Neurology Flashcards

Question

Managing complications of subarachnoid haemorrhage

Answer 1

Hydrocephalus refers to increased cerebrospinal fluid, causing expansion of the ventricles. Treatment options include: Lumbar puncture External ventricular drain (a drain inserted into the brain ventricles to drain CSF) Ventriculoperitoneal (VP) shunt (a catheter connecting the ventricles with the peritoneal cavity) Seizures are treated with anti-epileptic drugs.

Answer 2

Multiple sclerosis (MS) is a chronic and progressive autoimmune condition involving demyelination in the central nervous system. The immune system attacks the myelin sheath of the myelinated neurones. Multiple sclerosis typically presents in young adults (under 50 years) and is more common in women.

Answer 3

Myelin covers the axons of neurones and helps electrical impulses travel faster. Myelin is provided by cells that wrap themselves around the axons: Oligodendrocytes in the central nervous system Schwann cells in the peripheral nervous system Multiple sclerosis affects the central nervous system (the oligodendrocytes). Inflammation and immune cell infiltration cause damage to the myelin, affecting the electrical signals moving along the neurones. When a patient presents with symptoms of an MS attack (e.g., an episode of optic neuritis), there are often other demyelinating lesions throughout the central nervous system, most of which are not causing symptoms. In early disease, re-myelination can occur, and the symptoms can resolve. In the later stages of the disease, re-myelination is incomplete, and the symptoms gradually become more permanent. A characteristic feature of MS is that lesions vary in location, meaning that the affected sites and symptoms change over time. The lesions are described as “disseminated in time and space”.

Answer 4

The cause of the multiple sclerosis is unclear, but there is growing evidence that it may be influenced by: Multiple genes Epstein–Barr virus (EBV) Low vitamin D Smoking Obesity

Answer 5

Symptoms usually progress over more than 24 hours. Symptoms tend to last days to weeks at the first presentation and then improve. There are many ways MS can present, depending on the location of the lesions.

Answer 6

Optic neuritis is the most common presentation of multiple sclerosis. It involves demyelination of the optic nerve and presents with unilateral reduced vision, developing over hours to days. Key features are: Central scotoma (an enlarged central blind spot) Pain with eye movement Impaired colour vision Relative afferent pupillary defect A relative afferent pupillary defect is where the pupil in the affected eye constricts more when shining a light in the contralateral eye than when shining it in the affected eye. When testing the direct pupillary reflex, there is a reduced pupil response to shining light in the eye affected by optic neuritis. However, the affected eye has a normal pupil response when testing the consensual pupillary reflex. Other causes of optic neuritis include: Sarcoidosis Systemic lupus erythematosus Syphilis Measles or mumps Neuromyelitis optica Lyme disease Patients presenting with acute loss of vision need urgent ophthalmology input. Optic neuritis is treated with high-dose steroids. Changes on an MRI scan help to predict which patients will go on to develop MS.

Answer 7

Lesions affecting the oculomotor (CN III), trochlear (CN IV) or abducens (CN VI) can cause double vision (diplopia) and nystagmus. Oscillopsia refers to the visual sensation of the environment moving and being unable to create a stable image. Internuclear ophthalmoplegia is caused by a lesion in the medial longitudinal fasciculus. The nerve fibres of the medial longitudinal fasciculus connect the cranial nerve nuclei (“internuclear”) that control eye movements (the 3rd, 4th and 6th cranial nerve nuclei). These fibres are responsible for coordinating the eye movements to ensure the eyes move together. It causes impaired adduction on the same side as the lesion (the ipsilateral eye) and nystagmus in the contralateral abducting eye. A lesion in the abducens (CN VI) causes a conjugate lateral gaze disorder. Conjugate means connected. Lateral gaze is where both eyes move to look laterally to the left or right. When looking laterally in the direction of the affected eye, the affected eye will not be able to abduct. For example, in a lesion involving the left eye, when looking to the left, the right eye will adduct (move towards the nose), and the left eye will remain in the middle.

Answer 8

Multiple sclerosis may present with focal weakness, for example: Incontinence Horner syndrome Facial nerve palsy Limb paralysis Multiple sclerosis may present with focal sensory symptoms, for example: Trigeminal neuralgia Numbness Paraesthesia (pins and needles) Lhermitte’s sign Lhermitte’s sign describes an electric shock sensation that travels down the spine and into the limbs when flexing the neck. It indicates disease in the cervical spinal cord in the dorsal column. It is caused by stretching the demyelinated dorsal column. Transverse myelitis refers to a site of inflammation in the spinal cord, which results in sensory and motor symptoms depending on the location of the lesion.

Answer 9

Ataxia is a problem with coordinated movement. It can be sensory or cerebellar. Sensory ataxia is due to loss of proprioception, which is the ability to sense the position of the joint (e.g., is the joint flexed or extended). This results in a positive Romberg’s test (they lose balance when standing with their eyes closed) and can cause pseudoathetosis (involuntary writhing movements). A lesion in the dorsal columns of the spine can cause sensory ataxia. Cerebellar ataxia results from problems with the cerebellum coordinating movement, indicating a cerebellar lesion.

Answer 10

The disease course is highly variable between individuals. Some patients have mild relapsing-remitting episodes for life, while others have symptoms that progress without any improvement. There are classifications used to describe the patterns, which are not separate conditions but part of a spectrum of disease activity. Clinically isolated syndrome describes the first episode of demyelination and neurological signs and symptoms. Patients with clinically isolated syndrome may never have another episode or may go on to develop MS. Lesions on an MRI scan suggest they are more likely to progress to MS. Relapsing-remitting MS is the most common pattern when first diagnosed. It is characterised by episodes of disease and neurological symptoms followed by recovery. The symptoms tend to occur in different areas with each episode. It can be further classified based on whether the disease is active or worsening: Active: new symptoms are developing, or new lesions are appearing on the MRI Not active: no new symptoms or MRI lesions are developing Worsening: there is an overall worsening of disability over time Not worsening: there is no worsening of disability over time Secondary progressive MS is where there was relapsing-remitting disease, but now there is a progressive worsening of symptoms with incomplete remissions. Symptoms become increasingly permanent. Secondary progressive MS can be further classified based on whether the disease is active or progressing. Primary progressive MS involves worsening disease and neurological symptoms from the point of diagnosis without relapses and remissions. This can be further classified based on whether it is active or progressing.

