapsing-remitting (most common)
* Secondary progressive (R/R then continue progressing)
* Primary progressive (without R/R)
* Female sex, EBV, genetics, smoking, obesity
* present with variable sx depending on which part of CNS is affected (5 - motor, sensory, visual, cerebellar, autonomic)
* Based on CNS lesions "disseminated in time & space & greater than 1 yr in nature.
* MRI Brain & spial cord - plaques.
* LP - oligoclonal bands (antibodies) in CSF.
* **DMARDS**
* **Corticosteroids** for relapse (Methylpred)
41
Multiple-sclerosis: management
**To reduce relapse: DMARDS**
- types and general mechanism
**To treat relapses: croticosteroids**
- type
- **Symptomatic treatment**
- treatments for 5 main symptoms
**Conservative**
- Exercise, weight loss, smoking cessation
- (physio, OT)
* *Fingolimod* (S1P modulator) and *Natalizumab* (anti-A4B1) - biologic therapy- target specific leucoytes/interleukins to reduce immission.
* *Methylprednisolone*
*- Limb spasticity - *Baclofen/Gabapentin* & physiotherapy
- Fatigue - *Amantine* & CBT
- Depression - *SSRIs* & CBT
- Urge incontinence - *anticholinergics*
- Neuropathic pain - *Amitryptiline/Gabapentin*
42
**Visual effects**
- Optic neuritis (3 key sx, examination finding?, cause other than MS)
- Internuclear opthalmoplegia
- Conjugate gaze palsy (CN 6)
- Double vision
- Uhthoff's
**Optic neuritis**
1. **Visual **- cloudy vision, red desaturation, central scotoma
2. **Pain** - worse on mvmnt
3. **RAPD**
4. **O/E** - papilloedema
**Cause** - MS, syphillis, autoimmune conditions (SLE, DM), viruses (measles, mumps)
**INO**
damage to medial longitudinal fasciculus between CN3 (oculomotor) and CN6 (abducens)
- inability to adduct affected eye and horizontal nystagmus of opposite eye
**Conjugate gaze palsy**
inability to both both eyes together in straight line
**Double vision**
noted on H test (CN I)
**Uhthoff's**
visual changes are worsened by rise in body temp
43
Internuclear opthalmoplegia
* Demyelination or any damage to the MLF - medial longitudinal fasciculus (connection between CN3 and CN6)
* inability to **adduct eye of affected side** and nystagmus of other eye
44
Transverse myelitis
-definition; trigger
- symptoms below the level of the cord?
- L'hermitte's sign?
- treatment
* Focal inflammation extending across the spinal cord - triggered by infectious or autoimmune process
* Motor and sensory sx below level of cord
* E.g. L'hermitte's sign = sensory dissturbance (electric shock down back of neck/back - due to demyelination within cervical spinal cord) and tight-band sensation around trunk
* *Dexamethasone*
45
Wet, wobbly & wacky
(urinary incontinence, recurrent falls and dementia) is the classical triad for what?
What causes it?
Risk factors?
What therefore would you not expect?
Typical finding on cxr - or not a finding?
Mx?
**Normal pressure hydrocephalus**
* reversible dementia
* reduced absorption of CSF at the arachnoid villi -->progressive fluid build-up in brain -> develop sx over few months as large ventricles obstruct brain tissue
* increasing age, head injury, stroke, CNS infection
* x papilloedema/raised ICP
* cerebral imaging shows ventriculomegaly with lack of sulcal enlargement (aka ventricles are bigger but not causing raised ICP)
* mx - **ventriculoperitoneal shunt**
46
**Gait abnormalities** - cause, features
* Hemiplegic
* Diplegic
* Parkinsonian
* Ataxic
* Neuropathic
* Myopathic
* Antalgic
**Involuntary movements**
* Choreiform
* Akathitosis
* Ballismus
* **Circumduction.** UMN lesion (e.g. stroke) - spastic weakness of lower limbs.
**Scissoring**. (overlapping of lower limbs). Bilateral CVA (stroke), spinal cord lesion, MND.
* **Shuffling** --> festinant gait w slow initiation, reduced stride length, reduced arm swing, and stooped posture. PArkinson's disease/PD+/drugs/viral.
