Neurology Flashcards
Define Bell’s palsy?
Bell’s palsy is an idiopathic syndrome that causes damage to the facial nerve leading to a lower motor neuron facial palsy.
What causes Bell’s palsy?
The aetiology of Bell’s palsy remains unknown. Viral infections, particularly reactivation of herpes simplex virus type 1 (HSV-1), have been implicated as a possible cause.
Other viral pathogens, such as Epstein-Barr virus (EBV) and varicella-zoster virus (VZV), have also been suggested as potential triggers.
However, the exact mechanisms by which these viruses lead to facial nerve dysfunction are not fully understood.
Clinical features of Bell’s palsy?
Acute (but not sudden) onset of unilateral lower motor neuron facial weakness (classically affecting the forehead as this area has bilateral nervous supply from both sides of the brain)
Mild to moderate postauricular otalgia, which may precede the paralysis.
Hyperacusis
Nervus intermedius symptoms, such as altered taste and dry eyes/mouth.
Patients may subjectively describe “numbness” or “heaviness” without objective facial somatosensory disturbances.
What are the investigations for Bell’s palsy?
The diagnosis of Bell’s palsy is primarily clinical, based on the characteristic signs and symptoms.
However, in some cases, additional investigations may be considered to exclude other potential causes or to assess the severity of nerve dysfunction. These investigations may include:
Otoscopy to assess if any vesicular lesions are present in the external auditory meatus
Full blood count (FBC)
Erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP)
Viral serology (e.g. HSV-1, EBV, VZV)
Lyme serology (if suggestive symptoms or exposure)
Electromyography (EMG)
Imaging studies (e.g. CT or MRI Head)
What is the short term management for Bell’s palsy?
The management of Bell’s palsy includes:
Prompt administration of oral steroids: 50mg of oral prednisolone or prednisone once daily for 10 days, followed by a taper.
Supportive treatments:
Artificial tears and ocular lubricants to manage dry eyes.
Eye patch/tape to prevent corneal exposure and injury.
What may be considered in treatment for Bell’s palsy?
Although the pathophysiology of Bell’s palsy has not been definitively linked to active herpes virus infection, empirical treatment with aciclovir may be considered, particularly in cases where the clinical distinction between Bell’s palsy and Ramsay-Hunt syndrome is difficult.
What is the long term management of Bell’s palsy?
Pain management with analgesics or anticonvulsants may be necessary in cases of severe otalgia or neuropathic pain.
Physical therapy, including facial exercises and massage, may be recommended to maintain muscle tone and prevent contractures during the recovery phase.
Psychological support and counseling may be beneficial for patients experiencing emotional distress or body image concerns due to facial weakness.
What percentage of patients with Bell’s palsy have a complete recovery?
Complete recovery: Approximately 70-80% of patients with Bell’s palsy achieve complete recovery of facial function without residual deficits.
Most improvement occurs within the first 3 months, with the greatest recovery seen within the first 6 weeks. However, some patients may continue to show gradual improvement even beyond this timeframe.
What two factors can decrease the likelihood of recovery for Bell’s palsy?
Age and severity: Older age and more severe initial facial weakness are associated with a higher risk of incomplete recovery.
Define a brain abscess?
A brain abscess is a collection of pus within the brain parenchyma. It is a very serious condition which can lead to significant morbidity and mortality, especially if left untreated.
What causes brain abscesses?
Brain abscesses are usually result from direct extension of an infection. This may be sinusitis, dental or cranial procedures or injury. Sometimes, the bacteria may spread through the bloodstream, for example a septic embolus from endocarditis. The most commonly isolated bacteria is Streptococcus, though other pathogens such as oral bacteria, Staphylococci, gram-negatives, TB, fungi and parasites can also cause brain abscesses, particularly in susceptible people
What are the signs and symptoms of brain abscesses?
Signs of infection: fever, nausea & vomiting, meningism
Raised intracranial pressure: headache, third nerve palsy, papilloedema, seizures
Focal neurology depending on location of abscess
What are the investigations for brain abscesses?
Bloods - including FBC, CRP, clotting, VBG, cultures
Cross-sectional imaging:
MRI is most sensitive for brain abscess, and will show an enhancing lesion.
If stroke is suspected, an urgent CT head will be required as per local pathways.
Neurosurgical aspiration/excision & cultures
What is the management for brain abscesses?
