Neuro Flashcards
MND?
Neurodegenerative disorder that affects MN which control voluntary movement
Types of MND?
Amotrophic Lateral Sclerosis
Progressive Bulbar Palsy
Progressive Muscular Atrophy
Primary Lateral Sclerosis
What type of MND only affects the small muscles of the hands and feet?
Progressive Muscular Atrophy
What type of MND only affects UMN?
Primary Lateral Sclerosis
What type of MND affects only LMN of 9,10,12?
Progressive Bulbar Palsy
UMN signs?
Spasticty Babinksi Hoffman's reflex Brisk Reflexes Stiffness of upper movements Poor balance Weak upper limb extension + Lower limb flexion
LMN signs?
Fasciculations
Weakeness
Cramps
Reduces reflexes
Late stage signs of MND?
Bladder + Bowel incontinence
Oculomotor problems
Signs of MND?
Asymmetrical distal weakness
Brisk Reflexes
No changes in sensation or pain
Limb onset - Bulbar onset - Resp onset
Distribution of weakness seen in MND?
Asymmetrical distal weakness
Complication of MND?
Resp failure or Pneumonia
Diagnosis of MND?
Nerve conduction studies - fibrillation and fasciculations high amplitude and long duration
El Escorial criteria of UMN + LMN symptoms
CT/ MRI/ Bloods/ Muscle Biopsy - rule out
Criteria used to rate the severity of MND symptoms?
El Escorial
Management of MND?
Riluzole Ventilator PEG feed ACP Palliative care
Sign of cluster headache?
Severe pain + Unilateral lacrimation and redness of the eye lasting 1 to 2 hours
Rapid onset
Management of cluster headache?
Triptans
Short course steroids
Other: Verapamil, Topiramate or Lithium Carbonate
Migraine?
Recurrent unilateral headache associated with GI + Visual disturbance
Precipitating factors of Migraine?
Cheese OCP Caffeine Alcohol Anxiety Travel Exercise Noise Sleep Disturbances
Ddx of Migraine?
SAH, TIA, Meningitis
Management of Mild Migraine?
Paracetamol, NSAIDS, High dose Aspirin
Management of Moderate/ Severe Migraine?
Triptans
Migraine Prophylaxis?
Sodium Valproate
Propanolol
Amitriptyline
Botox
Management of recurrent migraines?
Pizotifen
Flunarizine
Pathophysiology of Trigeminal Neuralgia?
Vascular compression of the trigeminal nerve leading to central demyelination of the nerve root or MS, tumours, abnormalities of the skull base or AV malformations
Trigeminal Neuralgia?
Severe episodic facial pain in the distribution of 1 or more branches of the trigeminal nerve
Management of trigeminal neuralgia?
Carbamazepine post attack
GCA?
Chronic vasculitis characterised by granulomatous inflammation in the walls of medium/ large-sized arteries
Temporal or aorta + it’s branches
Signs of GCA?
Loss of vision Tender scalp Jaw claudication Systemic features Sore throat Hoarseness
Diagnosis of GCA?
History
Bloods - Normochromic Normocytic anaemia, Elevated ESR + CRP
Temporal artery biopsy
Management of GCA?
Prednisone
Diagnosis of a spinal cord compression?
MRI - Cause and site of compression
X-ray - Degenerative bone disease + destruction of the vertebrae
Management of spinal cord compression?
Surgical decompression + stabilisation of the spine
Dexamethasone
How does dexamethasone help decompress the spine?
Reduces oedema around the lesion
improves outcome in cord compression caused by malignancy
Cauda equina syndrome
Severe compression of the cauda equina - nerve roots L2 and beyond
Clinical features of Causa Equina syndrome?
Bladder + Bowel Incontinence
Saddle Numbness
Back Pain
Weakness in the legs
Ataxia?
Damage to the cerebellum causing patients to have symptoms that mimic being drunk
Friedreich’s Ataxia?