* **Broad based staggering.** Problem with balance: cerebellum, vestibular system or proprioception. E.g. alcoholic cerebella dysfunction, labrynthitis, sensory ataxia from spinl cord lesion/DM.
* **High steppage** foot drop/toe dragging. - weakness in distal muscles due to peripheral neurapathy. Common peroneal neuropathy (L4,L5,S1) - e.g. L5 radiculopathy or polyneuropathy/CMT.
* **Waddling;** weakness of hip abductors (minimus/medius). Pelvis dips on unsupported side. Positive Trendelenberg.
* **Abnormal gait due to pain** - e.g. #, osteoarthritis, RA.
* **Chorea** - Involuntary dance-like movements. Huntington's/Sydenham's.
* **Athetosis** - slower form of chorea - continuous, writhing movements ("without position)
* **Ballismus** - severe form of chorea - with violent flinging of limbs
47
Intracranial venous thrombosis: *includes sagittal sinus, cavernous sinus thrombosis and lateral sinus thrombosis*. It can result in a stroke/CVA.
- risk factors
- presentation (signs of raised ICP)
- ix and typical finding if sagittal sinus thrombosis
- mx
- hypercoaguable state, smoking
- headache, N+V, LoC
- MRI venography: "empty delta sign"
- anti-coagulants: LMWH
48
Intracranial hypertension
- risk factors
- presentation
- ix
- mx - lifestyle and medical
- fenale sex, obesity, COCP use, pregnancy, other drugs
- headache worse on bending forward, signs of raised ICP, papilloedema, enlarged blind spot
- MR venogram: "Empty sella sign"
- Weight loss
- Acetazolamide to reduce production of CSF
- +/- topiramate (also beneficial for weight loss)
- surgical options e.g. ventriculoperitoneal shunt
49
**Parkinson's plus syndromes**
- Multisystem atrophy (3 main features)
- Dementia with lewy bodies (distinction from PD)
- Progressive supranuclear palsy (PSP)
- Corticobasal degeneration
**Other causes of parkinsonism:**
- Drugs
- Infection - viral
- Wilson's disease
- Trauma
- Toxins
* basal ganglia (PD), cerebellum (ataxia) and autonomic dysfunction
* cognitive decline prior to motor symptoms and often fluctating congition +/- visual hallucinations
* PSP: impaired vertical gaze, instability/falls, parkinsonism, cognitive decline
*
50
Parkinson's medication
**1st line:**
Levodopa
- medicinial forms - what else is co-prescribed and why?
- side effects
**2nd line or 1st line if motor symptoms aren't main problem**
Example:
- Dopamine agonists
- MAO-B inhibitors
Adjuncts:
- Antimuscarinics
- COMT-Inhibitors
**Levodopa**
- Addition of a decarboxylase to prevent breakdown of dopamine before it reaches the brain
- Co-beneldopa (Levodopa and Benserazide)
- Co-careldopa (levodopa + Carbidopa)
- ses: dyskinesias
**Dopamine agonists**
- Bromocriptine
- Pergolide
- Carbergoline
**MAO-B inhibitors**
- Rasagline
- Selegiline
*Adjuncts*
**COMT-Inhibitors**
...capones
**Antimuscarinics**
to help DIP primarily for tremor and rigidity
Procyclidine
Benzotripine etc
51
**Bell's palsy; cranial nerve 7 palsy**
* Risk factors
* Presentation
* Management
Risk factors:
- Pregnancy
- 20-40yrs
- ? herpes simplex infection
Px - Sudden onset of
- Ipsilateral paralysis of face including forehead (LMN)
- Hyperacusis/ear ache (facial nerve supplies stapedius muscle)
- Loss of taste (CN VII- anterior 2/3 of tongue)
Mx:
- Prednisolone PO within 3 days
- Eye care (tape closed at night, artificial tears ifn necessary)
-
52
Herpes simplex encephalitis
- typically affects which lobes?
- typical causative organism?
- Presentation
- Investigations (LP), viral PCR, CT/MRI Brain, EEG - findings
- Mx
* temporal lobes
* HSV type 1
* meningism, seizures, coma
* LP: raised lymphocytes, normal protein, normal glucose
* CT: ptechiae, MRI brain - hyperintensity of temporal lobes, EEG - lateralised periodic discharges at 2Hz
* IV Aciclovir
53
**Myasthenia gravis**
Autoimmune condition of the NMJ, autoantibodies to acetylcholine receptor on post synaptic cleft of NMJ
- Presentation
- Fatiguability in muscles can be eliicited by (3)
- autoantibodies (most likely to least)
- ix name
- Positive test if ....