Answer 11

The diagnosis is made by a neurologist based on the clinical picture and symptoms suggesting lesions that change location over time. Other causes for the symptoms need to be excluded. Investigations can support the diagnosis: MRI scans can demonstrate typical lesions Lumbar puncture can detect oligoclonal bands in the cerebrospinal fluid (CSF)

Answer 12

A specialist multidisciplinary team (MDT) manages multiple sclerosis, including neurologists, specialist nurses, physiotherapists and occupational therapists. Disease-modifying therapies aim to induce long-term remission with no disease activity. Many options target various aspects of the immune system to reduce relapses and disease progression. Relapses may be treated with steroids. The NICE guidelines (2022) suggest either: 500mg orally daily for 5 days 1g intravenously daily for 3–5 days (where oral treatment has previously failed or where relapses are severe) Symptomatic treatments include: Exercise to maintain activity and strength Fatigue may be managed with amantadine, modafinil or SSRIs Neuropathic pain may be managed with medication (e.g., amitriptyline or gabapentin) Depression may be managed with antidepressants, such as SSRIs Urge incontinence may be managed with antimuscarinic medications (e.g., solifenacin) Spasticity may be managed with baclofen or gabapentin Oscillopsia may be managed with gabapentin or memantine

Answer 13

Motor neurone disease is a term that encompasses a variety of specific diseases affecting the motor nerves. Motor neurone disease is a progressive, eventually fatal condition where the motor neurones stop functioning. There is no effect on the sensory neurones. Sensory symptoms suggest an alternate diagnosis. Amyotrophic lateral sclerosis (ALS) is the most common and well-known type of motor neurone disease. Stephen Hawking had amyotrophic lateral sclerosis. Progressive bulbar palsy is the second most common form of motor neurone disease. It primarily affects the muscles of talking and swallowing (the bulbar muscles). Other types to be aware of are progressive muscular atrophy and primary lateral sclerosis.

Answer 14

Motor neurone disease involves a progressive degeneration of both the upper and lower motor neurones. The sensory neurones are spared. The exact cause is unclear, although several mechanisms have been considered. Many genes have been linked with an increased risk of developing the condition. Family history is important as around 5-10% of cases are inherited. There seems to be an increased risk with smoking and exposure to heavy metals and certain pesticides.

Answer 15

The typical patient is a late middle-aged (e.g., 60) man, possibly with an affected relative. There is an insidious, progressive weakness of the muscles throughout the body, affecting the limbs, trunk, face and speech. The weakness is often first noticed in the upper limbs. There may be increased fatigue when exercising. They may complain of clumsiness, dropping things more often or tripping over. They can develop slurred speech (dysarthria). Signs of lower motor neurone disease: Muscle wasting Reduced tone Fasciculations (twitches in the muscles) Reduced reflexes Signs of upper motor neurone disease: Increased tone or spasticity Brisk reflexes Upgoing plantar reflex

Answer 16

The diagnosis needs to be made very carefully. It is based on the clinical presentation after excluding other conditions. It should only be made by a specialist when there is certainty. The diagnosis is often delayed, causing stress.

Answer 17

There are no effective treatments for halting or reversing the progression of the disease. Riluzole can slow the progression of the disease and extend survival by several months in ALS. Non-invasive ventilation (NIV) can be used to support breathing when the respiratory muscles weaken. Management of the condition involves supporting the person and their family: Breaking bad news effectively and supportively Multidisciplinary team (MDT) input to support and maintain their quality of life Symptom control (e.g., baclofen for muscle spasticity and antimuscarinic medical for excessive saliva) Benzodiazepines may help breathlessness worsened by anxiety Advanced directives to document their wishes as the disease progresses End-of-life care Patients with motor neurone disease tend to die of respiratory failure or pneumonia.

Answer 18

Parkinson’s disease is a condition where there is a progressive reduction of dopamine in the basal ganglia of the brain, leading to disorders of movement. The symptoms are characteristically asymmetrical, with one side affected more than the other. There is a classic triad of features in Parkinson’s disease: Resting tremor Rigidity Bradykinesia

Answer 19

The basal ganglia are a group of structures situated in the middle of the brain. They are responsible for coordinating habitual movements such as walking or looking around, controlling voluntary movements and learning specific movement patterns. Part of the basal ganglia called the substantia nigra produces a neurotransmitter called dopamine. Dopamine is essential for the correct functioning of the basal ganglia. In Parkinson’s disease, there is a gradual but progressive fall in the production of dopamine.

Answer 20

The typical patient is an older aged man around the age of 70. Unilateral Tremor The tremor in Parkinsons has a frequency of 4-6 Hz, meaning it occurs 4-6 times a second. This is described as a “pill rolling tremor” because it looks like they are rolling a pill between their fingertips and thumb. It is more pronounced when resting and improves on voluntary movement. The tremor is worsened if the patient is distracted. Asking them to do a task with the other hand, such as miming the motion of painting a fence, can exaggerate the tremor. “Cogwheel” Rigidity Rigidity is a resistance to passive movement of a joint. If you take their hand and passively flex and extend their arm at the elbow, you will feel a tension in their arm that gives way to movement in small increments (like little jerks). This is what leads to the cogwheel description. Bradykinesia Bradykinesia describes how their movements get slower and smaller. This presents in a number of ways: Their handwriting gets smaller and smaller (this is a classic presenting complaint in exams) They can only take small steps when walking (“shuffling gait”) They have difficulty initiating movement (e.g. from standing still to walking) They have difficulty in turning around when standing, having to take lots of little steps They have reduced facial movements and facial expressions (hypomimia) Other Features There are a number of other features that often affect patients with Parkinson’s disease: Depression Sleep disturbance and insomnia Loss of the sense of smell (anosmia) Postural instability Cognitive impairment and memory problems

Answer 21

TOM TIP: A common exam task challenges you to distinguish between the tremor of Parkinson’s disease and benign essential tremor. The table below gives some tips on this: Parkinson’s Tremor Benign Essential Tremor Asymmetrical Symmetrical 4-6 hertz 5-8 hertz Worse at rest Improves at rest Improves with intentional movement Worse with intentional movement Other Parkinson’s features No other Parkinson’s features No change with alcohol Improves with alcohol

Answer 22

Multiple System Atrophy This is a rare condition where the neurones of multiple systems in the brain degenerate. It affects the basal ganglia as well as multiple other areas. The degeneration of the basal ganglia lead to a Parkinson’s presentation. The degeneration in other areas lead to autonomic dysfunction (causing postural hypotension, constipation, abnormal sweating and sexual dysfunction) and cerebellar dysfunction (causing ataxia). Dementia with Lewy Bodies This is a type of dementia associated with features of Parkinsonism. It causes a progressive cognitive decline. There are associated symptoms of visual hallucinations, delusions, disorders of REM sleep and fluctuating consciousness. Others Two other Parkinson’s-plus syndromes exist that involves a number of complex progressive neurological features: Progressive Supranuclear Palsy Corticobasal Degeneration

Answer 23

Parkinson’s disease is diagnosed clinically based on symptoms and examination. The diagnosis should be made by a specialist with experience in diagnosing Parkinson’s. NICE recommend using the UK Parkinson’s Disease Society Brain Bank Clinical Diagnostic Criteria.