AR spinocerebella degeneration that affects spinal cord, heart and pancreas
GAA repeats on chromosome 9 so less frataxin made so build-up of iron in mitochondria leads to oxidative damage
Spinal cord becomes thinner + nerve cells lose myelin
Signs of Friedreich’s Ataxia?
Slurred speech Foot deformities Scoliosis Ataxia Cardiac arrhythmia Diabetes Loss of dorsal columns - absent proprioception + vibration Loss of vision and hearing
Cause of death in people with Friedreich’s Ataxia?
Hypertrophic cardiomyopathy
Age of onset of Friedreich’s Ataxia?
5 to 20 years old
Initial symptoms of Friedreich’s Ataxia?
Loss of proprioception and weakness moving upwards
Difficulty walking
Fatigue
Diagnosis of Friedreich’s Ataxia?
Genetic testing - expanded GAA repeat
MRI or brain + spinal cord
Echo
Management of Friedreich’s Ataxia?
Rehab + Walking aids
ACEi - Cardiac abnormalities
Surgery - slow down progression of scoliosis
Syringomelia or Syringobular?
Fluid filled cavities within the spinal cord or brain stem. Pressure forced cerebellar tonsils through the foramen magnum
Pain and sensory loss in upper limbs
Benign brain tumours?
Meningioma
Neurofibroma
Signs of brain tumours?
Mass effects depending on surrounding structures
Raised ICP
Epilepsy
Signs of raised ICP?
Headache Vomiting Papiloedema Made worse by coughing or sneezing Mydriasis Dyspnoea Decerebate or Decorticate posturing
Hydrocephalus?
Accumulation of CSF in the cranium by obstruction of CSF flow or increased CSF production
Normal Pressure Hydrocephalus?
Dilation of the ventricles + excess CSF without increased ICP
Elderly, Dementia, Urinary Incontinence, Ataxia
Communicating hydrocephalus?
Non-obstructive
Impaired CSF reabsorption in the absence of obstruction between ventricles and subarachnid space
Haemorrhage, Meningitis, Abnormal arachnoid villi
Non-communicating hydrocephalus?
Obstructive
Obstruction of either foramen of Monroe, cerebral aqueduct, fourth ventricle, foramen of Luschka + Foramen of Magendie
Hydrocephalus Ex Vacuo
Compensatory enlargement of CSF space in response to brain parenchyma loss and not increased CSF production
Brain atrophy, Schizophrenia, Post-traumatic brain injuries
Causes of hydrocephalus in children?
Arnold Chiari malformation Aqueductal stneosis Meningitis Haemorrhage Neural tube defects Brain tumour
Signs of hydrocephalus in babies?
Rapid head growth
Vomiting
Sleepiness
Seizures
Signs of hydrocephalus in adults?
Headaches Diplopia Poor balance Urinary Incontinence Personality change 3rd Nerve Palsy
Management of hydrocephalus?
External ventricular drain
Shunt between ventricles and right atria/ Peritoneum
Monro Kellie hypothesis?
Sum of the volume of the brain is constant. An increase of one compartment causes a decrease in the other
Brain tissue, CSF
+ Blood
Causes of raised ICP?
Cerebral oedema - Acute hypoxia or trauma Intracranial SOL ↑CSF production or ↓CSF absorption Non-communicating hydrocephalus Idiopathic intracranial hypertension
Diagnosis of raised ICP?
ICP monitor
CT
Management of raised ICP?
Propofol - sedate
Mannitol - osmotic diuresis
Prophylactic anticonvulsant
Decompressive craniotomy or remove SOL or Drain
Signs of idiopathic intracranial hypertension?
Papilloedema Retrobulbar pain Visual field loss Headache Pulsatile tinnitus Photopsia Diplopia Temporary visual disturbance
Diagnosis of IIH?
Headache + Papilloedmea in the absence of hypertension
↑Opening pressure on LP
Normal neural image + CSF composition
Management of IIH?
Treat symptoms and save vision Acetazolamide Furosemide Optic sheath fenestration Weight loss
MOA acetazolamide?
Carbonic anhydrase inhibitor that decreases CSF production
Mononeuropathy?
