Neurology Flashcards
What are primary headaches and some examples of them?
Headaches with no underlying cause
- Migraine
- Cluster
- Tension
- (Trigeminal neuralgia)
What are red flag features for headaches?
- Sudden onset for longer than 5 minuets
- New onset over 50
- Progressive or persistent
What are red flag precipitating factors for headaches?
- Recent head trauma
- Headache worse lying down (raised ICP)
- Headache worse on standing (CSF leak)
- Household contacts with similar symptoms (CO poisoning)
What are red flag associated symptoms of headaches?
- Fever, photophobia or neck stiffness
- New neurological defect (stroke/raised ICP)
- Visual disturbance
- Vomiting (raised ICP, brain abscess and CO poisoning)
What is an important investigation to carry out for a headache?
fundoscopy which will look for papilledema which indicates raised ICP
What are some risk factors for migraines?
- FH
- Female
- Obesity
What are some triggers of migraines
CH- Chocolate
OC- Oral contraceptive
OL- alcohOL
A- anxiety
T- travel
E- exercise
CHOCOLATE
Other triggers can be red wine, bright lights and menstruation
What are some different types of migraine/
- Migraine without aura
- Migraine with aura
- Silent migraine (just the aura without the headache
- Hemiplegic migraine
What are the headache symptoms of a migraine?
Lasts between 4-72 hours
- Pounding or throbbing in nature
- Usually unilateral
- Photophobia
- Phonophobia
- Aura
- Nausea and vomiting
What is aura/
Aura is the term used to describe the visual changes associated with migraines symptoms can be:
- Sparks in vision
- Blurring vision
- Line across vision
- Loss of different visual fields
What is a hemiplegic migraine?
They can mimic a stroke need to rule out if patient has symptoms:
- Typical migraine
- Sudden onset
- Hemiplegia
- Ataxia
- Change in consciousness
What are the 5 stages of a migraine/
- Prodromal
- Aura
- Headache
- Resolution
- Prodromal
What is the diagnostic criteria for migraines with aura?
At least two headaches filling criteria
What is the diagnostic criteria for migraines without aura?
At least five headaches filling criteria
What is the management for migraines?
- Analgesia
- Oral triptan (500mg sumatriptan) as the headache starts (can also use aspirin)
- Antiemetics metoclopramide
What are triptans?
They are serotonin agonists and they cause
- Smooth muscle contraction in arteries
- Peripheral pain receptors to inhibit activation of pain
- Reduce neuronal activity in the central nervous system
What medications are used for migraine prophylaxis?
- Propranolol
- Topiramate (is teratogenic)
- Amitriptyline
What should not be given to a female who experiences migraines with aura?
The combined pill it increases the risk of a stroke
What are the non-pharmacological treatments for migraines?
- Acupuncture: if both propranolol and topiramate are ineffective or unsuitable
- Riboflavin (vitamin B2): **may be effective in some people, but avoid in pregnancy
What is amaurosis faugax?
A classical syndrome of painless short-lived monocular blindness. Is mainly caused by transient obstruction e.g. an emboli but can be caused by GCA
- Often described as a black curtain coming across the vision.
What is a tension headache?
Most common primary headache
Can be episodic (<15 days/month) or chronic (>15 days a month for at least 3 months)
What are the causes of tension headaches?
- Missed meals
- Stress
- Overexertion
- Lack of sleep
- Depression
What are the symptoms of a tension headache/
- Bilateral with a pressing/tight sensation of mild-moderate intensity
- Nausea or vomiting
- Photophobia
- Phonophobia
What is the main risk factor for a tension headache?
STRESS
What are cluster headaches?
Severe unilateral headaches often periorbital that come in clusters of attack
What is the typical presentation for cluster headaches?
A 0-50 year old male smoker. Attacks can be triggered by alcohol, strong smells and exercise
How long can cluster headaches last?
3-4 attacks a fay for weeks or months followed by a pain free period
Attacks can last for 15 minuets to 3 hours
What are the symptoms of a cluster headache?
- Severe intolerable pain
- Unilateral
- Red swollen watering eye
- Pupil constriction
- Eyelid dropping
- Nasal discharge
- Facial swelling
What is the acute management of cluster headaches?
- Triptans
- High flow oxygen
What are the prophylaxis for cluster headaches?
- Verapamil
- Lithium
- Prednisolone
What are the risk factors for trigeminal neuralgia?
- Female
- 50-60
- Increases with age
- Unilateral
- MS
What are the causes of trigeminal neuralgia?
Normally due to compression of the trigeminal nerve by a vascular loop often superior cerebellar artery
What are triggers for TGN?
- Light touch
- Washing
- Shaving
- Talking
- Cold weather
What is the headache like in trigeminal neuralgia?
Electric Shock Pain that lasts for seconds to minutes across the face
90% unilateral
10% bilateral
What is the diagnostic criteria for trigeminal neurlagia?
Clinical Dx
3 or more attacks with characteristic unilateral facial pain and symptoms
MRI- exclude secondary causes/other pathology
What is the treatment for TGM?
- First line: carbamazepine
- Second line: microvascular decompression
What is an acoustic neuroma?
A benign tumour of the Schwann cells surrounding the auditory nerve that innervates the inner ear
Are acoustic neuromas usually unilateral or bilateral?
Unilateral
Bilateral acoustic neuromas are almost always due to neurofibromatosis type II
What is the presentation of a acoustic neuroma?
40-60 year old patient with gradual onset of:
- Unilateral sensorineural hearing loss
- Unilateral tinnitus
- Dizziness
- A sensation of fullness in the ear
Can also be associated with a facial nerve palsy
What is the management of an acoustic neuroma?
Conservative management with monitoring may be used if there are no symptoms or treatment is inappropriate
Surgery to remove the tumour (partial or total removal)
Radiotherapy to reduce the growth
What is used to asses the hearing loss in an acoustic neuroma?
Audiometry
Heating will be more affected with loud sounds
What does the spinothalamic tract convey?
- Anterior tract is responsible for crude touch/or pressure
- Lateral tract is responsible for pain and temperature
What is the pathway of the spinothalamic tract, and what will a lesion present as?
- These pathways decussate within the spinal cord and travel up to the brain
So the lesion of the spinal cord will cause a of pain, crude touch, sensation
As nerve entering the Anterior lateral pathways tend to travel up 1 or 2 vertebral levels before Decussating
Spinothalamic and Dorsal Column medial lemniscal pathway synapses in the ventral posterio-lateral division (VPL) of the nucleus of thalamus.
What does the DCML tract convey?
- Fine touch and tactile sensation, vibration and proprioception
- Signals from lower limbs travel up ** fasciculus gracillis**
Signals from the upper limb (above T6) tend to travel in the Fasciculus Cuneatus
graciLis = L for Leg of Lower Limb
Outline the pathway of the DCML tract
What will a lesion in the DCML tract present as?
- Afferent signals are carried along first order neurons to and up the dorsal columns then synapse in the medulla
- 2nd order neurones decussate at the medulla and travel to the thalamus where they synapse again
- 3rd order neurones the order neurones then travel through the internal capsule to the somatosensory cortex in the Parietal mode (postcentral gyrus)
THESE TRACTS RUN IPSILATERALLY THROUGH THE SPINAL CORD AND DECUSSATE IN THE MEDULLA OF THE BRAINSTEM = so damage to spinal cord here would lead to loss of Ipsilateral fine touch, tactile sensation, proprioception etc.
What does the cortical spinal tract convey?
Carry brain signals for voluntary movemnts
Outline the pathway of the corticospinal tract?
After originating from the cortex, the neurones converge, and descend through the internal capsule
- this is particularly susceptible to compression from haemorrhagic bleeds
The fibres within the lateral corticospinal tract decussate
The anterior corticospinal tract remains ipsilateral, descending into the spinal cord.
From the ventral horn, the lower motor neurones go on to supply the muscles of the body.
What things can damage the spinal tracts/causes sign?