- additional ix if thymoma suspected (strong link)
- mx (2)
- if initial treatment not effective consider
- differentials
- **face**Facial/oculomotor problems - ptosis, diplopia, dysarthia, dysphagia;
- **limbs**Proximal limb weakness; fatiguability
- prolonged blinking --> ptosis; prolonged upward gazing --> diplopia, repeated arm abduction --> unilateral weakness
**autoantibodies** - anti: ACH-R (85%) antiMUSK (muscle specific tyrosine kinase antibodies)
**Ix**
- single fibre electromyography: high sensitivity (>92%)
- CT/MRI thorax - thymus
- Edrophonium test / or neostigmine: IV dose of antiacetylcholinesterase enzymes (blocks breakdown of Ach)
- positive if symptoms improve (temporary rise in ACh at NMJ relieves weakness)
**Mx**
- reversible acetylcholinesterase inhibitors - *Pyridostrigmine/Neostigmine* & immunosuppression eventually - reduce production of autoantibodies *(Prednisolone, Azathioprine etc)*
- ?mABs - *Rituximab*
- thymectomy
- Lambert-Eaten syndrome (NSCLC; squamous), cranial nerve III palsy, Guillan Barre syndrome/Miller Fisher syndrome starts in eyes, myopathy, muscular dystrophy (in MG the CK level is normal unlike in the latter)
54
What triggers a myasthenic crisis?
What are patients at risk of?
Treatment (2)
* Infection, stress, trauma, menstruatiom
* Respiratory failure due to weakness of resp muscles
* Iv IGs or plasma exchange (plasmapharesis)
* +/- NIV if severe
55
**Muscular dystrophies** - genetic conditions causing gradual muscle weakness/wasting
- Paeds: Duchenne's/Becker's
- Adults: Myotonic dystrophy
- Other rare forms .....
**Duchenne's/Becker's**
- genetic inheritenace of both
- mutation on gene Xp21 which codes for?
- which is the more severe form?
- Ix - raised CK; definitive diangosis by?
- Mx -??
- Associated with which condition
**Duchenne's**
- Presentation and age of onset
- positive sign on examination
- signs other than myopathy
**Becker's**
- key distinguishing points (2)
- X-linked recessive
- Dystrophin - endodess for msucle protein
- Duchenne's: frameshift mutation; becker's - non frameshift
- Duchenne's at 5, becker's at 10
- Genetic testing; definitive
- Steroids to slow progression and creatine supplements
- poor prog, most cannot walk by age 12
- dialted cardiomyopathy
**Duchenne's**
- Gower's sign positive = use arms to stand from squat
- Calf psuedohypertrophy
- progressive proximal muscle weakness from age 5
- intellectual impairment may be present
**Becker's**
- develops after 10 years
- rarely have intellectual impairment
56
**Myotonic dystrophy**
type of inhertied muscular dystrophy more common in adults (2 types)
- characterising features
- on examination
- associated with which ocular condition (req opthalmoscope)
- myotonia and slurred speech (myotonia of pharyngeal/oral muscles)
- on clenching fist and opening it, slow muscle relaxation
- normal sensation, normal reflexes
- symmetrical weakness proximal>distal
- cataracts
57
**Polymyositis**: DOE
- requires ruling out other causes of myopathy (endocirne, electrolyte, toxic etc)
It is an inflammatory myositis due to underlying causes - autoimmune, infection, drugs (statin), CTs
- what cancers is dermatomyositis associated with?
- Bloods - what is raised?
- Diagnosis obtained from..?
- Mx
- ovary,GI,breast
- CK raised
- muscle biopsy
- steroids
58
**Subacute combined degeneration of the cord **
- Causes
- Presentation (3)
- SAD therefore
- Like MS it can also cause which sign on neck flexion?