Answer 24

Treatment is initiated and guided by a specialist, and is tailored to each individual patient and their response to different medications. There is no cure, so treatment is focused on controlling symptoms and minimising side effects. Patients describe themselves as “on” when the medications are acting and they are moving freely, and “off” when the medications wear out, they have significant symptoms and their next dose is due. Levodopa This is synthetic dopamine given orally to boost their own dopamine levels. It is usually combined with a drug that stops levodopa being broken down in the body before it gets the chance to enter the brain. These are peripheral decarboxylase inhibitors. Examples are carbidopa and benserazide. Combination drugs are: Co-benyldopa (levodopa and benserazide) Co-careldopa (levodopa and carbidopa) Levodopa is the most effective treatment for symptoms but becomes less effective over time. It is often reserved for when other treatments are not managing to control symptoms. The main side effect of dopamine is when the dose is too high patients develop dyskinesias. Theses are abnormal movements associated with excessive motor activity. Examples are: Dystonia: This is where excessive muscle contraction leads to abnormal postures or exaggerated movements. Chorea: These are abnormal involuntary movements that can be jerking and random. Athetosis: These are involuntary twisting or writhing movements usually in the fingers, hands or feet. COMT Inhibitors The main example of this is entacapone. These are inhibitors of catechol-o-methyltransferase (COMT). The COMT enzyme metabolises levodopa in both the body and brain. Entacapone is taken with levodopa (and a decarboxylase inhibitor) to slow breakdown of the levodopa in the brain. It extends the effective duration of the levodopa. Dopamine Agonists These mimic dopamine in the basal ganglia and stimulate the dopamine receptors. They are less effective than levodopa in reducing symptoms. They are usually used to delay the use of levodopa and are then used in combination with levodopa to reduce the dose of levodopa that is required to control symptoms. One notable side effect with prolonged use is pulmonary fibrosis. Examples are: Bromocryptine Pergolide Carbergoline Monoamine Oxidase-B Inhibitors Monoamine oxidase enzymes break down neurotransmitters such as dopamine, serotonin and adrenaline. The monoamine oxidase-B enzyme is more specific to dopamine and does not act on serotonin or adrenalin. These medications block this enzyme and therefore help increase the circulating dopamine. Similarly to dopamine agonists, they are usually used to delay the use of levodopa and then in combination with levodopa to reduce the required dose. Examples are: Selegiline Rasagiline

Answer 25

Benign essential tremor is a relatively common condition associated with older age. It is characterised by a fine tremor affecting all the voluntary muscles. It is most notable in the hands but affects many other areas, for example causing a head tremor, jaw tremor and vocal tremor.

Answer 26

Fine tremor Symmetrical More prominent on voluntary movement Worse when tired, stressed or after caffeine Improved by alcohol Absent during sleep

Answer 27

Benign essential tremor is diagnosed clinically based on the presenting features. It is important to look for features to exclude other causes of a tremor. The key differential diagnoses of a tremor are: Parkinson’s disease Multiple sclerosis Huntington’s Chorea Hyperthyroidism Fever Medications (e.g. antipsychotics)

Answer 28

There is no definitive treatment for benign essential tremor. The tremor is not harmful and does not require treatment if not causing functional or psychological problems. Medications that can be tried to improve symptoms are: Propranolol (a non-selective beta blocker) Primidone (a barbiturate anti-epileptic medication)

Answer 29

Epilepsy is an umbrella term for a condition where there is a tendency to have seizures. Seizures are transient episodes of abnormal electrical activity in the brain. There are many different types of seizures. A diagnosis of epilepsy is made by a specialist based on the characteristics of the seizure episodes.

Answer 30

An electroencephalogram (EEG) can show typical patterns in different forms of epilepsy and support the diagnosis. An MRI brain can be used to visualise the structure of the brain. It is used to diagnose structural problems that may be associated with seizures and other pathology such as tumours. Other investigations can be used to exclude other pathology, particularly an ECG to exclude problems in the heart.

Answer 31

There are many types of seizures. There are different first line treatments for epilepsy with different types of seizures. The aim of treatment is to be seizure free on the minimum anti-epileptic medications. Ideally they should be on monotherapy with a single anti-epileptic drug. Treatment is initiated and guided by a specialist.

Answer 32

These are what most people think of with an epileptic seizure. There is loss of consciousness and tonic (muscle tensing) and clonic (muscle jerking) episodes. Typically the tonic phase comes before the clonic phase. There may be associated tongue biting, incontinence, groaning and irregular breathing. After the seizure there is a prolonged post-ictal period where the person is confused, drowsy and feels irritable or depressed. Management of tonic-clonic seizures is with: First line: sodium valproate Second line: lamotrigine or carbamazepine

Answer 33

Focal seizures start in temporal lobes. They affect hearing, speech, memory and emotions. There are various ways that focal seizures can present: Hallucinations Memory flashbacks Déjà vu Doing strange things on autopilot One way to remember the treatment is that they are the reverse of tonic-clonic seizures: First line: carbamazepine or lamotrigine Second line: sodium valproate or levetiracetam

Answer 34

Absence seizures typically happen in children. The patient becomes blank, stares into space and then abruptly returns to normal. During the episode they are unaware of their surroundings and won’t respond. These typically only lasts 10-20 seconds. Most patients (> 90%) stop having absence seizures as they get older. Management is: First line: sodium valproate or ethosuximide

Answer 35

Atonic seizures are also known as “drop attacks”. They are characterised by brief lapses in muscle tone. These don’t usually last more than 3 minutes. They typically begin in childhood. They may be indicative of Lennox-Gastaut syndrome. Management is: First line: sodium valproate Second line: lamotrigine

Answer 36

Myoclonic seizures present as sudden brief muscle contractions, like a sudden “jump”. The patient usually remains awake during the episode. They occur in various forms of epilepsy but typically happen in children as part of juvenile myoclonic epilepsy. Management is: First line: sodium valproate Other options: lamotrigine, levetiracetam or topiramate

Answer 37

Infantile spasms This is also known as West syndrome. It is a rare (1 in 4000) disorder starting in infancy at around 6 months of age. It is characterised by clusters of full body spasms. There is a poor prognosis: 1/3 die by age 25, however 1/3 are seizure free. It can be difficult to treat but first line treatments are: Prednisolone Vigabatrin

Answer 38

This is a first line option for most forms of epilepsy (except focal seizures). It works by increasing the activity of GABA, which has a relaxing effect on the brain. Notable side effects: Teratogenic so patients need careful advice about contraception Liver damage and hepatitis Hair loss Tremor There are a lot of warning about the teratogenic effects of sodium valproate and NICE updated their guidelines in 2018 to reflect this. It must be avoided in girls or women unless there are no suitable alternatives and strict criteria are met to ensure they do not get pregnant.

Answer 39

This is first line for focal seizures. Notable side effects are: Agranulocytosis Aplastic anaemia Induces the P450 system so there are many drug interactions

Answer 40

Notable side effects: Folate and vitamin D deficiency Megaloblastic anaemia (folate deficiency) Osteomalacia (vitamin D deficiency)

Answer 41

Notable side effects: Night terrors Rashes

Answer 42

Notable side effects: Stevens-Johnson syndrome or DRESS syndrome. These are life threatening skin rashes. Leukopenia

Answer 43

Status epilepticus is an important condition you need to be aware of and how to treat. It is a medical emergency. It is defined as seizures lasting more than 5 minutes or more than 3 seizures in one hour.

Answer 44

Take an ABCDE approach: Secure the airway Give high-concentration oxygen Assess cardiac and respiratory function Check blood glucose levels Gain intravenous access (insert a cannula) IV lorazepam 4mg, repeated after 10 minutes if the seizure continues If seizures persist: IV phenobarbital or phenytoin

Answer 45

Buccal midazolam Rectal diazepam

Answer 46

Neuropathic pain is caused by abnormal functioning of the sensory nerves delivering abnormal and painful signals to the brain.