Corticospinal damage - UPPER MOTOR NEURONE SIGN, AS SPINAL NERVES ARE UMN
Trauma
MS
Tumour/growth
Vascular supply of the spinal cord/spinal cord stroke?? - ANTERIOR SPINAL ARTERY STROKE INFARCTS CAN AFFECT TRACTS, —> can cause back pain as spine has pain receptors
Acute spinal chord and strokes can cause lower MN signs (spinal shock)
Syringomyelia - affects CRTS and STHAM - think this if see cape like distribution of weakness
What is Bell’s palsy?
It presents as a unilateral lower motor neurone facial nerve palsy.
The majority of patients fully recover over several week but recovery may take up to 12 moths
What is the treatment of Bell’s palsy?
prednisolone as treatment, either:
50mg for 10 days
60mg for 5 days followed by a 5-day reducing regime of 10mg a day
Patients also require lubricating eye drops
What is a brain abscess?
A pus-filled pocket of infected material in the brain
What are some bacterial causes of a brain abscess?
Streptococcus pyogenes/Strep milleri
Staph Aureus
Klebsiella pneumoniae,
Neisseria meningitides
What are some fungal causes of brain absces?
Aspergillus fumigates, Candida albicans
What are some parasitic causes of brain abscess?
Toxoplasma gondii,
What are the symptoms of a brain abscess?
Fever
Headache
Changes to mental state
Focal neurological deficits
Gradual mal seizures
Nausea
Vomiting
Neck stifness
What are the symptoms associated with a ruptured brain abscess/
suddenly worsening headache, followed by emerging signs of meningism
What are some investigations for a brain abscess?
FBC
ESR and CRP raised
Blood culture
MRI
CT scan: appears as a radiolucent space-occupying lesion
What is the management of a brain abscess?
Vancomycin+ metronidazole+ Ceftriaxone
drain intracranial collection
administer effective antibiotic therapy (early treatment is essential)
Steroids
What are the most common cancers that spread to the brain?
Lung cancer, 48%
Breast cancer, 15%
Genitourinary tract cancers, 11%
Osteosarcoma, 10%
Melanoma, 9%
What is bulbar palsy/
Refers to signs and symptoms linked to the impairment of the glossopharyngeal nerve (CN IX), the vagus nerve (CN X), the accessory nerve (CN XI), and the hypoglossal nerve (CN XII).
What conditions cause a bulbar palsy?
MND
GBS
Polio
Brainstem tumours
MG
Myotonic dystrophy
Toxic: Botulism
What are some signs and symptoms of bulbar palsy?
- Dysphagia
- Chewing difficulty
- Dysphonia
- Nasal speech
- Flaccid paralysis
The ocular muscles are spared and this differentiates it from myasthenia gravis.
What are the investigations for bulbar palsy?
electromagnetic articulography (EMA)
electropalatography (EPG)
pressure sensing EPG
routine bloods, brain/brainstem imaging, electromyography
What is the difference between bulbar and pseudobulbar palsy?
PSEUDO – affects the upper motor neurones
BULBAR – affects the lower motor neurones (of CN 9,10,11,12)
In contrast, pseudobulbar palsy is a clinical syndrome similar to bulbar palsy but in which the damage is located in upper motor neurons of the corticobulbar tracts in the mid-pons (i.e., in the cranial nerves IX-XII), that is the nerve cells coming down from the cerebral cortex innervating the motor nuclei in the medulla. This is usually caused by stroke.
What are the symptoms of cerebellar dysfunction?
D Dysdiadochokinesia
A Ataxia (gait and posture)
N Nystagmus
I Intention tremor
S Slurred, staccato speech
H Hypotonia/heel-shin test
What is ataxia?
A neurological sign consisting of lack of voluntary coordination of muscle movements that can include gait abnormalities, speech changes and abnormalities in eye movements
What can cause ataxia?
- Cerebellar problems
- Issues with proprioception
What test helps you distinguish between proprioceptive and cerebellar causes of ataxic gait?
If they can walk normally with their eyes open but bad with their eyes closed then it is a proprioception problem
If the problem is there all the time then it is a cerebellar cause
What are some general features of ataxia?
Wide based gait
Falls
Cannot walk hell-to-toe
Often worse in the dark or with eyes closed
Gait abnormalities
nystagmus/
intention tremors
Heart problems
Decreased tone/reflexes
What are some cerebellar causes of ataxia?
MS
Posterior fossa tumour
Alcohol
Direct injury
Gluten ataxia autoimmunity to purkinje cells or other neural cells
CNS vasculitis, multiple sclerosis, infection, bleeding, infarction, tumors, direct injury, toxins (e.g., alcohol), genetic disorders and neurodegenerative diseases
What side is affected in a cerebellar lesion?
Deficit is Ipsilateral If they are ataxic on the right, the problem is with the right side of the cerebellum) - UNLIKE IN CEREBAL LESIONS
What are some proprioceptive causes of an ataxic gait?
Sensory neuropathies- Low B12
Inner ear problems- affecting the vestibular system
What is Fredrich’s ataxia?
Genetic, progressive, neurogenerative movement disorder which typically presents at age 10-15 years
- Autosomal recessive on chromosome 9
What happens in Fredrich’s ataxia?
It can lead to peripheral neuropathy, due to the atrophy of the dorsal root ganglia and thinning of the dorsal roots
What blood tests are used in ataxia diagnosis?
- FBC, U&E, extended LFT’s
- HbA1c, B12, folate, TSH
- ESR, CRP
- gluten related serology* (can only be requested in Sheffield)
What is cerebral palsy?
- Permanent neurological problems resulting from damage to the brain around the time of birth
- It is not a progressive conditions however problems may change over time during growth and development
What are some causes of CP?
Antenatal:
Maternal infections
Trauma during pregnancy
Perinatal:
Birth asphyxia
Pre-term birth
Postnatal:
Meningitis
Severe neonatal jaundice
Head injury
What is spastic hypertonia CP?
Increased tone and reduced function resulting from damage to upper motor neurones
What is Dyskinetic CP?
- Problems controlling muscle tone with hypertonia and hypotonia
- It causes athetoid movements Twisting, turning, squirming, twitching, jerking, repeated motions, tremors, poor posture, unsteadiness, and grimacing
- It also causes oro-motor problems
Due to damage of the BASAL GANGLIA
What are some other types of CP?
Ataxic: problems with coordinated movement resulting from damage to the cerebellum
Mixed: a mix of spastic, Dyskinetic and/or ataxic features
What is the presentation of CP?
- Failure to meet milestones
- Increased or decreased tone
- Hand preference below 18 months
- Problems with coordination
- Feeding or swallowing problems
- Learning difficulties
What can be affected in cerebral palsy?
Monoplegia: one limb affected
Hemiplegia: one side of the body affected
Diplegia: four limbs are affects, but mostly the legs
Quadriplegia: four limbs are affected more severely, often with seizures, speech disturbance and other impairments
What gaits will indicate CP?
- Hemiplegic gait: it is caused by increased muscle tone and spasticity in the legs
- The leg will be extended with plantar flexion of the feet and toes
- This means they have to swing the leg around in a large semicircle when moving their leg from behind them to in front. There is not enough space to swing the extended leg in a straight line below them.
What are some complications associated with CP?
Learning disability
Epilepsy
Kyphoscoliosis
Muscle contractures
Hearing and visual impairment
Gastro-oesophageal reflux
What is the management of CP?
Physiotherapy is used to stretch and strengthen muscles, maximise function and prevent muscle contractures.
Occupational therapy is used to help patients manage their everyday activities, such as getting dressed and using the bathroom. That can involve techniques to perform tasks despite disability. They can also make adaptations and supply equipment, such as rails for assistance or fitting a hoist for a patient who is entirely wheelchair bound.
Speech and language therapy can help with speech and swallowing. When swallowing difficultly prevents them meeting their nutritional requirements they may require an NG tube or PEG tube to be fitted.
Dieticians can help ensure they meet nutritional requirements. Some children may require PEG feeding through a port on their abdomen that gives direct access to the stomach.
Orthopaedic surgeons can perform procedures to release contractures or lengthen tendons (tenotomy).