Compression of a single nerve affecting the muscle it innervates
Causes of carpal tunnel?
Compression, Entrapment or Direct damage
Signs of carpal tunnel?
Pain + paraethesia at night
Wasting + weakness of the thenar muscles
Management of carpal tunnel?
Nocturnal splint
Surgical decompression
Local steroid injection
What does B12 deficiency cause?
Polyneuropathy and subacute degeneration of the spinal cord
B12 deficiency signs?
Distal loss of proprioception and vibration
Absent ankle
Exaggerated knee + Babinski reflex
GBS?
Guillain-Barre Syndrome - post-infection inflammatory demyelinating polyneuropathy
Antibody-mediated nerve damage
Rapidly ascending symmetrical weakness
Miller Fisher Syndrome
Variant of GBS that affects cranial nerves of the eyes
Autonomic symptoms of GBS?
Postural hypotension
Cardiac Arrhythmia
Ileus
Bladder Dysfunction
Diagnosis of GBS?
Nerve conduction studies - Slow motor conduction due to demyelination
Neuro exam
LP - Raised protein + WCC
Management of GBS?
IVIg - decrease duration + severity of paralysis Plasmapheresis Monitor FVC PEG feed Heparin
What is a stroke?
Rapid onset neurological deficit lasting more than 24 hours
Causes of stroke?
Embolism Thrombosis AF Polycythemia Hypertension Atheroma Alcohol
Complications of stroke?
Haemorrhagic transformation of ischaemic stroke Cerebral oedema Seizures VTE Cardiac complications Infection Long term mobility problems Difficulties with ADL
Diagnosis of Stroke?
CT
MRI - any haemorrhagic transformation
Carotid doppler if suspected carotid stenosis
Tool used to classify the severity of stroke?
NIHSS - National Institute of
Health Stroke Scale - baso full neuro exam
15 item scale that measures the severity of stroke-related neurological deficits/monitors response to acute treatments
Management of confirmed ischaemic stroke?
Thrombolysis <4.5 hours (Alteplase) or Mechanical thrombectomy
or
300mg Aspirin followed by Clopidogrel if confirmed no haemorrhagic transformation
Secondary prevention of ischaemic stroke?
Dual platelet therapy for 3 months and lifestyle changes
Secondary prevention of ischaemic stroke in patients with AF?
Warfarin
HASBLED?
Risk of major bleed (intracranial bleed) in a patient with AF
Hypertension, Abnormal renal or liver function, Stroke, Labile INR, Elderly >65, Drugs or alcohol (antiplatelt or NSAIDS + more than 8 drinks weekly)
CHADVASC?
Risk of stroke in a patient with AF
BSC: Lacunar infarct?
Small vessel disease - occlusion of deep penetrating arteries
Pure sensory
Pure motor
Ataxic hemiparesis
Sensori-motor stroke
BSC: Posterior Circulation stroke?
Ipsilateral Cranial nerve palsy
Bilateral motor/ sensory deficit
Conjugate eye movements disorder e.g. gaze paresis
Cerebellar dysfunction
Isolated homonymous hemianopia or cortical blindness
Gaze palsy?
Inability of the eyes to move together on a single horizontal plane
Examples of cerebellar dysfunction?
Ataxia Nystagmus Vertigo Diplopia Dysarthria
BSC: TACS or PACS?
TACS - 3
PACS - 2
Unilateral weakness +/- sensory loss
Homonymous hemianopia
Dysphasia or Visuospatial disorder
TIA?
Focal neurological deficit lasting <24 hours due to ischaemia of blood vessel in the brain
Crescendo TIA?
2+ TIA in a week increasing the risk of stroke
Investigations of TIA?
Bloods - glucose, FBC, ESR, Creatinine, Electrolytes, Cholesterol, INR
MRI within 24 hours
ABCD2
Coronary artery CT or doppler?
ECG
ABCD2?
Risk of stroke after TIA Age >60 BP >140/90 Clinical features Diabetes + Duration
Management of TIA?