Answer 47

Neuropathic pain can affect a wide variety of areas with number of different causes: Postherpetic neuralgia from shingles is in the distribution of a dermatome and usually on the trunk Nerve damage from surgery Multiple sclerosis Diabetic neuralgia typically affects the feet Trigeminal neuralgia Complex Regional Pain Syndrome (CRPS)

Answer 48

Burning Tingling Pins and needles Electric shocks Loss of sensation to touch of the affected area

Answer 49

This is used to assess the characteristics of the pain and examination of the affected area. They are then scored out of 10 for their pain. A score of 4 or more indicates neuropathic pain.

Answer 50

There are four first line treatments for neuropathic pain: Amitriptyline is a tricyclic antidepressant Duloxetine is an SNRI antidepressant Gabapentin is an anticonvulsant Pregabalin is an anticonvulsant NICE recommend using one of these four medications to control neuropathic pain. If that does not work then stop and start an alternative and repeat this until all four have been tried. Other options Tramadol ONLY as a rescue for short term control of flares Capsaicin cream (chilli pepper cream) for localised areas of pain Physiotherapy to maintain strength Psychological input to help with understanding and coping Trigeminal neuralgia is a type of neuropathic pain however NICE recommend carbamazepine as first-line for trigeminal neuralgia and if that does not work to refer to a specialist.

Answer 51

This is a condition where areas are affected by abnormal nerve functioning causing neuropathic pain and abnormal sensations. It is usually isolated to one limb. Often it is triggered by an injury to the area. The area can become very painful and hypersensitive even to simple inputs such as wearing clothing. It can also intermittently swell, change colour, change temperature, flush with blood and have abnormal sweating. Treatment is often guided by a pain specialist and is similar to other neuropathic pain.

Answer 52

Facial nerve palsy refer to isolated dysfunction of the facial nerve. This typically presents with a unilateral facial weakness. It is important to understand some basics about the pathway of the facial nerve and the function of the facial nerve to consider the causes and management.

Answer 53

The facial nerve exits the brainstem at the cerebellopontine angle. On its journey to the face it passes through the temporal bone and parotid gland. It then divides into five branches that supply different areas of the face: Temporal Zygomatic Buccal Marginal mandibular Cervical

Answer 54

There are three functions of the facial nerve: motor, sensory and parasympathetic. Motor: Supplies the muscles of facial expression, the stapedius in the inner ear and the posterior digastric, stylohyoid and platysma muscles in the neck. Sensory: carries taste from the anterior 2/3 of the tongue. Parasympathetic: it provides the parasympathetic supply to the submandibular and sublingual salivary glands and the lacrimal gland (stimulating tear production).

Answer 55

A very common exam question is to distinguish between an upper motor neurone and lower motor neurone facial nerve palsy. It is essential to be able to make this distinction, because in a patient with a new onset upper motor neurone facial nerve palsy you should be referring urgently with a suspected stroke, whereas patients with lower motor neurone facial nerve palsy can be reassured and managed in the community. Each side of the forehead has upper motor neurone innervation by both sides of the brain. Each side of the forehead only has lower motor neurone innervation from one side of the brain. In an upper motor neurone lesion, the forehead will be spared and the patient can move their forehead on the affected side. In a lower motor neurone lesion, the forehead will NOT be spared and the patient cannot move their forehead on the affected side.

Answer 56

Unilateral upper motor lesions occur in: Cerebrovascular accidents (strokes) Tumours Bilateral upper motor neurone lesions are rare. They may occur in: Pseudobulbar palsies Motor neurone disease

Answer 57

Bell’s palsy is a relatively common condition. It is idiopathic, meaning there is no clear cause. It presents as a unilateral lower motor neurone facial nerve palsy. The majority of patients fully recover over several weeks but recovery may take up to 12 months. A third are left with some residual weakness. If patients present within 72 hours of developing symptoms, NICE guidelines recommend considering prednisolone as treatment, either: 50mg for 10 days 60mg for 5 days followed by a 5-day reducing regime of 10mg a day The NICE Clinical Knowledge Summaries do not recommend using antivirals but say an antiviral plus steroids may offer a “small benefit” (this should be discussed with a specialist). Patients also require lubricating eye drops to prevent the eye on the affected drying out and being damaged. If they develop pain in the eye they need an ophthalmology review for exposure keratopathy. Tape can be used to keep the eye closed at night.

Answer 58

Ramsay-Hunt syndrome is caused by the varicella zoster virus (VZV). It presents as a unilateral lower motor neurone facial nerve palsy. Patients stereotypically have a painful and tender vesicular rash in the ear canal, pinna and around the ear on the affected side. This rash can extend to the anterior 2/3 of the tongue and hard palate. Treatment should ideally be initiated within 72 hours. Treatment is with: Prednisolone Aciclovir Patients also require lubricating eye drops. TOM TIP: Ramsay-Hunt syndrome is a very popular presentation in your MCQ exams. Look out for that patient with a vesicular rash around their ear and a facial nerve palsy.

Answer 59

Infection: Otitis media Malignant otitis externa HIV Lyme’s disease Systemic disease: Diabetes Sarcoidosis Leukaemia Multiple sclerosis Guillain–Barré syndrome Tumours: Acoustic neuroma Parotid tumours Cholesteatomas Trauma: Direct nerve trauma Damage during surgery Base of skull fractures

Answer 60

Brain tumours are abnormal growths within the brain. There are many different types of brain tumour. They vary from benign tumours (e.g. meningiomas) to highly malignant (e.g. glioblastomas).

Answer 61

Often brain tumours do not have any symptoms, particularly when they are small. As they develop they present with focal neurological symptoms depending on the location of the lesion. Brain tumours often present with symptoms and signs of raised intracranial pressure. As a tumour grows within the skull it takes up space. This leaves less space for the other contents of the skull (such as the CSF) to squeeze in to and leads to a rise in the pressure within the intracranial space. TOM TIP: A common exam question asks the location of the lesion based on the neurology. A popular exam question describes a patient that has had an unusual change in personality and behaviour. This indicates a tumour in the frontal lobe. Remember that the frontal lobe is responsible for personality and higher-level decision making.

Answer 62

Anything that takes up additional space within the skull will increase the pressure in the intracranial space. Raised intracranial pressure causes symptoms that can lead to a diagnosis of a brain tumour. Papilloedema is a key finding on fundoscopy in patients with raised intracranial pressure. This is a key component to examination in patients with headaches or other concerning features.

Answer 63

Brain tumours Intracranial haemorrhage Idiopathic intracranial hypertension Abscesses or infection

Answer 64

Concerning features of a headache that should prompt further examination and investigation include: Constant Nocturnal Worse on waking Worse on coughing, straining or bending forward Vomiting Other presenting features of raised intracranial pressure may be: Altered mental state Visual field defects Seizures (particularly focal) Unilateral ptosis Third and sixth nerve palsies Papilloedema (on fundoscopy)

Answer 65

Papilloedema is a swelling of the optic disc secondary to raised intracranial pressure. Papill- refers to a small rounded raised area (the optic disc) and -oedema refers to the swelling. The sheath around the optic nerve is connected with the subarachnoid space. Therefore it is possible for CSF under high pressure to flow into the optic nerve sheath. This increases the pressure around the optic nerve where it connects with the back of the eye at the optic disc, causing optic disc swelling. This can be seen on fundoscopy examination. Fundoscopic Changes Blurring of the optic disc margin Elevated optic disc (look for the way the retinal vessels flow across the disc to see the elevation) Loss of venous pulsation Engorged retinal veins Haemorrhages around optic disc Paton’s lines which are creases in the retina around the optic disc TOM TIP: It can be tricky to learn to recognise papilloedema. When looking for elevation of the optic disc, look at the way the retinal vessels flow across the disc. Vessels are able to flow straight across a flat surface, whereas they will curve over a raised disc.