What medications can be used to manage the side effects of CP?
Muscle relaxants (e.g. baclofen) for muscle spasticity and contractures and Botox
Anti-epileptic drugs for seizures
Glycopyrronium bromide for excessive drooling
What is chronic fatigue syndrome?
A complex, chronic medical condition affecting multiple body systems and of unknown cause which has many different presentations
Known as myalgic encephalomyelitis
What can cause ME?
- 50-80% of people start suddenly with a flu-like illness
- Often found after infection
- Strong genetic disposition
- Life stressors
What are some key presentations of ME?
Onset is usually abrupt, often following a stressful event
viral-like illness with swollen lymph nodes, extreme fatigue, fever, and upper respiratory symptoms
Debilitating fatigue
Post-exertional malaise
Unrefreshing sleep or sleep disturbance
‘Brain fog’
Generalized pain
Significantly reduced ability to engage in occupational, educational, social or personal activities
What are some investigations for ME?
The diagnosis of CFS is made by the characteristic history combined with a normal physical examination and normal laboratory test results
normal FBC, U+Es, Creatine, ESR, TFTs
What is the management for ME?
Lifestyle advice - pace yourself, rest as needed, maintain health diet, work adjustments
CBT
No medicine treatment
What is diabetic neuropathy? What are some symptoms?
peripheral nerve damage as a result of diabetes mellitus
Commonly results in weakness; sensory symptoms such as numbness, tingling, or pain; or autonomic changes such as urinary symptoms.
These changes are thought to result from a microvascular injury involving small blood vessels that supply nerves (vasa nervorum).
What is benign essential tremor?
- A fine tremor affecting all the voluntary muscles it is most common in the hands
- Can also affect the head, jaw and vocal cords
What are the features of a benign essential tremor?
- Fine tremor
- Symmetrical
- More prominent with voluntary movement
- Worse when tired, stressed or after caffeine
- Improved by alcohol
- Absent during sleep
What are some differentials of a BET?
Parkinson’s disease
Multiple sclerosis
Huntington’s chorea
Hyperthyroidism
Fever
Dopamine antagonists
What is the management of a BET?
Propranolol (a non-selective beta blocker)
Primidone (a barbiturate anti-epileptic medication)
What is Parkinson’s disease?
A neurodegenerative disorder characterised by the loss of dopaminergic neurons within the substantia nigra pars compacta of the basal ganglia
What are the risk factors for developing PD?
Age
Gender: more common in men
Family history
Describe the pathophysiology of PD?
- There is a progressive loss of dopamine-producing neurons meaning there is a reduction in the amount produced
- This leads to a reduction in action of the direct pathway and an increase in the antagonistic indirect pathway which restricts movements
- This leads to the symptoms of bradykinesia and rigidity
- There is also formation of protein clumps Lewy bodies
What are the 3 key symptoms of PD?
- Bradykinesia
- Tremor
- Rigidity
Symptoms usually start unilateral and then become bilateral later in the disease course
What symptoms are not present in the early stages of PD?
- Incontinence
- Dementia
- Falls
- Symmetry
Can be a sign of normal pressure hydrocephalus
What are the effects of bradykinesia in PD?
- Small handwriting
- Shuffling gait
- Difficulty initiating movement
- Difficulty turning around when standing
- Reduced facial movements and expressions
What is the presentation of the tremor in PD?
- Unilateral resting tremor
- Described as a pill rolling tremor
- Worse when resting and distracted using other hand
Frequency of 4-6 times a second
What is the rigidity like in PD?
- If you take their hand and passively flex and extend their arm at the elbow you will feel tension in their arm that gives way to movement in small increments (little jerks)
- Described as cogwheel
What are some other symptoms of PD?
- Depression
- Sleep disturbance
- Loss of smell
- Postural instability
- Cognitive impairment
What are some differences between BET and PD?
- PD= asymmetrical BET= symmetrical
- PD= frequency= 4-6 BET= 5-8
- PD= worse at rest BET= better at rest
- PD= improves with intentional movement BET= worse
- PD= no change with alcohol/worse BET= better with alcohol
What is used to diagnose PD?
a clinical diagnosis that showed be suspected in a patient with bradykinesia and at leas tone of:
- Tremor
- Rigidity
- Postural instability
What is the treatment for PD?
Motor symptoms not affecting quality of life:
A choice of one of the following:
Dopamine agonist(non-ergot derived)
- Pramipexole, ropinirole
Monoamine oxidase B inhibitor (MOA-B)
- Selegiline, rasagiline
- Stop breakdown of circulating dopamine
Motor symptoms affecting the quality of life:
- Synthetic dopamine levodopa given with a drug that stops it being broken down
- Peripheral decarboxylase inhibitors
Co-benyldopa (levodopa and benserazide)
Co-careldopa (levodopa and carbidopa)
What is Huntington’s disease?
An Autosomal dominant condition that is neurodegenerative and causes a deterioration in the nervous system
What causes HD?
- It is a trinucleotide repeat disorder
- There is a repeat of CAG which codes for glutamine 36 times in a row so patients have 6 glutamine in a row on the Huntington protein
- These mutated proteins aggregate within neuronal cells of the caudate. This leads to decreased ACh and GABA synthesis
- This leads to an increase in dopamine production
What is anticipation and how is it linked to HD?
- A feature of trinucleotide repeat disorders. When coping the HTT gene DNA polymerase can lose track of which CAG it’s on and add an extra CAG.
- This leads to successive generations having more repeats in the gene resulting in:
- Early age of onset
- Increased severity of the disease
What are the symptoms of HD?
Patients asymptomatic until 30-50
- Begin with cognitive, psychiatric or mood problems
- Chorea
- Eye movement disorders
- Dysarthria: speech difficulties
- Dysphagia: swallowing difficulties
- Dementia
What medications are given for symptoms relief in HD?
- Life expectancy is 15-20 years after the onset of symptoms
- Antipsychotics (e.g. olanzapine)
- Benzodiazepines (e.g. diazepam)
- Dopamine-depleting agents (e.g. tetrabenazine)
What is GCA?
A vasculitis affecting the medium and large arteries it is the most common cause of systemic vasculitis
It usually affects branches of the carotid artery
What are the risk factors for GCA?
- Age usually over 50
- Female
- Caucasians
- Polymyalgia rheumatica associated in 50% of cases
What causes GCA and which arteries are affected?
It is a granulomatous vasculitis. Arteries become inflamed and thickened and there is a narrowed lumen which prevents blood flow
Cerebral arteries are affected:
Superficial temporal: headache and scalp tenderness
Mandibular: Jaw claudication
Ophthalmic artery: visual loss due to retinal ischaemia
What are the signs of GCA?
- Superficial temporal artery tenderness
- Absent temporal pulse
- Reduced visual activity
What are the symptoms of GCA?
- Main symptom is headache that us unilateral and around forehead and temple.
- Irreversible painless complete sight loss
- Jaw claudication
- Scalp tenderness may be noticed when brushing hair
What are some systemic symptoms of GCA?
- Fever
- Muscle aches
- Fatigue
- Weight loss
- Loss of appetite
- Peripheral oedema
What are the investigations for GCA?
- ESR a value of greater than 50
- A temporal artery biopsy is gold standard and will show multinucleated giant cells negative results don’t rule out as there can be skip lesions
What is the treatment of GCA?
- Corticosteroids 40-60mg IV methylprednisolone
- Oral aspirin to prevent ischaemic cranial complications
What are the complications of GCA?
- Aortic aneurysms
- Glucocorticoid toxicity
- Vision loss
- Cerebrovascular accident
What is the ongoing management for GCA?
Once the diagnosis is confirmed they will need to continue high dose steroids (40-60mg) until the symptoms have resolved. They then need to slowly wean off the steroids. This can take several years. This is a similar process to managing polymyalgia rheumatica.
What is Guillain-Barré syndrome?
- It is a autoimmune, rapidly progressing demyelinating condition of the peripheral nervous system
What are the risk factors for developing Guillain-Barré syndrome?
- Male
- Age
- Malignancies
- Vaccines
- Infecitons
What are the most common infections that can trigger GBS?