It is important to remember that when a patient is acutely unwell, start with an A-E assessment and consider initiating the sepsis six if the patient is showing signs. Call for help early - the exact team will depend on local protocols & clinical context. If the the patient is displaying acute neurological symptoms, initiate the stroke pathway as per local guidelines.
Exact management will be guided by seniors in neurology/neurosurgery & microbiology, but may include:
Neurosurgical intervention: aspiration or excision
Medical: antibiotics - initially a 3rd-generation cephalosporin + metronidazole. This may differ based on culture results and immune status of the patient. Typically, antibiotics continue for several weeks.
Symptom management - for example anticonvulsants for seizures, dexamethasone for severely raised intracranial pressure
What are the complications of brain abscesses?
Sepsis
Raised intracranial pressure & herniation
Permanent neurological deficits
Death
What are risk factors for brain abscesses?
Risk factors for brain abscess include:
Sinusitis
Dental, head & neck procedures
Cardiac defects & endocarditis
Poor immune response including HIV, immunocompromise
What would a CT reveal in a brain abscess?
ring enhancing lesion
Define brain metastases?
Brain metastases are secondary brain tumours that occur following spread from a tumour away from the central nervous system.
What causes brain metastases?
Malignancies usually spread to the brain haematogeneously. Cells seed into the brain’s small blood vessels and are often protected from anti-cancer therapies by the blood-brain barrier.
What are the most common primary malignancies that metastasise to the brain?
Lung cancer
Breast cancer
Colorectal cancer
Melanomas
Renal cell carcinoma
Signs and symptoms of brain metastases:
Brain metastases may occur in people with a known cancer, or may be their first presentation of malignancy. Symptoms can be due to local interruption of the nerve pathways or due to raised intracranial pressure. Key clinical features include:
Headaches
Seizures
Altered mental status, including confusion
Cerebellar signs, including ataxia
Nausea and vomiting
Visual disturbance
Focal neurology
Patients may also have signs of the primary malignancy, for example breathlessness and haemoptysis for lung cancer.
What are the investigations for brain metastases?
In a patient presenting with acute neurological features, it is important to rule out acute intracranial events, for example stroke. Therefore, a head CT is often employed first-line.
According to NICE, MRI is the best and initial imaging modality for diagnosing brain metastases. Further investigations may be necessary to identify the primary malignancy, for example chest imaging and biopsy.
What is the management for brain metastases?
Management of brain metastases requires a multidisciplinary approach. There are many factors to consider including the nature of the metastases, the patient’s age, performance status and primary tumour.
Systemic anti-cancer therapy is advised for people who have brain metastases likely to respond effectively, for example, germ cell tumours or small-cell lung cancer
Symptomatic management involves corticosteroids (usually dexamethasone) to reduce oedema, anti-seizure medications and antiemetics. Specific treatment for raised intracranial pressure may be necessary as well. Therapeutic options include radiotherapy (targeted, and sometimes whole-brain), chemotherapy and surgical resection.
Following treatment, patients will often have serial imaging follow up to assess any ongoing symptoms/potential progression
What is chronic fatigue syndrome also referred to as?
Myalgic encephalomyelitis
Define chronic fatigue syndrome?
CFS is a chronic illness characterised by persistent or recurrent fatigue that is not relieved by rest and is associated with significant functional impairment. It is a chronic multisystemic condition, with common features being post-exertional malaise, cognitive difficulties, unrefreshing sleep, autonomic dysfunction and pain. Symptoms should not be explained by any other cause.
The diagnosis should be suspected if fatigue has been present for at least 6 weeks with associated functional impairment, and symptoms are not secondary to another illness
Risk factors for chronic fatigue syndrome?
Risk factors include:
Family history
Epstein-Barr infection in adolescence
Covid-19 infection
How is chronic fatigue syndrome classified?
CFS can be classified by symptom severity
This is a way of approximating the impact of symptoms on daily life
However these categories are not clear-cut as symptoms may fluctuate in intensity and some symptoms may be more severe than others
There are additional management considerations for patients with severe or very severe CFS (see Management section for details)
What are the symptoms of mild chronic fatigue syndrome?
Patients are self-caring
May need some support with domestic tasks
Mobility may be affected
Often still in education or work, may need reduced hours and days off
Leisure and social activities usually curtailed
What are the symptoms of moderate chronic fatigue syndrome?
Restricted in all activities of daily living (ADLs)
Reduced mobility
Usually have stopped education or work
Sleep is usually poor quality
Rest periods usually needed
What are the symptoms of severe chronic fatigue syndrome?