- Typical example patient
- Vitamin B12 (pernicious anaemia) or Vitamin E deficiency - degeneration of the spinal cord due to demyelination
- **spastic paresis**, bilateral- UMN signs below level of cord (x corticospinal) - (but note can have absent ankle reflexes - doesn;t fit with rest)
- **loss of vibration/proprioception**, bilateal ( xdorsal columns)
- **Ataxia**, bilateral Romberg's +ve
- SAD - still in pain (spinothalamic intact)
- L'hermitte's sign
*Post-Chron's op, presenting with paraesthesia in toes and fingers, clumsiness and ataxia*
59
**Friedrich's ataxia**
*most common inherited form of ataxia, presents similarly to SADCD*
- General presentation
- Affects what other system due to mitochondria dysfunction?
-Spastic paresis, ataxia, and loss of vibration/proprioception
- also note INTENTION TREMOR
- NS, heart and pancreas
60
Central cord syndrome
- cause
- presentation
- hyperextension of neck, e.g. car injury
- most common form of incomplete cord lesion
- Bilateral spastic paresis UL>LL
- +/- sensory involvement
61
Complete spinal cord lesions have no function below site of injury. Incomplete have some function (motor/sensory) below site.
Types of incomplete spinal cord lesion (4)
- Brown-Sequard (unilateral signs)
- Central/cervical cord syndrome
- Anterior cord syndrome
- Posterior cord syndrome
-
62
Anterior cord syndrome
- caused by
- sx
- presentation (motor and sensory deficit)
- Anterior spinal artery occlusion (aortic dissection, sudden disc rupture, tumour compression etc)
- chest/back pain and spinal shock
- Bilateral spastic paresis and bilateral loss of pain/temp sensation
63
Syringomyelia
- CSF filled cyst in the spinal cord. Starts medially in cord and then expands outwards.
- Treat cause of syrinx or shunt.
- Risk factors
- Presentation
- Chairi malformation (cerebellar herniation); tumours, trauma, meningitis
- Cape-like distribution of loss of pain/temp sensation *(superheroes feel no pain)* damage to spinothalamic tract
- Spastic weakness LL>UL
-
64
Neurosyphillis: what is tabes dorsalis
- wasting of the dorsal columns
- loss of proprioception/vibration sensation
65
**Degnerative cervical myelopathy**
Risk factors
Presentation
What is Hoffmann's sign?
Investigation and finding
Mx - requires urgent insput from..
Definitive surgery
Differentials (2)
* Smoking, genetics, occupation (high axial loading)
* Neck or limb pain
* Loss of motor function +/- sensory/autonomic sx
* Impaired gait and balance
E.g. neck pain, loss of digital dexterity and clumsiness
- MRI Spine- disc degeneration and ligament hypertrophy
- Urgent neurosurgical assessment
- Decompressive surgery
**Differentials**
- Carpal tunnel syndrome
- Cervical disc prolapse
66
Cerebellar syndrome
- Symptoms (DANISH)
- Causes
**Symptoms**
Dysdiadokinesia
Ataxia
Nystagamus (horizontal)
Intention tremor, impaired finger-nose test
Slurred staccato speech
Hypotonia, impaired heel-shin test
**Causes**
- Friedrich's ataxia (most common hereditary cause: ataxia, spastic weakness, impaired dorsal columns + intention tremor. Affects heart & pancreas too)
- SACDC (ataxia, bilateral spastic weakness [UMN], impaired proprioception/vibration sense)
- Alcoholic liver disease
- Multiple sclerosis
- Pareneoplastic (lung cancer)
- Drugs: phenytoin, lead poisoning
- Cerebellar stroke
- Cancer - cerebellar haemangioma
- Ataxic telangectasia
- Multisystematrophy (parkinson's plus syndrome)
67
**Brain abscess**
May be caused by sepsis from middle ear/sinuses, trauma/surgery to scalp, penetrating head injury or embolism (endocarditis).
Presenting sx depend on site of abscess -other general signs:
Ix -
Mx - surgical and abx following
Px - fever, raised ICP signs (papilloedema, nausea, seizures), focal neurology, headache (dull, persistent)
Ix - non-contrast CT head
Mx - craniotomy + debridement
IV Abx - Cephalopsorin + Metronidazole
Manage raised ICP - Dexamethasone
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Facial nerve palsy
Supply - face, ear, taste, tear
Face - muscles of facial expression (motor)
Ear - nerve to stapedius (causing hyperacusis, ear pain)
Taste - supplies anterior 2/3 of tongue
Tear - paraysympathetic fibres to lacrimal glands
Causes of bilateral facial nerve palsy? (4 main)
Causes of unilateral?