Aspirin or Clopidogrel (Wafarin if AF)
Secondary prevention - control DM + HTN
Surgery - Carotid endarterectomy
No driving for 1 month
Rfx of Intracerebral haemorrhage?
Hypertension Smoking Alcohol Increased age Aneurysm
Management of Intracerebral haemorrhage?
Stop anticoagulation
Control hypertension
Mannitol + Ventilation to decrease ICP
Subdural haematoma?
Accumulation of blood in the subdural space following rutpure of vein in saggital sinus
Headache, Drowsiness, Confusion
Banana CT
Surgical removal
Most at risk of subdural haematoma?
Atophic brains e.g. elderly or Alcoholics
Extradural Haemorrhage?
Bleeding between endosteal dura and skull following head injury
Rapid deterioration + focal neurological sings
Surgical drainage
Subarachnoid Haemorrhage?
Spontaneous bleed in the subarachnoid space following rupture of an aneurysm or congenital AV malformation
Clinical features of SAH?
Asymptomatic until rupture Sudden thunderclap headache 3rd nerve pasly Nausea LOC Neck stiffness + Kernig sign Small warning headaches before
Kernig sign?
When the hip is flexed there is limited/ painful knee extension
Brudzinski sing?
Passive neck flexion elicits hip + knee flexion
Management of SAH?
Nimodipine CCB
0.9% saline
Obliteration of aneurysm
Manage hypertension
How does Nimodpine aid the management of SAH?
Decreases cerebral artery spasms
Primary Infection - Varicella?
Replicates within skin or resp epithelium then picked up by immune cells and carried to lymph nodes
Primary viraemia VSV?
Attacks the reticuloendothelial system that is made of phagocytic cells
Secondary viraemia VSV?
2 weeks later when the virus starts attacking T cells starts
Tzank cells?
giant multinucleated cells made of fused keratinocytes
Secondary infection of VSV? Skin
T cells express proteins that bind to receptors on the skin
Release virus into skin and infect keratinocytes - spreading from cell to cell
Tzanck cells release INF a and INF B which inhibit viral protein synthesis and nearby cells being infected = Tiny lesions separated by normal areas of skin
Secondary infection of VSV? Neurones
Virus travels retrogradely back to the dorsal root ganglion or trigeminal ganglion
Adaptive immunity kills the virus all over the body apart from the ones hiding in the ganglion
Moment of weak immune system causes reactivation then virus travels back to skin
Complication of VSV?
Secondary bacterial infections (Hepatitis or pneumonia or encephalomeningitis)
Varicella symptoms?
2 weeks after virus enters body
Fever, headache, weakness
Skin lesion - Macules to Papules to Fluid filled vesilces to Scabs
Painful sores on mucosal surfaces
Herpes simplex symptoms?
Painful, itching or tingling localised to a specific dermatome on one side of the body
Rash lasts 4 weeks
Post-herpatic neuralgia >90 days
Diagnosis of VSV?
Rash
Tzanck test
Bloods - antibodies
PCR for viral DNA
Management of VSV?
Topical antipuritic Pain relief Anti-viral - if immunocompromised VZ Ig Varicella vaccine
Anti-virals that can be given in an immunocompromised patient with VSV?
Acyclovir
Famciclovir
Valacylovir
Why is aspirin CI in patients with VSV?
Reye syndrome risk
Liver is affected by both the virus and aspirin resulting in a toxic build-up of ammonia
What is the function of thiamine?
Thiamine Pyrophosphate is a metabolically active version of thiamine
Glucose metabolism
Metabolises lipids and carbohydrates
Maintain normal amino acid + neurotransmitter levels
Propagation of neural impulses
How does alcohol abuse result in decreased B1 function?
Blocks the phosphorylation of thiamine
Ethanol decreases B1 absorption in the duodenum
Liver Cirrhosis - less storage space for thiamine
Other: Malnutrition, Anorexia, GI cancer, IBD
WE?
Reversible and Acute
Ophthalmoplegia (weakness/ paralysis of eye muscles)
Ataxia
Change in mental state (Confusion, apathy or difficulty concentrating)
KE?