No or minimal self-care (e.g. washing fash)
Mobility affected e.g. may require a wheelchair, often unable to leave the house
May spend most of the time in bed
Severe cognitive difficulties
Often hypersensitive to light and sound
What are the symptoms of very severe chronic fatigue?
Patients are bedbound
Dependent for all self-care including personal hygiene and eating
May require nutritional support (e.g. PEG feeding)
What are the signs and symptoms of chronic fatigue syndrome?
Patients should have all of the following symptoms, which have persistent over at least 3 months
Disabling fatigue
Worse on activity
Not significantly relieved by rest
Not due to excessive physical or mental exertion
Post-exertional malaise
Disproportionate to the activity
Prolonged e.g. may last days/weeks/longer
May be delayed in onset by hours/days
Sleeping difficulties
Sleep may be disturbed and/or unrefreshing
Patients may feel exhausted and stiff on waking
May have altered sleep pattern or hypersomnia
Cognitive difficulties
May describe “brain fog”
Poor concentration
Difficulties speaking or word-finding
Slow responses
Short-term memory impairment
What are the other symptoms of chronic fatigue syndrome?
Pain
May have generalised myalgia
May have headaches or eye/abdominal/joint pain
No signs of arthritis (e.g. erythema or swelling)
Being touched may be painful
Sensory sensitivities
e.g. to light/sound/taste/touch smell
May have temperature hypersensitivity (e.g. sweating/chills/hot flushes/feeling cold)
May be intolerant to alcohol/foods/certain chemicals
Autonomic dysfunction including orthostatic intolerance
Dizziness
Palpitations
Fainting
Nausea
Neuromuscular symptoms
Twitching or myoclonic jerks may be seen
Flu-like symptoms
Sore throat
Tender lymph nodes
Patients should be examined to look for signs of differential diagnoses
A mental state examination should be done to assess mood and cognition
Postural orthostatic tachycardia syndrome (POTS) may be seen (when the heart rate rises significantly when standing from lying down)
Postural hypotension may also be present
What investigations should we do for chronic fatigue syndrome?
There are no investigations that can diagnose CFS and the diagnosis is clinical.
However, all patients presenting with persistent fatigue should have the following blood tests to screen for an underlying physical cause:
Full blood count looking for anaemia, polycythaemia, inflammatory markers and haematological malignancy
U&Es for renal impairment or electrolyte imbalance
Liver function tests for liver disease
Bone profile to check calcium and phosphate
Myeloma screen especially in older patients or those with hypercalcaemia
Thyroid function tests for hypo or hyperthyroidism
HbA1c for diabetes
Ferritin which may be raised in inflammation or low in iron deficiency
ESR which is raised in inflammation or infection as well as some malignancies
Creatine kinase for muscle damage e.g. muscular dystrophy
IgA tissue transglutaminase for coeliac disease, which may present without gastrointestinal symptoms
What might urinalysis show in chronic fatigue syndrome?
Urinalysis should also be done, which may show glycosuria in diabetes, haematuria in renal tract inflammation, infection or malignancy, or proteinuria in inflammation or chronic kidney disease
Name some other random investigations based on clinical presentation for chronic fatigue syndrome?
Vitamin D in patients at risk of deficiency
Vitamin B12 and folate e.g. in anaemic patients or those with a restricted diet
Early morning cortisol to screen for adrenal insufficiency
HIV test in at risk patients (or all patients living in a high-prevalence area)
Hepatitis serology in at risk patients (e.g. those from an endemic area)
Monospot test for glandular fever in patients under the age of 40
Chest X-ray and sputum samples for tuberculosis in patients at risk (e.g. travel to an area where TB is endemic)
ELISA for Lyme disease if there is a history of tick bite
CT or MRI head if there are focal neurological signs or symptoms to rule out an intracranial cause for fatigue
Describe the management for chronic fatigue syndrome:
Management of CFS should be person-centred and tailored to individual needs
All patients should be referred to a specialist multidisciplinary team for confirmation of diagnosis and development of a care and support plan
Patients with severe/very severe CFS may require home visits for assessment and support
Advice should include:
Education on CFS and its management
Signposting to national and local resources and support
How to manage daily activity and not to “push through” symptoms of fatigue
Resting as much as needed (which may involve alterations to their daily activities)
Incorporating relaxation techniques into rest periods
Managing flare-ups and relapses
Maintaining a balanced diet and fluid intake
Developing good sleep habits
How to access social care support and assessment e.g. for disability benefits, adaptations to the home such as a stairlift
For moderate/severe/very severe CFS, consider the need for mobility aids (e.g. wheelchairs)
Support patients in work or education and ensure reasonable adjustments are put in place
Energy management is an important component of CFS care
This is a self-management strategy supported by the multidisciplinary team
Patients are encouraged to recognise their personal energy limits
Activities and rest periods should be planned accordingly
All types of activities including social, cognitive and physical should be considered
Plans should be regularly reviewed
Physiotherapy and occupational therapy input may be helpful for support with mobility and physical activity
Exercise programmes should be supported by specialist services for patients who would like to incorporate this into their CFS management
Cognitive behavioural therapy should be discussed
Patients are supported to manage symptoms, improve functioning and relieve distress
It is structured and time-limited
It focuses on the specific challenges faced by the patient
Comorbid conditions such as depression should be identified and managed
Associated symptoms such as orthostatic intolerance may require specialist input - this is usually managed with lifestyle changes; medications such as beta-blockers may be used
Additional investigations and analgesia may be required for pain management
Dietetic assessment is required for patients with weight loss or gain, those at risk of malnutrition e.g. due to a restrictive diet, or those with severe/very severe CFS
Vitamin D supplements should be considered for housebound patients
What are the complications for chronic fatigue syndrome?