- Campylobacter jejuni (most common)
- Cytomegalovirus
- EBV
What causes GBS?
- A pathogenic antigen resembles myelin gangliosides in the peripheral nervous system
- The immune system targets the antigen and attacks the myelin sheath of sensory and motor neurones
- it occurs in patches along the length of the axon so is called segmental demyelination
What antibodies can be found in GBS?
- Anti-ganglioside antibodies (anti-GMI)
What are the signs and symptoms of GBS?
Symptoms usually appear within 4 weeks of a preceding infection
- Symmetrical ascending weakness
- Reduced reflexes
- Loss of sensation and pain
- Cranial nerve involvement such as facial nerve weakness
- Autonomic features (sweating, raised pulse)
- Struggling to breathe
What is used to diagnose GBS?
A clinical diagnosis that is evidenced by progressive weakness and hyporeflexia in the weaker limbs.
The Brighton criteria is used for diagnosis.
What are the differentials of GBS?
- Myasthenia gravis
- Transverse myelitis
- Polymyositis
What is the treatment for Guillain-Barré syndrome?
- IV immunoglobulins IV IG
- Plasma exchange (alternative to IV IG)
- Venous thromboembolism prophylaxis (PE is the leading cause of death)
What is the prognosis for Guillain-Barré syndrome?
80% will fully recover
15% will be left with some neurological disability
5% will die
What is shingles?
also known as herpes zoster virus, shingles is a disease which triggers a painful skin rash in a dermatomal distribution
What causes shingles?
Human herpes virus-3
A primary infection usually occurs in childhood, producing chickenpox
After that the virus lies dormant in the sensory nervous system and is prone to reactivation in patients over 50 years old and is associate with immunosuppressive illness, or psychological/physical trauma
What is malaria?
An infectious disease caused by members of the plasmodium family of protozoan parasites
What insects transmit malaria?
- The bite from the female anopheles mosquitoes
What is the most common and severe form of malaria?
Plasmodium falciparum
What are some other forms of malaria?
Plasmodium vivax
Plasmodium ovale
Plasmodium malariae
Plasmodium knowlesi
Which forms of malaria can lie dormant and for how long?
P. vivax and P. ovale
For up to four years
What is the life cycle of malaria?
The parasite lies dormant in the mosquitos gut producing sporozoites
When someone is bitten by mosquito these are injected into blood sporozoites travel to the liver of the newly infected person
They mature in the liver into merozoites which infect RBCs causing them to rupture releasing more into the blood stream causing haemolytic anaemia
For P. vivax and P. ovale, this rupture and release of merozoites occurs every 48 hours, causing a fever spike every other day. A fever every 48 hours is referred to as tertian malaria. P. falciparum has more frequent (“subtertian“) or irregular fever spikes, and P. malariae has spikes every 72 hours (“quartan“).
What is the presentation of malaria?
Malaria should be suspected in someone that has travelled to an area where malaria is present. The incubation period is usually 1-4 weeks after exposure, although it can lie dormant for years.
Many of the symptoms are non-specific:
- Fever sweats and rigors
- Fatigue
- Myalgia
- Headache
- Nasuea
- Vomiting
What are some signs of malaria?
- Pallor due to anaemia
- Hepatosplenomegaly
- Jaundice
How can malaria be diagnosed?
- Made using a malaria blood film
Three negative samples taken over three consecutive days are required to exclude malaria due to the parasites being released from red blood cells into the blood every 48-72 hours
What is the management of malaria?
Artemether with lumefantrine (Riamet) is the usual first choice
Quinine plus doxycycline
Quinine plus clindamycin
What is the treatment of severe malaria?
Artesunate is the usual first choice (haemolysis is a common side effect)
Quinine dihydrochloride
What are some complications of malaria?
Cerebral malaria
Seizures
Reduced consciousness
Acute kidney injury
Pulmonary oedema
Disseminated intravascular coagulopathy (DIC)
Severe haemolytic anaemia
Multi-organ failure and death
What are some antimalarials?
Proguanil with atovaquone (Malarone)
Doxycycline
Mefloquine (risk of psychiatric side effects)
Chloroquine with proguanil (less often used due to high resistance)
What is Meniere’s disease?
A long term inner ear disorder that causes recurrent attacks of vertigo
What is the pathophysiology of Meniere’s disease?
e excessive build up of endolymph in the labyrinth of the inner ear, causing a higher pressure than normal and disrupting the sensory signals.
This increased pressure of the endolymph is called endolymphatic hydrops.
What is the triad of symptoms typically seen in Meniere’s disease/
- Hearing loss
- Vertigo
- Tinnitus
It is sensorineural hearing loss, generally unilateral and affects low frequencies first.
What are some other symptoms of Meniere’s disease?
A sensation of fullness in the ear
Unexplained falls (“drop attacks”) without loss of consciousness
Imbalance, which can persist after episodes of vertigo resolve
Usually presents in 40-50 year olds
There may also be spontaneous nystagmus during an attack
What is the management of Meniere’s disease?
Managing symptoms during an acute attack
Prophylactic medication to reduce the frequency of attacks
What is used during an acute attack of Meniere’s disease?
Prochlorperazine
Antihistamines
What is used for prophylaxis in Meniere’s disease?
Betahistine
What is motor neurone disease (MND)?
A progressive neurodegenerative disease where both upper and lower motor neurones stop functioning but there is no effect on sensory neurons
What are the different types of MND?
Amyotrophic lateral sclerosis- most common 50% of cases
Primary lateral sclerosis (PLS) UMN
Progressive muscular atrophy LMN
Progressive bulbar palsy
What are the signs of progressive muscular atrophy?
It is an anterior horn cell lesion so LMN signs only affects distal muscle groups before proximal
In contrast ALS or PLS there is an absence of:
- Brisk reflexes
- Spasticity
- Babinski’s sign
What are the signs of Progressive bulbar palsy? What is affected?
UMN + LMN + Cranial Nerve IX, X, XI, XII signs
Lower cranial nerve nuclei affected causing dysarthria, dysphagia, nasal regurgitation of fluids, choking
What is the pathology of MND?
Degenerative condition affecting motor neurons – mainly the anterior horn cells
There is relentless and UNEXPLAINED destruction of UMN and anterior horn cells in the brain and spinal cord
Causes both UMN and LMN dysfunction
UMN and LMN affected but no sensory or sphincter loss – distinguishes from MS
Never affects eye movements – distinguishable from myasthenia gravis
What are some causes of MND?
- Family history
- Smoking
- Heavy metal
- Pesticides
- Excessive exercise
What is the initial presentation of MND?
Insidious onset of weakness throughout the body
Weakness is often first noticed in upper limbs and increasing fatigue when exercising
They may complain of clumsiness, dropping things or tripping over and also slurred speech
What are LMN signs?
- Muscle wasting
- Reduced tone
- Fasciculations
- Reduced reflexes
What are some UMN signs?
- Increased tone
- Brisk reflexes
- Rigidity + spasticity
- Babinski reflex positive
How can you diagnose MND?
- Definite: LMN + UMN signs in 3 regions
- Probable: LMN + UMN signs in 2 regions
- Probable with lab support: LMN + UMN signs in 1 region, or UMN sign in more than 1 region + electromyography (EMG) shows acute denervation in more than 2 limbs
What investigations can you do to help diagnose Motor neurone disease?
- Electromyography:in MND there will be evidence of fibrillation potentials - due to denervation of muscles due to LMN dysfunction - ( EMG is a technique for evaluating and recording the electrical activity produced by skeletal muscles.)
- Nerve conduction studies:may show modest reductions in amplitude
- MRI spine:imaging can help exclude spinal pathology which may mimic MND, such as cervical cord compression and myelopathy
- Lumbar puncture: to exclude inflammatory causes
- Pulmonary function tests:patients with MND are at risk of respiratory failure
Blood tests e.g. raised Creatinine Kinase (due to muscle destruction),
What are some differentials for MND?