**Bilateral**
- Sarcoidosis
- GBS
- Lyme disease
- Bilateral acoustic neuroma (Neurofibromatosis type 2)
- Bell's (unlikely)
**Unilateral**
LMN - all include forehead
- Bell's
- Ramsay hunt (VZV)
- Parotid tumour
- MS
- HIV
- DM
UMN
- stroke (forehead sparing)
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TIA: sudden onset focal neurology caused by brain, spinal cord or retinal ischemia, without acute infarction (generally features resolve in 1hr
Managenent;
1. Immediate antithrombotic
2. Specialist review with stroke team - urgent (criteria x3?)within 24hrs or within 7 days
Ix - recommend CT ehad not done unless clinical suspcion of alternative diagnosis. What is the preferred method ofimaging if required - same day or at specialist assessment?
Further management? Like stroke
1. Aspirin 300mg (unless bleeding disorder, on anticoagulant/low-dose aspirin anyway, contraindications)
2. Urgent if suspected **cardioembolic source/severe carotid stenosis/crescendo TIA.**
Review within 24hrs if suspected TIAin last 7 days, otherwise if longer can be seen wtihin 7 days
Imaging: MRI diffision weighted
Others: carotid imaging - doppler
Further management: anti-platelet (clopidogrel or aspirin & dipyridamole) and statin (atorvastatin 20-80mg daily)
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**Brain tumours**
* Presentation
* O/E - fundoscopy signs for raised ICP
Progressive focal neurological symptoms, signs of raised ICP and systemic signs, altered mental state, focal seizures.
**Papilloedema**= swelling of the optic disc, elevtated optic disc, haemorrhages, Paton's lines (folds in retina around optic disc), engorged retinal veins, loss of venous pulsation
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**Types of brain tumour**
1.**Gliomas** (arise from glial cells in brain/SC) - 3 main types from benign to most malignant:
2. **Meningiomas** (arise from meningeal cells): effect?
3. **Pituitary adenomas**(arise from pituitary gland) can be secretory/non-sec. Sx due to hormone excess? (Or depletion):
4. **Vestibular schwannoma/acoustic neuroma**: Nerve origin, location, association
**Gliomas:**
1. Ependymoma
2. Oligodendroglioma
3. Astrocytoma - most common/aggressive is *glioblastoma*
**Meningiomas:**
- Generally benign
- Mass effect rather than invasive
**Pituitary adenoma**
1. Cushings (excess cortisol)
2. Acromegaly (excess GH)
3. Hyperprolactinaemia (excess prolactin)
4. Thyrotoxicosis (excess TSH, thyroid)
**Vestibular schwannoma**
- CN 8 (vestibulocochlear)
- cerebellopontine angle
- unilateral s/n hearing loss, tinnitus, dizziness +/- facial nerve palsy (compression on CN7)
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Stroke mx
- Initial
- Intervention
- 2ndary prevention
Stroke & AF mx
-secondary preventiON
- how soon after stroke should this be started
*Initial* Aspirin 300mg stat (continue 2 weeks)
*Intervention* - thrombolysis - alteplase if within 4.5 hours of onset, thrombectomy - if last well between 6-24hrs ago, or if past 24 hours - bot (and if PCA/basilar artery or potential to salvage brain tissue)
*2ndary prevention: antiplatelets; lifelong*
1. Aspirin & dypridamole
2. Clopidogrel 75mg
+ Statin (atorvastatin after 48hrs)
If stroke + AF --> secondary prevention with anticoagulants, e.g. DOAC or Warfarin: apxaban AFTER 2 WEEKS
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**Malignant spinal cord compression**
- oncology emergency
- extradural compression from tumour on spinal cord (lung, breast and prostate most common)
- features
- urgent investigation within 24hrs
- management
- urgent oncology assessment for consideration of:
* Back pain
* Neurological signs - increased reflexes below lesion, reduced at level of lesion.
* Paraesthesia and lower limb weakness
* Urgent MRI Spine
* High-dose Dexamethasone oral
* Consideration for radiotherapy or surgery