Negative impacts on work, education, relationships and quality of life
Depression and/or anxiety
Malnutrition due to restrictive diet, poor appetite, food intolerances or difficulty with chewing/swallowing
Falls due to weakness and deconditioning
Pressure ulcers due to immobility
Deep vein thrombosis due to immobility
Contractures due to prolonged immobility
Vitamin D deficiency if leaving the house if difficult
Loss of earnings - 50% of patients with CFS have to stop work due to their symptoms
What is the prognosis for chronic fatigue syndrome?
Negative impacts on work, education, relationships and quality of life
Depression and/or anxiety
Malnutrition due to restrictive diet, poor appetite, food intolerances or difficulty with chewing/swallowing
Falls due to weakness and deconditioning
Pressure ulcers due to immobility
Deep vein thrombosis due to immobility
Contractures due to prolonged immobility
Vitamin D deficiency if leaving the house if difficult
Loss of earnings - 50% of patients with CFS have to stop work due to their symptoms
Define encephalitis:
Encephalitis is a pathological condition characterised by inflammation of the brain parenchyma.
What causes encephalitis?
The aetiology of encephalitis is predominantly viral, with the most common pathogen being the herpes simplex virus type 1 (HSV-1). Other viral causes can include HSV-2, enterovirus, cytomegalovirus, Epstein-Barr virus, varicella-zoster virus, HIV, and flaviviruses, including the West Nile virus.
Additionally, bacterial pathogens like those that cause meningitis, Lyme disease and Mycoplasma can cause meningoencephalitis. Parasitic infections with malaria or toxoplasmosis should be considered, especially with recent travel or immunocompromise. Autoimmune encephalitis, such as NMDA-receptor-antibody-associated encephalitis, should also be considered in the differential diagnosis.
Signs and symptoms of encephalitis?
Clinical features of encephalitis primarily include altered mental status, more so than in meningitis.
Other suggestive features include:
Fever
Flu-like prodromal illness
Seizures
Focal neurological deficits
Headaches
Behavioural changes
Meningismus
What is meningismus?
clinical syndrome of headache, neck stiffness, and photophobia, often with nausea and vomiting
What are the investigations for encephalitis?
Encephalitis should be suspected in any patient presenting with sudden onset behavioural changes, new seizures, and unexplained acute headache accompanied by meningism.
The standard work-up for encephalitis parallels that for meningitis and typically includes:
A routine panel of blood tests including FBC, U+E, LFTs (as part of a septic screen)
Blood cultures and viral PCR
Cerebrospinal fluid (CSF) analysis with viral PCR
Consideration for malaria blood films in cases of exposure
Central Nervous System (CNS) imaging through CT and MRI can provide valuable insight. For instance, HSV typically affects the temporal lobes and bilateral multifocal haemorrhage is typical.
Other tests may include sputum cultures, throat swab, chest X-ray and EEG (particularly if presenting with seizures)
What is the management for encephalitis?
Management of suspected encephalitis is empirical and involves broad-spectrum antimicrobial cover with 2g IV ceftriaxone BD and 10 mg/kg aciclovir TDS. Once the causative organism is identified, targeted treatment may begin.
Supportive management of complications is also crucial and can include the termination of seizure activity using anticonvulsants.