Answer 66

The common cancers that metastasise to the brain are: Lung Breast Renal cell carcinoma Melanoma

Answer 67

Gliomas are tumours of the glial cells in the brain or spinal cord. There are three types to remember (listed from most to least malignant): Astrocytoma (glioblastoma multiforme is the most common) Oligodendroglioma Ependymoma Gliomas are graded from 1-4. Grade 1 are most benign (possibly curable with surgery). Grade 4 are the most malignant (glioblastomas).

Answer 68

Meningiomas are tumours growing from the cells of the meninges in the brain and spinal cord. They are usually benign, however they take up space and this mass effect can lead to raised intracranial pressure and neurological symptoms.

Answer 69

Pituitary tumours tend to be benign. If they grow large enough they can press on the optic chiasm causing a specific visual field defect called a bitemporal hemianopia. This causes loss of the outer half of the visual fields in both eyes. They have the potential to cause hormone deficiencies (hypopituitarism) or to release excessive hormones leading to: Acromegaly Hyperprolactinaemia Cushing’s disease Thyrotoxicosis

Answer 70

Acoustic neuromas are tumours of the Schwann cells surrounding the auditory nerve that innervates the inner ear. They occur around the “cerebellopontine angle” and are sometimes referred to as cerebellopontine angle tumours. They are slow-growing but eventually grow large enough to produce symptoms and become dangerous. Acoustic neuromas are usually unilateral. Bilateral acoustic neuromas are associated with neurofibromatosis type 2. Classic symptoms of an acoustic neuroma are: Hearing loss Tinnitus Balance problems They can also be associated with a facial nerve palsy.

Answer 71

There is massive variation in brain tumours from completely benign to extremely malignant. Surgery is dependent on the grade and behaviour of the brain tumour. Management options include: Palliative care Chemotherapy Radiotherapy Surgery

Answer 72

Trans-sphenoidal surgery Radiotherapy Bromocriptine to block prolactin-secreting tumours Somatostatin analogues (e.g. ocreotide) to block growth hormone-secreting tumours

Answer 73

Huntington’s chorea is an autosomal dominant genetic condition that causes a progressive deterioration in the nervous system. Patients are usually asymptomatic until symptoms begin around aged 30 to 50. Huntington’s chorea is a “trinucleotide repeat disorder” that involves a genetic mutation in the HTT gene on chromosome 4.

Answer 74

Huntington’s chorea displays something called genetic “anticipation”. Anticipation is a feature of trinucleotide repeat disorders. This is where successive generations have more repeats in the gene, resulting in: Earlier age of onset Increased severity of disease TOM TIP: Anticipation is a common topic of exam questions. It is worth remembering the features and connection with Huntington’s for your exams.

Answer 75

Huntington’s chorea usually presents with an insidious, progressive worsening of symptoms. It typically begins with cognitive, psychiatric or mood problems. These are followed by the development of movement disorders. Chorea (involuntary, abnormal movements) Eye movement disorders Speech difficulties (dysarthria) Swallowing difficulties (dysphagia)

Answer 76

Diagnosis is made in a specialist genetic centre using a genetic test for the faulty gene. It involves pre-test and post-test counselling regarding the implications of the results.

Answer 77

There are currently no treatment options for slowing or stopping the progression of the disease. The key to management of the condition is supporting the person and their family. Effectively breaking bad news Involvement of MDT in supporting and maintaining their quality of life (e.g. occupational therapy, physiotherapy and psychology) Speech and language therapy where there are speech and swallowing difficulties Genetic counselling regarding relatives, pregnancy and children Advanced directives to document the patients wishes as the disease progresses End of life care planning Medical treatment is based on symptomatic relief. As the disease progresses medication requirements may change. It is important to discontinue unnecessary medication to minimise adverse effects. Medications that can suppress the disordered movement: Antipsychotics (e.g. olanzapine) Benzodiazepines (e.g. diazepam) Dopamine-depleting agents (e.g. tetrabenazine) Depression can be treated with antidepressants.

Answer 78

Huntington’s chorea is a progressive condition. Life expectance is around 15-20 years after the onset of symptoms. As the disease progresses patients become more susceptible and less able to fight off illnesses. Death is often due to respiratory disease (e.g. pneumonia). Suicide is a more common cause of death than in the general population.

Answer 79

Myasthenia gravis is an autoimmune condition that causes muscle weakness that gets progressively worse with activity and improves with rest. Interestingly myasthenia gravis affects men and women at different ages. Typical patients are either a woman under the age of 40 or a man over the age of 60. There is a strong link between thymoma (tumours of the thymus gland) and myasthenia gravis. 10-20% of patients with myasthenia gravis have a thymoma. 20-40% of patients with a thymoma develop myasthenia gravis.

Answer 80

Motor nerves communicate with muscles at neuromuscular junctions. At the 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 at these junctions is called acetylcholine. This acetylcholine travels across the synapse and attached to receptors on the post-synaptic membrane. They stimulate the receptors, and this signal leads to muscle contraction. In around 85% of patients with myasthenia gravis, acetylcholine receptor antibodies are produced by the immune system. These bind to the postsynaptic neuromuscular junction receptors. This blocks the receptor and prevents the acetylcholine from being able to stimulate the receptor and trigger muscle contraction. As the receptors are used more during muscle activity, more of them become blocked up. This leads to less effective stimulation of the muscle with increased activity. There is more muscle weakness the more the muscles are used. This improves with rest as more receptors are freed up for use again. These antibodies also activate the complement system within the neuromuscular junction, leading to damage to cells at the postsynaptic membrane. This further worsens the symptoms. There are two other antibodies that cause the other 15% of cases of myasthenia gravis. These are antibodies against muscle-specific kinase (MuSK) and antibodies against low-density lipoprotein receptor-related protein 4 (LRP4). MuSK and LRP4 are important proteins for the creation and organisation of the acetylcholine receptor. Destruction of these proteins by autoantibodies leads to inadequate acetylcholine receptors. This causes the symptoms of myasthenia gravis.

Answer 81

The severity of symptoms can vary dramatically between patients. They can be mild and subtle or life threateningly severe. The characteristic feature is weakness that gets worse with muscle use and improves with rest. Symptoms are typically minimal in the morning and worst at the end of the day. The symptoms most affect the proximal muscles and small muscles of the head and neck. It leads to: Extraocular muscle weakness causing double vision (diplopia) Eyelid weakness causing drooping of the eyelids (ptosis) Weakness in facial movements Difficulty with swallowing Fatigue in the jaw when chewing Slurred speech Progressive weakness with repetitive movements

Answer 82

There are a few ways to elicit fatiguability in the muscles: Repeated blinking will exacerbate ptosis Prolonged upward gazing will exacerbate diplopia on further eye movement testing Repeated abduction of one arm 20 times will result in unilateral weakness when comparing both sides Check for a thymectomy scar. Test the forced vital capacity (FVC).