- Multiple sclerosis
- Polyneuropathies
- Myasthenia gravis
- Diabetic amyotrophy
- Guillain-Barre syndrome
- Spinal cord tumours
What is the management for MND?
- Riluzole prolongs survival by 2-4 months by protecting motor neuron damage form glutamate
- Respiratory support:patients with reduced FVC can use non-invasive ventilation at home, usually BiPAP; prolongs survival by 7 months
-
Supportive treatment:
- Antispasmodics: such as baclofen
What is never affected in MND?
- Eye movements
- Sensory loss
What is MS?
A autoimmune cell-mediated demyelinating disease of the central nervous system
What are some risk factors for MS?
- Vitamin D deficiency
- Family history: HLA-DR2 is implicated; 30% monozygotic twin concordance
- EBV
- Smoking
- Obesity
- Gender (more common in females)
Describe the pathophysiology of MS?
- T-cells get through the blood brain barrier and are activated by myelin. The T-cell then changes the BBB to allow more immune cells in
- MS is a type IV hypersensitivity reaction. T-cells release cytokines and these recruit more immune cells whilst also damaging the oligodendrocytes
- B-cells will make antibodies that will destroy the myelin of the oligodendrocytes. leaving behind areas of plaque/sclera
How does MS progress?
In early disease, re-myelination can occur and symptoms can resolve. In the later stages of the disease, re-myelination is incomplete and symptoms gradually become more permanent.
A characteristic feature of MS is that lesions vary in their location over time, meaning that different nerves are affected and symptoms change over time
MS lesions change location over time is that they are “disseminated in time and space”.
What are the different types of MS?
- Relapsing-remitting:
- Secondary progressive
- Primary progressive
- Progressive relapsing
- Clinically isolated syndrome
What is Relapsing-remitting: MS?
- The most common pattern 85% of cases
- Episodic flare-ups separated by periods of remission. There isn’t full recovery after flare ups so disability increases over time.
60% will develop secondary within 15 years
What is secondary progressive MS?
Initially, the disease starts with arelapsing-remitting course, but then symptoms get progressively worse withnoperiods of remission
What is primary progressive MS?
- Symptoms get progressively worse from diseaseonsetwithno periods of remission
- Accounts for 10% of cases and is more common inolder patients
What is Progressive relapsing MS?
- One constant attack but there are bouts superimposed during which the disability increases even faster
What is clinically isolated syndrome MS?
- This describes the first episode of demyelination and neurological signs and symptoms. MS cannot be diagnosed on one episode as the lesions have not been “disseminated in time and space”.
- Patients with clinically isolated syndrome may never have another episode or develop MS. If lesions are seen on MRI scan then they are more likely to progress
What is the most common presentation of MS?
Optic neuritis- demyelination of the optic nerve
What are some features of optic neuritis?
Central scotoma (an enlarged central blind spot)
Pain with eye movement
Impaired colour vision
Relative afferent pupillary defect- pupil constricts more when light is shined in contralateral eye than when in actual eye
What are some other causes of optic neuritis?
Sarcoidosis
Systemic lupus erythematosus
Syphilis
Measles or mumps
Neuromyelitis optica
Lyme disease
What are some signs and symptoms of MS?
- Optic neuritis
- Eye movement abnormalities- double vision
- Focal weakness (incontinence, limb paralysis, Bell’s palsy)
- Focal sensory symptoms (numbness, pins and needles)
- Ataxia
What are two specific signs of MS?
- Lhermitte’s sign is an electric shock sensation that travels down the spine and into the limbs when flexing the neck
- Uhthoff’s phenomenon: worsening of symptoms following a rise in temperature, such as a hot bath
What is the primary investigation for MS and what would it show?
MRI of the brain and spine Active Lesions will take up contrasts, Old ones will not ==> Can also see Demyelinated Plaques, known as Dawson’s Fingers
Lumbar puncture with CSF electrophoresis = inflammatory proteins found in the CSF, not serum - shows you that there’s an inflammatory response in the CNS eg Oligoclonal IgG bands = CNS inflammation -
What is the diagnostic criteria for MS?
McDonald criteria
What is the McDonald criteria?
2 or more relapses and either:
- Objective evidence of two or more lesions
- Objective evidence of one and a reasonable history of a previous relapse
‘Objective evidence’ is defined as an abnormality on neurological exam, MRI or visual evoked potentials
What is used to treat a MS relapse?
- Oral or IV methylprednisolone
- Plasma exchange
What is used for maintenance of MS?
Disease-modifying drugs
Beta-interferon: decreases the level of inflammatory cytokines
- Monoclonal antibodies e.g. alemtuzumab (anti-CD52) and natalizumab (anti-α4𝛃1-integrin)
What are some complications of MS?
- GU: urinary tract infections due to retention and incontinence
- Constipation
- Depression
- Visual impairment
- Mobility impairment
- Erectile dysfunction
What is transverse myelitis?
A rare neurological condition caused by inflammation of the spinal cord.
The swelling damages the nerves and can leave permanent scars of lesionr
What are some causes of transverse myelitis?
- 60% of cases are idiopathic
- Can be seen in MS and neuromyelitis optica
Infections: TB, HIV, HSV, CMV, EBV
What are some signs and symptoms of transverse myelitis?
Muscle weakness in legs
Mobility problems
Unusual sensations and numbness
Bladder problems
Sexual problems
back pain
A lesion of the thoracic segment (T1–12) will produce upper motor neuron signs in the lower limbs, presenting as a spastic paraparesis. This is the most common location of the lesion, and therefore most individuals will have weakness in the lower limbs
Lesions of the lower cervical region (C5–T1) will cause a combination of upper and lower motor neuron signs in the upper limbs, and exclusively upper motor neuron signs in the lower limbs.
What is Myasthenia gravis?
A chronic autoimmune disorder of the post synaptic membrane at the neuromuscular junction of skeletal muscle
When does Myasthenia Gravis affect men and women?
- Symptoms peak in women in 20/30s
- Symptoms peak in men 50/60s
What are the risk factors for developing Myasthenia Gravis?
- Female 2x as common
- Autoimmune: linked to rheumatoid and SLE
-Thymoma or thymic hyperplasia: 10-15% have a thymoma and 70% have thymic hyperplasia
Describe the normal physiology of a neuromuscular junction
- Axons of motor nerves are situated across a synapse from the post-synaptic membrane on the muscle cell
- The axons release a neurotransmitter from the pre-synaptic membrane
- The neurotransmitter is acetylcholine which travels across the synapse and attaches to nicotinic receptors on the post-synaptic membrane stimulating muscle contraction
What is the main antibody that causes MG?
Acetylcholine receptor antibodies are found in patients with MG.
These antibodies bind to postsynaptic membrane acetylcholine receptors blocking them and preventing stimulation by ACh.
These antibodies also activate the complement system which damages cells further making the problem worse
What are the two other antibodies associated with MG?
- Muscle specific kinase (MuSK)
- low-density lipoprotein receptor-related protein 4 (LRP4).
They are both proteins that are important for making the acetylcholine receptor. These antibodies lead to inadequate acetylcholine receptors causing MG
What is a key feature of MG?
The more the receptors are used during muscle activity, the more they become blocked. There is less effective stimulation of the muscle with increased activity. With rest, the receptors are cleared, and the symptoms improve.
More movement=more symptoms
What is the presentation of MG?
Symptoms affect proximal muscles of the limbs and small muscles of the head and neck with:
- Difficulty climbing stairs
- Extraocular muscle weakness causing double vision
- Eyelid weakness causing drooping of the eyelids
- Weakness in facial movements
- Difficulty swallowing
- Fatigue in jaw
- Slurred speech
What are some good ways to elicit fatigability in the muscles?
Repeated blinking will exacerbate ptosis
Prolonged upward gazing will exacerbate diplopia on further testing
Repeated abduction of one arm 20 times will result in unilateral weakness when comparing both sides
What are some investigations for MG?
AChR antibodies (around 85%)
MuSK antibodies (less than 10%)
LRP4 antibodies (less than 5%)
A CT or MRI of the thymus gland is used to look for a thymoma.