Irreversible and Chronic
Affects limbic system due to damage of mamillary bodies
Anterograde/ Retrograde Amnesia
Confabulation
What must be given after thiamine treatment to prevent metabolic acidosis?
Glucose
Examples of BPS of dementia?
Hallucinations, Delusion, Agitation, Depression, Anxiety, Withdrawal, Inappropriate sexual behaviour
Parkinsons?
Progressive movement disorder caused by the depletion of dopamine secreting cells of the SN
Cause of death in patients with Parkinsons?
Bronchopneumonia or Aspiration Pneumonia
Drugs that can cause drug-induced Parkinsons?
Antiemetics (phenthiazine)
Anaesthesia
Antipsychotics
Dopamine dysregulation syndrome?
Compulsive pattern of dopaminergic drug misuse due to prolonged use of dopaminergic medication
e.g. Gambling or Sexual behaviour
Early sign of Parkinson’s?
Mood changes
Ansomia
Acting out dreams during their sleep
Visual/ Auditory hallucinations
Chorea?
Continuous quick movements of hands and feet during voluntary movements but stop when sleeping
Sydenham’s Chorea?
Chorea in childhood following rheumatic fever
Antigens of strep bacteria similar proteins found on membranes of neurones of basal ganglia
Transient rapid irregular movements of limbs, trunk + face
Athetosis?
Slow, Writhing, Fixed movements of distal limbs due to degeneration of globus pallidus
Huntington’s disease?
AD Chromosome 4 mutation of huntingtin protein
Chorea of limbs, face, dysphasia then dementia
Hemiballismus?
Violent swinging of 1 side of the body due to infarction or haemorrhage in the contralateral subthalamic nucleus
Myoclonus?
Involuntary jerk of single muscle or group of muscle when falling asleep
Management of dystonia?
Anticholinergics - Botox, Procyclidine, Benzotropine
Cerebral palsy?
Motor disorder arising from damage to the brainstem during fetal life = Non-progressive loss of muscle control
Spastic cerebral palsy?
UMN lesion
Tight stiff movements
Hypertonia
Scissor gait or Toe walk gait
Spastic diplegia?
Atonic then spastic lower limbs
Starts <1 years old then usually resolves by 5
Types of cerebral palsy?
Spastic (UMN)
Ataxic (Cerebellum)
Dyskinetic (Basal ganglia)
Sciatica?
Pain, Tingling, Numbness which arises from an impingement of lumbosacral nerve root as they emerge from the spinal canal and are felt in a specific dermatome
Causes of lumbosacral impingement?
Herniated interverterbral disc
Spondylolisthesis
Spinal stenosis
Infection or met cancer
Signs of sciatica?
Unilateral leg pain radiating to the knee, foot or toes Lower back pain Dermatomal altered sensation \+ve straight leg test Babinski +ve
STarT Back?
Risk stratification tool used to identify modifiable risk factors for back pain disability
Common cause of myelopathy?
Disc osteophytes cord compression Congenital stenosis Trauma Disc Herniation Degenerative disease
Examples of lateralising signs?
Gaze paresis
Inattention
Agitated at one side
Myelopathy?
Injury/ Severe compression of the spinal cord
UMN signs
Loss of fine finger movements, legs don’t feel like their own, can’t feel the ground underneith, legs don’t d what I tell them
They leave the way they come in
Presenting neuro findings?
- Mr X, XX year old man, Who refereed them
- They described: (go through pain tool)
- Of note in background history (PMH, Dx, Allergies, Sx, Fx)
- Clinically, general appearance, vital signs (full details), positive findings on examination. Of note, Negative finding include absence of
- In summary, this is Mr X, XX year old man, with symptoms of XX and signs XX, suggestive of XX syndrome
Squint?
Misalignment of the visual axis. Eyes are not directed at the same object at the same time
Difference between a Manifest squint and Latent squint?
Manifest (Tropia) - When 1 eye us fixed on the object then the other eye is deviated
Latent (Phoria) - Squint occurs when the use of both eyes is interrupted - fusional control present
Rfx of squint?