Answer 83

Diagnosis can be made testing directly for the relevant antibodies: Acetylcholine receptor (ACh-R) antibodies (85% of patients) Muscle-specific kinase (MuSK) antibodies (10% of patients) LRP4 (low-density lipoprotein receptor-related protein 4) 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.

Answer 84

Patients are given an IV dose of edrophonium chloride (or neostigmine). Normally, cholinesterase enzymes in the neuromuscular junction break down acetylcholine. Edrophonium block these enzymes and stop the breakdown of acetylcholine. As a result the level of acetylcholine at the neuromuscular junction increases. It briefly and temporarily relieves the weakness. This establishes a diagnosis of myasthenia gravis.

Answer 85

Reversible acetylcholinesterase inhibitors (usually pyridostigmine or neostigmine) increases the amount of acetylcholine in the neuromuscular junction and improve symptoms Immunosuppression (e.g. prednisolone or azathioprine) suppresses the production of antibodies Thymectomy can improve symptoms even in patients without a thymoma Monoclonal antibodies Rituximab is a monoclonal antibody that targets B cells and reduces the production of antibodies. It is available on the NHS if standard treatment is not effective and certain criteria are met. Eculizumab is a monoclonal antibody that targets complement protein C5. This could potentially prevent the complement activation and destruction of acetylcholine receptors. There is ongoing research and debate about whether the evidence is strong enough to offer it on the NHS. It is currently not recommended by NICE.

Answer 86

Myasthenic crisis is a severe complication of myasthenia gravis. It can be life threatening. It causes an acute worsening of symptoms, often triggered by another illness such as a respiratory tract infection. This can lead to respiratory failure as a result of weakness in the muscle of respiration. Patients may require non-invasive ventilation with BiPAP or full intubation and ventilation. Medical treatment of myasthenic crisis is with immunomodulatory therapies such as IV immunoglobulins and plasma exchange.

Answer 87

Lambert-Eaton myasthenic syndrome has a similar set of features to myasthenia gravis. It causes progressive muscle weakness with increased use as a result of damage to the neuromuscular junction. The symptoms tend to be more insidious and less pronounced than in myasthenia gravis. Lambert-Eaton syndrome typically occurs in patients with small-cell lung cancer. It is a result of antibodies produced by the immune system against voltage-gated calcium channels in small cell lung cancer (SCLC) cells. These antibodies also target and damage voltage-gated calcium channels in the presynaptic terminals of the neuromuscular junction where motor nerves communicate with muscle cells. These voltage-gated calcium channels are responsible for assisting in the release of acetylcholine into the synapse of the neuromuscular junction. This acetylcholine then binds to the acetylcholine receptors and stimulates a muscle contraction. When these channels are destroyed, less acetylcholine is release into the synapse.

Answer 88

The symptoms of Lambert-Eaton syndrome tend to develop slowly. The proximal muscles are most notably affected, causing proximal muscle weakness. It most notably presents with proximal leg muscle weakness. It can also affect the intraocular muscles causing double vision (diplopia), the levator muscles in the eyelid causing eyelid drooping (ptosis) and the oropharyngeal muscles causing slurred speech and swallowing problems (dysphagia). Patients may also experience dry mouth, blurred vision, impotence and dizziness due to autonomic dysfunction. Patients with Lambert-Eaton have reduced tendon reflexes. A notable finding is that these reflexes become temporarily normal for a short period following a period of strong muscle contraction. For example, the patient can maximally contract the quadriceps muscle for a period, then have their reflexes tested immediately afterwards, and display an improvement in the response. This is called post-tetanic potentiation.

Answer 89

It is important to diagnose and manage any underlying malignancy. In older smokers with symptoms of Lambert-Eaton syndrome consider investigating for small cell lung cancer. Amifampridine allows more acetylcholine to be released in the neuromuscular junction synapses. It works by blocking voltage-gated potassium channels in the presynaptic cells, which in turn prolongs the depolarisation of the cell membrane and assists calcium channels in carrying out their action. This improves symptoms in Lambert-Eaton syndrome. Other options: Immunosuppressants (e.g. prednisolone or azathioprine) IV immunoglobulins Plasmapheresis

Answer 90

Charcot-Marie-Tooth disease is an inherited disease that affects the peripheral motor and sensory nerves. There are various types of Charcot-Marie-Tooth with different genetic mutations and different pathophysiology. They cause dysfunction in the myelin or the axons. The majority of mutations are inherited in an autosomal dominant pattern. Symptoms usually start to appear before the age of 10 years but the onset of symptoms can be delayed until 40 or later.

Answer 91

There are some classical features of Charcot-Marie-Tooth to look out for when examining patient. Not all of these features will apply to all patients with the condition but they are a helpful set of features to look out for, particularly in your OSCEs: High foot arches (pes cavus) Distal muscle wasting causing “inverted champagne bottle legs” Weakness in the lower legs, particularly loss of ankle dorsiflexion Weakness in the hands Reduced tendon reflexes Reduced muscle tone Peripheral sensory loss

Answer 92

A – Alcohol B – B12 deficiency C – Cancer and Chronic Kidney Disease D – Diabetes and Drugs (e.g. isoniazid, amiodarone and cisplatin) E – Every vasculitis TOM TIP: This is a common OSCE scenario. You will have to perform a neurological examination on a patient that has a peripheral neuropathy. Charcot-Marie-Tooth is a relatively common (1 in 2,500 people) condition with good signs that has a high chance of appearing in your exams. Look for the other features, suggest the diagnosis, then run through the ABCDE mnemonic to suggest the possible other causes.

Answer 93

There is no treatment to alter the underlying disease or prevent it progressing. Management is purely supportive with input from various members of the multidisciplinary team: Neurologists and geneticists to make the diagnosis Physiotherapists to maintain muscle strength and joint range of motion Occupational therapists to assist with activities of living Podiatrists to help with foot symptoms and suggest insoles and other orthoses to improve symptoms Orthopaedic surgeons to correct disabling joint deformities

Answer 94

Guillain-Barré syndrome is an “acute paralytic polyneuropathy” that affects the peripheral nervous system. It causes acute, symmetrical, ascending weakness and can also cause sensory symptoms. It is usually triggered by an infection and is particularly associated with to campylobacter jejuni, cytomegalovirus and Epstein-Barr virus.

Answer 95

Guillain-Barré is thought to occur due to a process called molecular mimicry. The B cells of the immune system create antibodies against the antigens on the pathogen that causes the preceding infection. These antibodies also match proteins on the nerve cells. They may target proteins on the myelin sheath of the motor nerve cell or the nerve axon.

Answer 96

Symmetrical ascending weakness (starting at the feet and moving up the body) Reduced reflexes There may be peripheral loss of sensation or neuropathic pain It may progress to the cranial nerves and cause facial nerve weakness

Answer 97

Symptoms usually start within 4 weeks of the preceding infection. The symptoms typically start in the feet and progresses upward. Symptoms peak within 2-4 weeks, then there is a recovery period that can last months to years.