The edrophonium test can be helpful where there is doubt about the diagnosis.
What is the edrophonium test?
Patients are given IV neostigmine. Normally cholinesterase enzymes in the neuromuscular junction break down ACh.
As a result, the level of acetylcholine at the neuromuscular junction rises, temporarily relieving the weakness.
A positive result suggests a diagnosis of myasthenia gravis.
What are some treatments of MG?
Pyridostigmine is a cholinesterase inhibitor that prolongs the action of acetylcholine and improves symptoms
Immunosuppression (e.g., prednisolone or azathioprine) suppresses the production of antibodies
Thymectomy can improve symptoms, even in patients without a thymoma
Rituximab (a monoclonal antibody against B cells) is considered where other treatments fail
What is a major complication of MG?
Myasthenic crisis where there is an acute worsening of symptoms, often triggered by another illness such as a respiratory tract infection. Respiratory muscle weakness can lead to respiratory failure.
What is the treatment of a MG crisis?
Patients may require non-invasive ventilation or mechanical ventilation.
Treatment is with IV immunoglobulins and plasmapheresis.
What is neurofibromatosis?
A genetic condition that causes nerve tumours to develop throughout the nervous system
These tumours are benign but can cause neurological and structural problems
Neurofibromatosis type 1 is more common than neurofibromatosis type 2.
What is the genetic mutation for neurofibromatosis?
Neurofibromatosis type 1 gene is found on chromosome 17.
It codes for a protein called neurofibromin, which is a tumour suppressor protein
autosomal dominant pattern
What are the features of neurofibromatosis?
CRABBING
C- Cafe-au-lait spots
R- Relative with NF1
A- Axillary or inguinal freckling
BB- Bony Bony dysplasia, such as Bowing of a long bone or sphenoid wing dysplasia
I- Iris hamartomas (Lisch nodules), which are yellow-brown spots on the iris
N- Neurofibromas
G- Glioma of the optic pathway
What are some complications of NF1?
Malignant peripheral nerve sheath tumours
Gastrointestinal stromal tumours
What is hydrocephalus?
A build up of CSF in the brain and spinal cord. It is usually caused by overproduction of CSF or a problem with the drainage or absorption of it
Where is CSF usually found?
CSF is found in the ventricles and it provides a cushion for brain tissue
What in the brain creates CSF?
The choroid plexus in the ventricles and also the walls of the ventricles
How is CSF absorbed?
It is absorbed into the venous system by the arachnoid granules in the subarachnoid space
What are some types of hydrocephalus?
Communicating: where there is communication between the ventricles and the subarachnoid space and the problem and the problem lies outside of the ventricular system (e.g. due to reduced absorption or blockage of the venous drainage system), may also be due to increased CSF production
Non-communicating/obstructive - the flow of CSF is obstructed within the ventricles or between the ventricles and the subarachnoid space
Normal pressure hydrocephalus is a condition with low-grade hydrocephalus with intermittently raised ICP.
What is the most common cause of hydrocephalus?
- Most common cause is aqueduct stenosis leading to insufficient drainage of CSF
- The cerebral aqueduct that connects the third and fourth ventricle is stenosed (narrowed). This blocks the normal flow of CSF out of the third ventricle, causing CSF to build up in the lateral and third ventricles.
What are some other causes of hydrocephalus?
Arachnoid cysts can block the outflow of CSF if they are large enough
Arnold-Chiari malformation is where the cerebellum herniates downwards through the foramen magnum, blocking the outflow of CSF
Chromosomal abnormalities and congenital malformations can cause obstruction to CSF drainage.
What is normal pressure hydrocephalus?
Its a form of communicating hydrocephalus in which excess cerebrospinal fluid (CSF) builds up in the ventricles, leading to normal or slightly elevated cerebrospinal fluid pressure.
What is the classic triad in NPH?
- Gait abnormality
- Urinary incontinence
- Dementia
What are the investigations for NPH?
- Neuroimaging: CT will show enlarged ventricles including temporal horns but with normal sulci.
- High volume lumbar puncture with removal of 30-50ml of CSF. Gait and cognitive function are typically tested just before LP to asses for signs of symptomatic improvement
What is the treatment for NPH?
The most common type is a ventriculoperitoneal shunt.
- It drains CSF to the peritoneal cavity. Adjustable valves allow fine-tuning of CSF drainage
- Symptoms improve in 70-90% of patients
What is narcolepsy?
A chronic neurological disorder in which the brain loses its normal ability to regulate the sleep-wake cycle
What are the two types of narcolepsy?
Type 1: Involves cataplexy and patients have low levels or orexin (also known as hypocretin) in the CSF
Type 2: does not involves cataplexy
What does hypocretin do normally and how is this affected in type 1 narcolepsy?
It’s a neuropeptide that regulates arousal, wakefulness and appetite
It is thought in type 1 narcolepsy that mutations lead to the loss of the hypothalamic hypocretin-containing neurons, via autoimmune destruction.
What gene mutation is found in 95% of patients with type 1 narcoplepsy?
HLA DR2
What is cataplexy?
A bilateral loss of tone in antigravity muscles provoked by emotions such as laughter startle, excitement or anger
What are the features of a cataplexy attack?
- Severity can vary from barely susceptible slackening of the facial muscles, dropping of the jaw or the entire head, to weakness at the knees or collapse onto floor
- Slurred speech and visual symptoms
- Intact hearing, awareness and consciousness
- Variable frequency
What are the key presentations of narcolepsy?
- Excessive daytime sleepiness
- Disrupted night-time sleep and vivid dreams
- Cataplexy
- hypnopompic hallucinations
- sleep paralysis
What are some differentials for narcolepsy?
Sleep apnoea
Sleep deprivation
Automatism
Sleep paralysis
What is the management for narcolepsy?
Scheduled naps
- CNS stimulants: modafinil, or Methylphenidate
- Antidepressants: (for cataplexy) e.g. clomipramine, SSRIs, venlafaxine
Damage to which structure is likely to cause Horner’s syndrome?
- Damage to the cervical sympathetic chain by removing sympathetic innervation to the head and neck
What are some causes of Horner syndrome?
- Stroke
- MS
- Swelling
- Syringomyelia (cyst in the spinal cord)
- Pancoast tumour (apex of the lung)
- Trauma
- Thyroidectomy
- Carotid aneurysm
- Carotid artery dissection
- Cavernous sinus thrombosis
- Cluster headache
What are the symptoms of Horner syndrome?
- Ptosis (eyelid drooping)
- Miosis (excessive constriction of the pupil)
- Anhidrosis (Decreased sweating in half of the face)
What is an relative afferent pupillary defect?
- Normally light shone into either eye should constrict both pupils equally
- In RAPD ( affecting either the retina or optic nerve) light directed into the affected eye will cause only mild constriction of both pupils (due to decreased response to light from the afferent defect)
- Meanwhile, light in the unaffected eye will cause normal constriction of both pupils (due to an intact efferent path and an intact consensual pupillary reflex)
What can cause RAPD?
MS
Glaucoma
Severe retinal disease
Optic nerve lesion
What is the consensual pupillary light reflex?
A consensual pupillary reflex is response of a pupil to light that enters the contralateral (opposite) eye.
What branch of the autonomic nervous system causes a dilated pupil and what branch causes a constricted pupil?
- Pupil constriction is mediated via parasympathetic activation
- Pupil dilation via sympathetic activation
a lesion on the parasympathetic nervous system of CN3 will lead to what kind of pupil?
A fixed dilated pupil
A lesion on the sympathetic chain innervating the eye will lead to what?
Fixed constricted pupil, as only parasympathetic innervation is received - think in horners syndrome
What is myelopathy and what are some causes of it?
Any disorder that affects the spinal cord
Can be caused by:
- Trauma
- Degenerative causes
- Inflammation
- Tumour
- Vascular
- Infectious diseases
What is the difference between a myelopathy and a radiculopathy?
Radiculopathy is the term used to describe pinching of the nerve roots as they exit the spinal cord or cross the intervertebral disc, rather than the compression of the cord itself (myelopathy).