Low birth weight
Maternal smoking
Hypermetropia (long sighted)
Family history
Penalization therapy?
Vision in the normal eye is deliberately blurred to force the child to use their amblyopic eye or use atropine drops
Horner’s syndrome?
Oculosympathetic paresis. Ipsilateral damage of the sympathetic trunk
Causes of Horner’s syndrome?
Pancoast tumour
Traumatic brain injury
Spinal cord lesions
Signs of horner’s syndrome?
Miosis Partial ptosis Anhidrosis Enophthalmus Flushing
Diagnosis of Horner’s syndrome?
Apraclonidine - cocaine eyedrops/ alpha agonists used to reduce occular pressure and dilate pupils
Paredrine - Causes more norepinephrine to be released to induce pupil dilation
Bell’s palsy?
Temporary inability to control facial muscles on one side of the face within 72 hours
Cause of Bell’s Palsy?
Pressure on the facial nerve as it travels within the auditory canal
Scoring system used to assess the severity of damage to the facial nerve?
House-Brackman Score
Meningitis?
Inflammation of the leptomeninges of the brain and spinal cord
Aetiology of meningitis?
Autoimmune
Intrathecal medications
Drug induced from using antibiotics
Infectious
Bacterial causes of meningitis?
Neisseria Meningitdis
Strep Pneumonia
Haemophilus Influenzae
TB
Signs of Meningitis?
Headache Neck stiffness Photophobia \+ve Kernig + Brudzinski sign Phonophobia Seizure Systemic features
Complications of meningitis?
Meningococcal septicaemia Cerebral infarction Hearing loss Seizures Cognitive impairment Communicating hydrocephalus
Prophylaxis of meningitis?
Rifampicin + Ciprofloxacin
Management of meningitis?
Benzylpenicillin, Cefortaxime or Chloramphenicol
Encephalitis?
Inflammation of the brain parenchyma which causes abnormal mental states + motor/sensory defects
Signs of encephalitis?
Flu-like symptoms Fever, Headache, drowsiness Hemiparesis Dysarthria Seizures + Coma
Investigations of encephalitis?
CT or MRI - cerebral oedema
CSF - increased lymphocytes
Viral serology of CSF or blood
Abscesses Summary?
Pussified swelling of the brain
Cerebral (Contrast-Enhanced CT) or Spinal Epidural (MRI)
Antibiotics + Surgical Decompression
Transmissible Spongiform Encephalopathy?
Prions - Proteins that trigger normal brain proteins to fold abnormally
As proteins build up it causes the brain shrink and form holes
Progressive dementia + Spongiform changes to the brain
Myasthenia gravis?
autoimmune disorder of the NMJ characterised by weakness + fatiguability of occular, bulbar and proximal limb weakness
Pathophysiology of myasthenia gravis?
Autoantibodies against acetylcholinesterase receptors of the post-synaptic membrane of the NMJ
70% associated with thymic tumour
What other autoimmune conditions are associated with myasthenia gravis?
RA
Pernicious anaemia
Autoimmune thyroid disease
Ddx of myasthenia gravis?
Graves disease
Cranial nerve lesions
MND
Lambert Eaton Myasthenic Syndrome
What is Lambert Eaton myasthenic syndrome?
Variation of myasthenia gravis but affects the Pre-synaptic membrane of the NMJ
Investigations used to confirm myasthenia gravis?
Nerve conduction studies - Repetitive stimulation of a nerve will show decreased muscle action potential
Bloods - autoantibodies
CT/ MRI - thymic tumour
Tensilon test
Management of myasthenia gravis?
Acetylcholinesterase inhibitor e.g. Pyridostigmine or Rivastigmine
Azathioprine
Thymectomy
2 types of autoantibodies seen in patients with myasthenia gravis?
Anti-acetylcholine receptor antibodies
Anti-musk (muscle specific tyrosine kinase) antibodies
Signs of myasthenia gravis?