Answer 98

A diagnosis of Guillain-Barré syndrome is made clinically. The Brighton criteria can be used for diagnosis. Diagnosis can be supported by investigations: Nerve conduction studies (reduced signal through the nerves) Lumbar puncture for CSF (raised protein with a normal cell count and glucose)

Answer 99

IV immunoglobulins Plasma exchange (alternative to IV IG) Supportive care VTE prophylaxis (pulmonary embolism is a leading cause of death) In severe cases with respiratory failure patients may need intubation, ventilation and admission to the intensive care unit.

Answer 100

80% will fully recover 15% will be left with some neurological disability 5% will die

Answer 101

Neurofibromatosis is a genetic condition that causes nerve tumours (neuromas) to develop throughout the nervous system. These tumours are benign, however they do cause neurological and structural problems. There are two types of neurofibromatosis with different features. Neurofibromatosis type 1 is more common than type 2. The majority of this section focuses on type 1.

Answer 102

The neurofibromatosis type 1 gene is found on chromosome 17. It codes for a protein called neurofibromin, which is a tumour suppressor protein. Inheritance of mutations in this gene is autosomal dominant.

Answer 103

There are clear diagnostic criteria for NF1 based on the classical features of the condition. There must be at least 2 of the 7 features to indicate a diagnosis. You can remember this with the mnemonic CRABBING. C – Café-au-lait spots (6 or more) measuring ≥ 5mm in children or ≥ 15mm in adults R – Relative with NF1 A – Axillary or inguinal freckles BB – Bony dysplasia such as Bowing of a long bone or sphenoid wing dysplasia I – Iris hamartomas (Lisch nodules) (2 or more) are yellow brown spots on the iris N – Neurofibromas (2 or more) or 1 plexiform neurofibroma G – Glioma of the optic nerve

Answer 104

Diagnosis is based on clinical criteria and no investigations are required to make a definitive diagnosis. Genetic testing can be used where there is doubt. Xrays can be used to investigate bone pain and bone lesions. Imaging with CT and MRI scans can be used to investigate lesions in the brain, spinal cord and elsewhere in the body.

Answer 105

There is no treatment of the underlying disease process or to prevent the development of neurofibromas or complications. Management is to control symptoms, monitor the disease and treat complications.

Answer 106

Migraines Epilepsy Renal artery stenosis causing hypertension Learning and behavioural problems (e.g. ADHD) Scoliosis of the spine Vision loss (secondary to optic nerve gliomas) Malignant peripheral nerve sheath tumours Gastrointestinal stromal tumour (a type of sarcoma) Brain tumours Spinal cord tumours with associated neurology (e.g. paraplegia) Increased risk of cancer (e.g. breast cancer) Leukaemia

Answer 107

The neurofibromatosis type 2 gene is found on chromosome 22. It codes for a protein called merlin, which is a tumour suppressor protein particularly important in Schwann cells. Mutations in this gene lead to the development of schwannomas (benign nerve sheath tumours of the Schwann cells). Inheritance is autosomal dominant. Neurofibromatosis type 2 is most associated with acoustic neuromas. These are tumours of the auditory nerve innervating the inner ear. Symptoms of an acoustic neuroma are: Hearing loss Tinnitus Balance problems Schwannomas can also develop in the brain and spinal cord with symptoms based on the location of the lesion. Surgery can be used to resect tumours although there is a risk or permanent nerve damage. TOM TIP: Bilateral acoustic neuromas almost certainly indicate neurofibromatosis type 2. This is a popular association in exams so worth remembering.

Answer 108

Tuberous sclerosis is a genetic condition that causes features in multiple systems. The characteristic feature is the development of hamartomas. These are benign neoplastic growths of the tissue that they origin from. Hamartomas cause problems based on the location of the lesion. They commonly affect the: Skin Brain Lungs Heart Kidneys Eyes Tuberous sclerosis is caused by mutations in one of two genes: TSC1 gene on chromosome 9, which codes for hamartin TSC2 gene on chromosome 16, which codes for tuberin Hamartin and tuberin interact with each other to control the size and growth of cells. Abnormalities in one of these proteins leads to abnormal cell size and growth.

Answer 109

Ash leaf spots are depigmented areas of skin shaped like an ash leaf Shagreen patches are thickened, dimpled, pigmented patches of skin Angiofibromas are small skin coloured or pigmented papules that occur over the nose and cheeks Subungual fibromata are fibromas growing from the nail bed. They are usually circular painless lumps that grow slowly and displace the nail Cafe-au-lait spots are light brown “coffee and milk” coloured flat pigmented lesions on the skin Poliosis is an isolated patch of white hair on the head, eyebrows, eyelashes or beard

Answer 110

Epilepsy Learning disability and developmental delay

Answer 111

Rhabdomyomas in the heart Gliomas (tumours of the brain and spinal cord) Polycystic kidneys Lymphangioleiomyomatosis (abnormal growth in smooth muscle cells, often affecting the lungs) Retinal hamartomas

Answer 112

The classical presentation is a child presenting with epilepsy found to have skin features of tuberous sclerosis. It can also present in adulthood.

Answer 113

Management is supportive with monitoring and treating complications such as epilepsy. There is no treatment for the underlying gene defect.

Answer 114

Tension headaches Migraines Cluster headaches Secondary headaches Sinusitis Giant cell arteritis Glaucoma Intracranial haemorrhage Subarachnoid haemorrhage Analgesic headache Hormonal headache Cervical spondylosis Trigeminal neuralgia Raised intracranial pressure (brain tumours) Meningitis Encephalitis

Answer 115

It is important to consider red flags for serious conditions (such as raised intracranial pressure and intracranial haemorrhage) when taking a history and managing a patient with a headache. The NICE Clinical Knowledge Summaries on headache have a good summary of how to assess a headache. This is not an exhaustive list but includes the key symptoms to look out for: Fever, photophobia or neck stiffness (meningitis or encephalitis) New neurological symptoms (haemorrhage, malignancy or stroke) Dizziness (stroke) Visual disturbance (temporal arteritis or glaucoma) Sudden onset occipital headache (subarachnoid haemorrhage) Worse on coughing or straining (raised intracranial pressure) Postural, worse on standing, lying or bending over (raised intracranial pressure) Severe enough to wake the patient from sleep Vomiting (raised intracranial pressure or carbon monoxide poisoning) History of trauma (intracranial haemorrhage) Pregnancy (pre-eclampsia) Fundoscopy examination to look for papilloedema is an important part of an assessment of a headache. Papilloedema indicates raised intracranial pressure, which may be due to a brain tumour, benign intracranial hypertension or an intracranial bleed. TOM TIP: Practice asking red flag questions so you can demonstrate in an exam that you are thinking about serious causes. This will score extra points and also help you document well when you start seeing patients.