Outline some degenerative and inflammatory disorders and infectious diseases that can cause myelopathy
!!The commonest cause of myelopathy is disc-osteophyte cord compression
Infectious Diseases: Tuberculosis, HIV-associated myelopathy, or viral infections
Degenerative Disorders: Cervical spondylosis (age-related changes in the spine), spinal stenosis (narrowing of the spinal canal), or herniated discs
Inflammatory Disorders: multiple sclerosis, transverse myelitis,
What are some symptoms of a myelopathy?
Proximal muscle weakness
Clumsy hands, struggling with keyboard, texting on mobile etc
Also difficulty walking, legs feel ‘not my own’, ‘like lead boots, not doing what I tell them’ - Similar to peripheral neuropathy
Bowel/bladder symptoms and sexual dysfunction
What are some signs of a myelopathy?
Upper motor neurone signs (Long tract signs)
Babinski
Clonus
Cross-adductors sign
Hoffman’s sign
Loss of fine finger movements - treacle hands
What are some investigations for a myelopathy?
Because the term myelopathy describes a clinical syndrome that can be caused by many pathologies the differential diagnosis of myelopathy is extensive
The best way to visualize the spinal cord is magnetic resonance imaging (MRI)
CT and xrays
What is a radiculopathy?
A pinched nerve that refers to a set of conditions in which one or more nerves are affected and do not work properly
The problem occurs at or near the root of the nerve shortly after its exit from the spinal cord. But symptoms radiate to other parts of the body innervated by that nerve
What are the main causes of a radiculopathy?
Changes in tissue surrounding the nerve roots
For example intravertebral disc herniation ( Most commonly at C7 and then C6 level)
osteoarthritis,
ligamentous hypertrophy,
spondylolisthesis
Cancer
Infections like Shingles, HIV, Lyme Disease
Proximal Diabetic neuropathy
What will the patient complain of in a radiculopathy?
99% patients have radiating limb pain, often in the pattern of the dermatome, sharp/shooting in character, - going down limbs
What nerve roots are most commonly affected in the upper limb?
C6 and C7
C6 Thumb up - C6 nerve root - myotone - Biceps (6 letters
Middle finger - C7 - mytone - Triceps (7 letters)
What are the most common nerve roots affected in the lower limb?
L5 and S1
L5: the dorsum or top of foot/big toe and dorsiflexion
S1: all the S’s: side of foot, sole of foot, small toe area
Plantar flexion
Ankle jerk
What is the management of radiculopathy?
Medications, like nonsteroidal anti-inflammatory drugs, opioid medicines or muscle relaxants, to manage the symptoms
Weight loss strategies to reduce pressure on the problem area
Physical therapy to strengthen the muscles and prevent further damage
Steroid injections to reduce inflammation and relieve pain
Surgery to reduce pressure on nerve root
What is spinal cord compression?
Compression of the spinal cord resulting in UMN signs and symptoms depending on where the compression is
It is a medical emergency and can lead to paralysis
What are some causes of spinal cord compression?
Vertebral body neoplasms (most common cause)
-
Spinal pathology
-
Disc herniation
- When centre of disc (nucleus pulposus) has moved out through the annulus (outer part of disc) resulting in pressure on nerve root and pain
-
Disc herniation
-
Disc prolapse
- When nucleus pulposus moves and presses against the annulus - can cause a bulge in the disc
- Primary spinal cord tumour e.g. glioma, neurofibroma
- Infection e.g. epidural abscess
- Haematoma
What are the most common cancers that metastasis to the spinal cord?
- Lung
- Breast
- Prostate
- Kidney
- Myeloma
- Lymphoma
What are the symptoms of spinal cord compression?
- Sensory loss 1-2 cord segments below the level of lesion
- UMN signs below the level of lesion
- Progressive symmetrical weakness of legs
LMN signs at level of lesion
Bladder sphincter involvement – hesitancy, frequency, painless retention
What are some UMN signs?
Hypertonia - an abnormally high level of muscle tone or tension
Hyperreflexia - overactive or overresponsive bodily reflexes, twitching
Spasticity
Positive Babinski sign - extension of large toe when plantar surface of foot is stroked
What are some LMN signs?
Hyporeflexia/ areflexia - decreased or absent reflexes
Hypotonia/ atonia - loss of muscle tone
Flaccid muscle weakness or paralysis
Fasciculations – small involuntary muscle twitches,
Muscle atrophy
What would be the features of a complete spinal cord compression?
All motor and sensory function lost below the SCI level
What is the concern if there is sphincter involvement in spinal cord compression
This is a late and bad sign signalling a poorer prognosis
What investigation would you do in suspected spinal chord compression?What investigation would you do in suspected spinal chord compression?
Do not delay investigations
- MRI: gold standard; identifies cause and site of compression
- Biopsy/surgical exploration may be required to identify the nature of any mass
- Screening blood tests: FBC, ESR, B12, U&E’s, syphilis serology, LFT, PSA
- Chest x-ray: to check for TB or lung malignancy
What is the treatment for spinal cord compression?
Neurosurgery to decompress cord
Dexamethasone to reduce inflammation
What is the cauda equina?
It’s a nerve bundle formed by the lumbar, sacral and coccygeal nerves, as they travel down the spinal canal together to reach their corresponding openings.
Distal to level of termination of spinal cord at L1/L2.
Cauda equina syndrome caused by damage to the peripheral nerves at the cauda equina
What functions do nerves in teh cauda equina have?
nerves in the cauda equina carry motor innervation for the genitals, both internal and external anal sphincter, detrusor vesicae, and muscles of the leg. They are also responsible for skin sensations in these regions.
What is cauda equina syndrome?
Cauda equina syndrome (CES) is a neurosurgical emergency which occurs when the bundle of nerves below the end of the spinal cord are compressed.
Name some causes of cauda equina syndrome
Herniation of lumbar disc – most commonly at L4/L5 and L5/S1
Spondylolisthesis – most commonly anterolisthesis (vertebra moves forward)
Trauma – car crash, gun shot, bleeding via haematomas
Tumours
Infection
Post-op haematoma
Causes of cauda equina syndrome - what is spondylolisthesis? What pathophysiological effects will be seen as a result of spondylolisthesis?
Spondylolisthesis is where one of the bones in your spine, called a vertebra, slips forward
most commonly anterolisthesis (vertebra moves forward)
Slippage of one vertebra over the one below
Nerve root comes out ABOVE the disc therefore root affected will be the one BELOW the disc herniation
E.g. L4/L5 herniation 🡪 L5 nerve root compression
What are some symptoms/signs of cauda equina syndrome
Saddle anaesthesia
Less bladder and bowel control – increased tone of anal sphincter and muscle wall of bladder
Erectile dysfunction (or other sexual dysfunction)
Lumbosacral pain
Leg weakness – flaccid and areflexic
Paraplegia
Signs
Areflexia
Fasciculations
Loss of bowel/bladder control
Urinary retention
WILL SEE LOWER MOTOR NEURON SIGNS ONLY*
What are some investigations for cauda equina syndrome?
MRI spinal cord (diagnostic)
PR exam - feel for any abnormalities, and check anal tone
Testing nerve roots/reflexes
Knee flexion – test L5-S1
Ankle plantar flexion – test S1-S2
Straight leg raising – L5, S1
Femoral stretch test – L4
What is the management for cauda equina syndrome?
Management
Refer to neurosurgeon ASAP to relive pressure
Surgical decompression
High dose dexamethasone
Corticosteroids
What is the difference between cauda equina syndrome and spinal chord compression?
Spinal cord compression is more likely to be thoracic with neurology in the upper limbs with bladder & bowel changes being a very late sign.
Whereas in cauda equina, the lower limbs are classically affected with earlier bladder & bowel dysfunction and saddle anaesthesia.
What is anterior spinal chord syndrome? What is the main cause
Anterior spinal artery syndrome (also known as cord) is caused by the ischaemia of the anterior spinal artery
This results in loss of function of the anterior two thirds of the spinal cord
It is usually the result of a flexion/compression injury
What is affected in anterior spinal chord syndrome?