Fatiguability of occular muscles
Bulbar symptoms
Eventual resp difficulties
Unable to hold arms straight
Myasthenic Crisis? SLUDGE
Salivation Lacrimation Urination Defecation GI distress Emesis
What is the Tensilon test?
Inject acetylcholinesterase inhibitor Edrophonium to increase concentration of AcH in the NMJ to temporarily improve symptoms
Management of myasthenic crisis?
Plasmapheresis
IV immunoglobulins
Muscular dystrophy?
X-lined recessive group of diseases that causes progressive weakness and wasting of muscles
Defect in dystrophin protein
Thigh - Pelvis - Arms
Myotonia?
Delayed muscle relaxation after contraction
Dystrophia myotonica?
AD progressive distal weakness with myotonia, ptosis, facial muscle weakness and wasting
Signs of dystrophia myotonica?
Facial muscle weakness Ptosis Baldness Cataracts Hypogonadism Cardiomyopathy Mild mental handicap Glucose intolerance
Management of Dystrophia myotonica?
Phenytoin or Procainamide
Multiple sclerosis?
Autoimmune demyelinating disease of the CNS
Pathophysiology of MS?
Mylin - oligoendrocytes + BBB
T-cells with special ligands attach to the BBB and enter then attach to myelin then release cytokines which cause blood vessel dilation and allow macrophages and B cells to enter
Macrophage engulfs Oligodendrocytes and B-cells make antibodies against myelin
Inflammation = Demyelination = Exposed axon = Plaque formation (scar tissue)
Regulatory cells come late to the party to tell everyone to chill out
Types of MS?
Relapsing Remittant
Secondary Progressive
Primary Progressive
Progressive Relapsing
Aetiology of MS?
Genetics - Female or HLA DR2 EBV infection (antigens similar to myelin)
What may a patient with suspected MS present with a history of?
Optic neuritis
Transverse Myelitis
Cerebellar related symptoms
Brainstem syndromes
Signs of MS?
History of flare up of neurological symptoms e.g.:
- Diplopia
- Vision loss
- Ascending sensory disturbance/ Weakness
- Coordination problems
- Lhermitte’s sign
- Uhthoff Phenomena
20 to 50 years old
Investigations of MS?
Full bloods - rule out any other causes McDonald Criteria MRI - White plaques CSF - antibodies Nerve conduction studies MOG +ve
MOG+ve?
Myelin Oligodendrocyte glycoprotein (adhesion molcule) found in myelin sheath of nerve cells
Antibodies can be made against it
Acute Management of MS?
Acute Relapse - Methylprednisolone or Cyclophosphamide
MS fatigue?
Amantadine
MS spasticity?
Baclofen
MS Oscillopsia?
Sensation of environment moving even when stationary
Gabapentin or Memantine
MS disease progression management?
SC Beta interferon - Decrease inflammatory cytokines and increase T regulatory
Glatiramer Acetate - Take me instead T-cell
Fingolimod - Locks lymphocytes in lymph node purgatory
Second-line MS disease progression management?
Natalizumab + Mitoxantrone
Risk of multifocal leukoencephalopathy + cardiac toxicity
Seizure?
Prolonged deplorisation of groups of adjacent neurones + decreased inhibitory neurotransmitters
Epilepsy - tendency of recurrent seizures
Difference between simple partial and complex partial?
Simple partial doesn’t affect consciousness or memory
Types of generalised seizures?
Grand Mal (GTC) - tense, tongue bite, incontinence
Petite Mal (Absence)
Myoclonic Akinetic
Tonic - isolated movements
Ddx of epilepsy?
Vasovagal syncope Psychogenic non-epileptic seizure TIA Panic attacks with hyperventilation Migraine
Management of acute epilepsy?
Protect from injury
+ Check airway + Recovery position
Buccal Midazolam
Rectal Diazepam
IV Lorazepam
Long term 1st line Management of Partial seizures?
Lamotrigene or Carbamazepine
Long term 2nd line management of Partial seizures?
Levetiracetam
Long term 1st line management of grand mal seizures?