Acute encephalitis is a notifiable disease and should be reported to the local health protection team.
Ampicillin can also be added if Listeria monocytogenes is suspected
Define epilepsy?
Epilepsy is a neurological disorder characterised by an enduring predisposition to generate epileptic seizures and the neurobiological, cognitive, psychological, and social consequences of this condition.
Seizures are transient occurrences of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.
What causes epilepsy?
- Primary epilepsy is idiopathic
- Secondary causes:
- Tumour
- Meningitis
- Vasculitis
- Alcohol withdrawal
- Haemorrhage
- Metabolic
What conditions are associated with epilepsy?
- Cerebral palsy
- Tuberous sclerosis
- Mitochondrial disease
What type of seizures are there?
Focal seizure
Generalised Seizure
Status epilepticus
What is a focal seizure?
- Seizure localised to specific cortical regions
- May be complex (consciousness affected) or non-complex (consciousness not affected)
What is a generalised seizure?
Affects the whole brain and consciousness is lost immediately
- Absence
- Tonic
- Atonic
- Tonic-clonic
- Myoclonic
What is Status epilepticus and how do we treat?
seizure lasting longer than 5 minutes or ≥2 seizures within a 5-minute period without the person returning to normal between them
IV lorazepam or PR diazepam
Features of a frontal lobe focal seizure?
- Motor convulsions
- May show post-ictal flaccid weakness (post-ictal meaning just after seizure ends)
- Jacksonian march (clonic movements starting in 1 extremity and moving proximally through body) → usually starts as twitching/tingling of area like little toe or finger
Features of a temporal lobe focal seizure?
Most common type of partial seizure
- Aura- involving?
- Weird smells
- Involuntary movements
- Deja vu
- Abdo pain
- Lip smacking/plucking/grabbing (automatisms)
- Post-ictal dysphasia
- Hallucinations
Features of an occipital lobe seizure?
Visual disturbances (flashers and floaters)
Features of a parietal lobe seizure?
Sensory issues (paraesthesia- tingling, numbness)
Features of a tonic-clonic seizure?
- Vague symptoms before attack e.g. irritability
- Tonic phase (generalised muscle spasm- goes stiff and falls to floor)
- Clonic phase (repetitive synchronous jerks- jerking limbs or loss of bladder control)
- Urinary incontinence
- Tongue biting
- Post-ictal phase- including? (3)
- Impaired consciousness
- Lethargy
- Confusion
Features and treatment for an absence seizure?
- Onset in childhood
- Loss of consciousness but maintained posture (don’t fall down)
- No post-ictal phase
- Often begins abruptly without warning and ends abruptly
- Patient has no recollection of episode
- Stares blankly into space
3 Hz spike and wave
Treat with Ethosuximide
Features of myoclonic seizure?
- convulsions without the muscle tensing (tonic phase)
- Sudden jerking of limb, trunk or face with preserved consciousness
Features of atonic seizures?
- Sudden muscle relaxation causing patients to fall to the ground and then may motionless
- Can also result in incontinence
- Can result in post-ictal confusion
Features of a tonic seizure?
muscle tensing without convulsions (clonic phase)
General features of focal seizures?
With impaired consciousness (‘complex’): patients lose consciousness, usually post an aura or at seizure onset. Commonly originate from the temporal lobe, and post-ictal symptoms such as confusion are common.
Without impaired consciousness (‘simple’): patients retain consciousness, experiencing only focal symptoms. Post-ictal symptoms are absent.
Evolving to a bilateral, convulsive seizure (‘secondary generalised’): patients first experience a focal seizure that evolves into a generalized seizure, typically tonic-clonic
What is a Jacksonian march seizure?
A type of focal motor seizure that progressively ‘marches’ through adjacent areas of the brain
Typically starts in the hand or face then gradually spreads to other muscle groups following the smatotopic organisation of the motor cortex (starting from hand and spreading to arm, shoulder and face)
The seizure may progress into a generalised tonic clonic seizure
Often associated with structural brain lesions
What is Todd’s paresis?
Refers to temporary postictal weakness or paralysis following a seizure
Usually lasts minutes to hours but can last up to 48 hours
Usually unilateral but can be bilateral
It is transient so the patient will reover following resolution of the postictal state
What are the CSF findings in meningitis?
What is the International Headache Society criteria for migraine without aura:
What are the different motor neurone disease subtypes and do they cause upper or lower motor neurone disease signs?
Imagine findings in multiple sclerosis:
What is the Mcdonald criteria for multiple sclerosis?