- It affects the descending corticospinal tract and the ascending spinothalamic tract as well as autonomic fibres
- This results in loss of motor function, loss of pain and temperature sensation
- Also causes hypotension
What would you see on examination of someone with anterior chord syndrome?
- Lower limbs more affected
- Loss of pain and temperature
- Loss of motor function
Preserved the DC tract: proprioception and vibratory sensation
What is Peripheral Neuropathy?
What are the types of peripheral nerve disease?
Nerve pathology outside of the CNS that affects the peripheral nerves
Mononeuropathy: a process affecting a single nerve
Polyneuropathy: many nerves involved. Usually describes a symmetrical disease, and it usually begins distally. Can be sensory, motor or mixed.
What are some causes of peripheral nerve disease?
DAVIDE
Diabetes
Alcohol
Vitamin B12
Infective: GBS
Drugs: Isoniazid
Every vasculitis
Multiple myeloma
What are some examples of mononeuropatheis?
- Carpal tunnel syndrome
- Wrist drop
- Claw hand
- Foot drop
What is carpal tunnel syndrome?
A collection of symptoms caused by the compression of the median nerve in the carpal tunnel
What are some causes/risk factor of CTS?
- Mostly idiopathic
HODPARAR
- Hypothyroidism
- Obesity
- Diabetes
- Pregnancy
- Acromegaly
- Rheumatoid Arthritis
- Amyloidosis
- Repetitive Strain Injury
Females due to narrower wrists so more likely to have compression
Over 30s
exam tip - if you see a patient with bilateral carpal tunnel syndrome, look for features that might suggest underlying rheumatoid arthritis, diabetes, acromegaly or hypothyroidism.
What movements are affected in carpal tunnel syndrome?
Weakness of thumb movements
Weakness of grip strength
Difficulty with fine movements involving the thumb
Wasting of the thenar muscles
What muscles in CTS?
Thenar muscles
Flexor pollicis brevis
Abductor pollicis
Opponens Pollicis
What are some tests that can be used to diagnose CTS?
Phalen Test
Tinel Test
Nerve conduction studies:
small electrical current is applied by an electrode (nerve stimulator) to the median nerve on one side of the carpal tunnel -
Recording electrodes over the median nerve on the other side of the carpal tunnel record the electrical current that reaches them
What is Phalen’s test?
Phalen’s test - flex the wrists are far as possible and hold that position for a minute, this results in numbness in the areas of the hand innervated by the median nerve in people with carpal tunnel syndrome
What is Tinel’s test?
tap the transverse carpal ligament, this reproduces the symptoms of tingling or feelings of pins and needles in areas of the hand served by the median nerve
TOM TIP: I think of tapping a tin can (Tinel’s) to remember the difference between Phalen’s and Tinel’s test.
What is the management for CTS?
Rest and altered activities
Wrist splints that maintain a neutral position of the wrist can be worn at night (for a minimum of 4 weeks)
Steroid injections
Surgery
What can cause wrist drop?
Damage to the radial nerve
Compression of the radial nerve at the humerus
What is the presentation of an ulnar nerve palsy?
Claw hand (4th/5th fingers claw up)
What is the treatment Wrist drop and Claw hand?
Splint
Analgesia
What are the nerve roots of the common peroneal nerve?
L4-S1
(Branch off the Sciatic nerve)
What are some causes of foot drop?
Common peroneal nerve palsy!1
muscle or spinal cord trauma
Toxins
More commonly: Diseases, such as:
Stroke, ALS,, Charcot Marie Tooth, MS, Cerebral Palsy, GBS
Axillary nerve - what does it do? When should you check its function
supplies motor and sensation to deltoid
Check sensation to the deltoid with a neuro tip if they’ve broken/dislocated their shoulder, for axillary nerve damage
What is Mononeuritis Multiplex?
A type of peripheral neuropathy where there is damage to several individual nerves due to systemic causes.
What are the causes of Mononeuritis Multiplex?
Systemic necrotizing vasculitis
* Polyarteritis nodosa
* ANCA-associated (Churg–Strauss syndrome, Granulomatosis with polyangiitis)
Vasculitis associated with connective tissue disorders
* Rheumatoid arthritis.
* Sjögren’s syndrome.
* SLE.
Hypersensitivity vasculitis
* Drug-induced vasculitis.
* Malignancy.
Infections
* Bacterial: Lyme disease, TB, syphilis.
* Viral: HIV, herpes zoster, CMV
What are polymyositis and dermatomyositis?
Autoimmune disorders where there is inflammation in the muscles.
Polymyositis refers to chronic inflammation of the muscles. Dermatomyositis is a connective tissue disorder where there is chronic inflammation of the skin and muscles
Leads to proximal muscle weakness and muscle pain
What is the key enzyme for diagnosing polymyositis and dermatomyositis?
The creatine kinase blood test. It is an enzyme that is found inside muscle. Inflammation of the muscles can lead to the release of it.
It can also be raised in Acute kidney injury, Myocardial infarction, Statins
Strenuous exercise
What is the antibody found in polymyositis?
Anti-Jo-1 antibodies: polymyositis (but often present in dermatomyositis)
What is the management for polymyositis and dermatomyositis?
Patients should be assessed for underlying cancer
Corticosteroids are first line
Then immunosuppressants
Then infliximab - (targets TNF-a )
What is Wernicke’s encephalopathy?
A neurological emergency resulting from thiamine deficiency
What is Korsakoff’s Syndrome? How is it related to Wernicke’s?
Hypothalamic damage & cerebral atrophy due to thiamine (vitamin B1) deficiency (eg in alcoholics).
Wernicke’s encephalopathy is the acute, reversible stage of the syndrome, and if left untreated it can later lead to Korsakoff syndrome, which is chronic and irreversible.
What are some risk factors for Wernicke’s encephalopathy?
- Alcohol abuse
- Malnutrition
- Anorexia
- Malabsorption due to stomach cancer and IBD
- Prolonged vomiting e.g. due to chemotherapy, hyperemesis
How can a lack of thiamine (vit B1) affect the brain?
- Thiamine deficiency impairs glucose metabolism and this leads to a decrease in cellular energy
- The brain is particularly vulnerable to impaired glucose metabolism since it utilises so much energy
What is the classic triad seen in Wernicke’s?
- Confusion
- Ataxia
- *ophthalmoplegia** (nystagmus,
lateral rectus or conjugate gaze palsies).
What does Wernicke - Korsakoff syndrome predominantly target? What symptoms does this cause?
Mainly targets the limbic system, causing severe memory impairment:
- Anterograde amnesia: inability to create new memories
- Retrograde amnesia: inability to recall previous memories.
- Confabulation: creating stories to fill in the gaps in their memory which they believe to be true.
- Behavioural changes
What is the management for Wernicke’s?
- Give thiamine
- Oral supplementation until no loner at risk
- Correct magnesium deficiency
- If there is coexisting hypoglycaemia, correct it
Why do you need to give Thiamine before you give glucose in a patient with Wernicke’s
It is important to stabilise thiamine levels because without thiamine pyrophosphate, most of the glucose will become lactic acid and that can lead to metabolic acidosis. (often the case in
this group of patients),
What investigations would you do in suspected Wernicke’s encephalopathy?
- Diagnosis is typically made based on clinical presentation
- Bloods including LFTs: measure thiamine levels, measure blood alcohol levels, liver function may be deranged in alcoholism
Outline what is assessed in the GCS
Eyes:
Eye-opening spontaneously 4 points
Eye-opening to sound 3 points
Eye-opening to pain 2 points
No response 1 point
Verbal response (V)
Orientated 5 points
Confused conversation 4 points
Inappropriate words 3 points
Incomprehensible sounds 2 points
No response 1 point
Motor response (M) - You should score the patient based on the highest scoring response you were able to elicit in any single limb
Obeys command 6 points
Localises to pain 5 points
Withdraws to pain (normal flexion) 4 points
Abnormal Flexion (decorticate posture 3 points)
Abnormal extension (decerebrate posture 2 points)
No response 1 point