Answer 116

Tension headaches are very common. Classically they produce a mild ache across the forehead and in a band-like pattern around the head. This may be due to muscle ache in the frontalis, temporalis and occipitalis muscles. Tension headaches comes on and resolve gradually and don’t produce visual changes. Associations Stress Depression Alcohol Skipping meals Dehydration Treatment Reassurance Basic analgesia Relaxation techniques Hot towels to local area

Answer 117

Secondary headaches give a similar presentation to a tension headache but with a clear cause. They produce a non-specific headache secondary to: Underlying medical conditions such as infection, obstructive sleep apnoea or pre-eclampsia Alcohol Head injury Carbon monoxide poisoning

Answer 118

Sinusitis causes a headache associated with inflammation in the ethmoidal, maxillary, frontal or sphenoidal sinuses. This usually produces facial pain behind the nose, forehead and eyes. There is often tenderness over the affected sinus, which helps to establish the diagnosis. Sinusitis usually resolves within 2-3 weeks. Most sinusitis is viral. Nasal irrigation with saline can be helpful. Prolonged symptoms can be treated with steroid nasal spray. Antibiotics are occasionally required.

Answer 119

An analgesic headache is a headache caused by long term analgesia use. It gives similar non-specific features to a tension headache. They are secondary to continuous or excessive use of analgesia. Withdrawal of analgesia important in treating the headache, although this can be challenging in patients with long term pain and those that believe the analgesia is necessary to treat the headache.

Answer 120

Hormonal headaches are related to oestrogen. They produce a generic, non-specific, tension-like headache. They tend to be related to low oestrogen: Two days before and first three days of the menstrual period Around the menopause Pregnancy. It is worse in the first few weeks and improves in the last 6 months. Headaches in the second half of pregnancy should prompt investigation for pre-eclampsia. The oral contraceptive pill can improve hormonal headaches.

Answer 121

Cervical spondylosis is a common condition caused by degenerative changes in the cervical spine. It causes neck pain, usually made worse by movement. However, if often presents with headache. It is important to exclude other causes of neck pain such as inflammation, malignancy and infection. It is also important to exclude spinal cord or nerve root lesions.

Answer 122

The trigeminal nerve is made up of three branches: Ophthalmic (V1) Maxillary (V2) Mandibular (V3) Trigeminal neuralgia can affect any combination of the branches. The cause is unclear but it is thought to be caused by compression of the nerve. 90% of cases are unilateral, 10% are bilateral. Around 5-10% of people with multiple sclerosis have trigeminal neuralgia. It presents with intense facial pain that comes on spontaneously and last anywhere between a few seconds to hours. It is often described as an electricity-like shooting pain. Attacks often worsen over time. There are a number of possible triggers for the pain in patients with trigeminal neuralgia. These include things like cold weather, spicy food, caffeine and citrus fruits. Treatment NICE recommend carbamazepine as first-line for trigeminal neuralgia. Surgery to decompress or intentionally damage the trigeminal nerve is an option.

Answer 123

Migraines are a complex neurological condition that cause headache and other associated symptoms. They occur in “attacks” that often follow a typical pattern. There are several types of migraine: Migraine without aura Migraine with aura Silent migraine (migraine with aura but without a headache) Hemiplegic migraine The pathophysiology of migraine has been studied for decades. Various mechanisms and theories have developed. There is no simple explanation for why migraines occur and it may be a combination of structural, functional, chemical, vascular and inflammatory factors.

Answer 124

Headaches last between 4 and 72 hours. Typical features are: Moderate to severe intensity Pounding or throbbing in nature Usually unilateral but can be bilateral Discomfort with lights (photophobia) Discomfort with loud noises (phonophobia) With or without aura Nausea and vomiting

Answer 125

Aura is the term used to describe the visual changes associated with migraines. There can be multiple different types of aura: Sparks in vision Blurring vision Lines across vision Loss of different visual fields

Answer 126

Hemiplegic migraines can mimic stroke. It is essential to act fast and exclude a stroke in patients presenting with symptoms of hemiplegic migraine. Symptoms of a hemiplegic migraine can vary significantly. They can include: Typical migraine symptoms Sudden or gradual onset Hemiplegia (unilateral weakness of the limbs) Ataxia Changes in consciousness

Answer 127

Migraines can be have specific triggers that are individual to the person. Often it is not possible to identify triggers. Potentially triggers are: Stress Bright lights Strong smells Certain foods (e.g. chocolate, cheese and caffeine) Dehydration Menstruation Abnormal sleep patterns Trauma

Answer 128

The course of a migraine can be broken down into 5 stages. These stages are not typical of everyone and they will vary between patients. Some patients may only experience one or two of the stages. The prodromal stage can involve several days of subtle symptoms such as yawning, fatigue or mood changes prior to the onset of the migraine. Premonitory or prodromal stage (can begin 3 days before the headache) Aura (lasting up to 60 minutes) Headache stage (lasts 4-72 hours) Resolution stage (the headache can fade away or be relieved completely by vomiting or sleeping) Postdromal or recovery phase

Answer 129

Patients often develop their own patterns for helping to relieve their symptoms. Often patients will go to a dark quiet room and sleep. Options for medical management are: Paracetamol Triptans (e.g. sumatriptan 50mg as the migraine starts) NSAIDs (e.g ibuprofen or naproxen) Antiemetics if vomiting occurs (e.g. metoclopramide)

Answer 130

Triptans are used to abort migraines when they start to develop. They are 5HT receptors agonists (serotonin receptor agonists). They have various mechanisms of action and it is not clear which mechanisms are responsible for their effects on migraines. They act on: Smooth muscle in arteries to cause vasoconstriction Peripheral pain receptors to inhibit activation of pain receptors Reduce neuronal activity in the central nervous system

Answer 131

Keeping a headache diary can be helpful in identifying the triggers. Avoiding triggers can reduce the frequency of the migraine. A headache diary is also useful in demonstrating the response to treatment. Certain medications can be used long term to reduce the frequency and severity of attacks: Propranolol Topiramate (this is teratogenic and can cause a cleft lip/palate so patients should not get pregnant) Amitriptyline Acupuncture is an option recommended by NICE recommend for the treatment of migraines. It is reported to be as effective as prophylactic medications. Supplementation with vitamin B2 (riboflavin) may reduce frequency and severity. In migraine specifically triggered around menstruation, prophylaxis with NSAIDs (e.g. mefanamic acid) or triptans (frovatriptan or zolmitriptan) can be used as a preventative measure. Migraines tend to get better over time and people often go into remission from their symptoms.

Answer 132

Cluster headaches cause severe and unbearable unilateral headaches, usually around the eye. They are called cluster headaches as they come in clusters of attacks and then disappear for a while. For example, a patient may suffer 3 – 4 attacks a day for weeks or months followed by a pain-free period lasting 1-2 years. Attacks last between 15 minutes and 3 hours. A typical patient with cluster headaches in your exams is a 30 – 50 year-old male smoker. Attacks can be triggered by things like alcohol, strong smells and exercise.

Answer 133

Cluster headaches are often described as one of the most severe and intolerable pains in the world. They are sometimes referred to as “suicide headaches” due to the severity of the pain. Symptoms are typically all unilateral: Red, swollen and watering eye Pupil constriction (miosis) Eyelid drooping (ptosis) Nasal discharge Facial sweating

Answer 134

Acute management: Triptans (e.g. sumatriptan 6mg injected subcutaneously) High flow 100% oxygen for 15-20 minutes (can be given at home) Prophylaxis options: Verapamil Lithium Prednisolone (a short course for 2-3 weeks to break the cycle during clusters)