Sodium Valproate
Lamotrigene
Why may carbamazepine not be used in newly diagnosed GTC seizures?
May exacerbate myoclonic and absence seizures?
Long term 1st line management of absence seizures?
Ethosuximide or Sodium Valproate
Narcolepsy?
Inability to regulate sleep-wake cycles
Pathophysiology of Narcolepsy?
Hypothalamic Neurones release Orexin/ Hypocretin A + B to regulate sleep-wake cycles
Autoimmune damage of Orexin during adolescence
Clinical features of Narcolepsy?
Daytime sleepiness (despite sleeping normal night hours, symptoms improve with naps, vivid dreams, sleep cycle lasts mins before REM)
Cataplexy (Episodes of muscle weakness triggered by emotions, face or whole-body)
Hallucinations (Hypnogogic or Hypnopompic)
Sleep Paralysis
Fragmented Sleep
Diagnosis of Narcolepsy?
Polysomnography (EEG, ECG, EOG, EMS)
Multiple sleep latency test
Management of Narcolepsy?
Good sleep hygiene
Stimulant - Methylphenidate, Dexamphetamine
Sodium Oxybate - Helpful for cataplexy 2 to 3 hours before a meal
Antidepressant
Neurofibromatosis?
AD mutation of the NF1 gene on chromosome 17 or NF2 gene on chromosome 22
Benign nerve sheath tumour of the PNS
NF1?
Small cutaneous or large plexiform neurofibromas on the skin
Freckles in the axilla or inguinal region
Lisch nodules of the eye
Optic pathway ganglion
Cafe au Lait spots
Other: disfigurements, LD, hypertension, Epilepsy, Probems with bones e.g. hypermobility, fracture, curved spine, changes to shape of chest wall
Maccune Albright Syndrome?
Cafe Au Lait Spots + Endocrine Dysfunction
NF2?
Bilateral Acoustic Neuroma - Schwannoma
Bilateral Cataracts
Meningioma - Benign (Brain + spinal cord) or psammomatous meaning heavily calcified lesions (spine)
Ependymoma - Ependymal cells from the lining of the ventricles (4th usually) - present with large lateral and 3rd ventricles + Raised ICP
GCS eyes?
Spontaneous
Speech
Pain
Nothing
GCS Verbal?
Orientated Confused Inappropriate words Incomprehensible Nothing
GCS motor?
Obeys command Moved to localised pain Flexion withdrawal Decorticate Decerebrate (abnormal extension) No Response
Radiculopathy?
Sharp shooting radiating limb pain in the pattern of a dermatome
e.g. Spinal Claudication - Bilateral radiating leg pain or paraesthesia that comes with walking and made better by rest or leaning forward
Vertebral pain syndrome?
Wear and Tear degenerative disease
Localised aching pain with limited radiation, stiff muscles and worse with activity
No neurological signs
Visible or palpable paravertebral muscle spasms, tenderness or restricted movements
Anterior cord syndrome?
Syndrome that affects the anterior 2/3 of the spinal cord resulting in motor paralysis below the level of the lesion (corticospinal tracts)
loss of pain + temp (Bilateral spinothalamic)
Back pain + autonomic dysfunction
Causes of anterior cord syndrome?
Ischaemia of the anterior spinal artery (anterior and lateral horns)
Iatrogenic - Thoracic or Thoracoabdominal aortic aneurysm
Hypotension
Increased Spinal canal pressure
Signs of anterior cord syndrome?
Bilateral weakness and loss of pain + temp below the level of the lesion
+/- autonomic dysfunction
+/- back pain
Investigation of anterior cord syndrome?
MRI - T2 hyperintensities on sagittal view showing vertical pencil-like lesion
LP, Bloods, CSF testing
Management of anterior cord syndrome?
IV fluids + Lumber drain to maintain pressure within the spinal cord (post aortic surgery)
Ddx of anterior cord syndrome?
Cauda equina syndrome Transverse myelitis Disc Herniation MS Brown Sequard
Brown Sequard?
Contralateral Pain + Temp
Ipsilateral Weakness
