Neurology conditions Flashcards
What is Bell’s palsy?
Acute unilateral peripheral facial nerve palsy, physical exam and history unremarkable.
Fully evolves within 72 hours
What are the LMN causes of Bell’s palsy?
Idiopathic Pregnancy Diabetes Mellitus Iatrogenic Infective (Herpes) Ramsay hunt syndrome (Herpes zoster) Trauma Neurological Neoplastic Hypertension Sarcoidosis Sjorgen's Melkersson-Rosenthal syndrome
What are the causes of UMN Bell’s palsy?
Cerebrovascular disease
Intracranial tumours
Multiple slcerosis
Syphillis
HIV
Vasculitides
IF B/L immunosuppression, GBS or lyme disease
Recurrent: Lymphoma, sarcoidosis, lyme disease
Children - Lyme disease and middle ear disease
How can you tell the difference between LMN or UMN causes of Bell’s palsy?
A UMN bell’s palsy is forehead-sparing
Can still lift their eyebrows
What are the risk factors for Bell’s palsy?
Intracranial influenza vaccinaiton Pregnancy Upper respiratory tract infection Black or Hispanic ancestry Arid/cold climate Hypertension Family history of Bell's palsy Diabetes
What are the symptoms for Bell’s palsy?
Onset and progression of palsy Presence of fever Prior episodes of facial palsy Otological symptoms Presence of other cranial neuropathies Collagen vascular Use of neurotoxic medicaitons Known, prior or current malignancy Pregnancy Immunosuppression
What are the signs of Bell’s palsy?
Head and neck examination
Cranial nerve exam
Eyebrow sparing
What investigations are done for Bell’s palsy?
Serology (Lyme, Herpes and zoster)
Check BP in children
Why do you check BP in children with Bell’s palsy?
Children present with facial palsy in Aortic coarctation
What is the management for Bell’s palsy?
Steroids (prednisolone to people >16 within 72 hours)
Antivirals (moderate benefit)
Surgery (if no reduced paralysis)
What are the complications for Bell’s palsy?
Keratoconjunctiva sicca
Ectropion (Sagging eyelid)
Contracture and synkinesis
Gustatory hyperlacrimation
What are CNS tumours?
Tumours of the CNS
Cannot truly differentiate into benign and malignant as cause severe damage either way
How do you differentiate CNS tumours?
High-grade
Low-grade
Metastases
What makes a high-grade tumour?
Glioma and glioblastoma multiforme
Primary central lymphoma
Medulloblastoma
What is a low-grade tumour?
Meningioma Acoustic neuroma Neurofibroma Pituitary tumour Craniopharyngioma Pineal tumour
What cancers cause brain metastases?
Lung Breast Stomach Prostate Thyroid Colorectal
What causes CNS tumours?
Arise from any cells in the CNS
e.g. glial cells, ependymal cells, oligodendrocytes
What are the risk factors for CNS tumours?
Ionising radiation
Immunosuppression (e.g. HIV)
Inherited syndromes (e.g. Neurofibromatosis, tuberous sclerosis)
What are the signs and symptoms of CNS tumours?
Depends on size and location of tumour Headache (Worse in morning and laying down) Nausea and vomiting Seizures Progressive focal neurological deficits Cognitive and behavioural symptoms Papilloedema
What clinical features localise a tumour to the temporal lobe?
Dysphasia
Contralateral homonymous hemianopia
Amnesia
Odd/inexplicable phenomena
Which clinical features localise a tumour to the frontal lobe?
Hemiparesis Personality change Broca's dysphasia Unilateral anosmia Concrete thinking Executive dysfunction
What clinical features localise a tumour to the parietal lobe?
Hemisensory loss
Reduced 2 point discrimination
sensory inattention
Dysphasia
What clinical features localise a tumour to the occipital lobe?
Contralateral visual field defects
What are the investigations for CNS tumours?
CRP/ESR (eliminate causes)
CT/MRI/PET (visualise tumour)
MRI angiography (see blood supply to tumour)
Biopsy/Tumour removal
Distant mets are rare with primary CNS tumours
What are cluster headaches?
Neurological disorder characterised by recurrent, severe headaches on one side of the head typically around the eye, tending to recur over a period of weeks
What are the 2 types of cluster headaches?
Episodic
Chronic
What are episodic cluster headaches?
Occur in periods lasting 7 days - 1 year
Separated by pain-free periods lasting a month or longer
What are chronic cluster headaches?
Occurring for 1 year without remission or with short-lived remissions of less than a month.
Can arise de novo or from episodic cluster headaches
What causes cluster headaches?
Unknown
May be surperficial temporal artery smooth muscle hyperreactivity to 5HT
Genetic factor implicated (Autosomal Dominant gene has a role)
What are the risk factors for cluster headaches?
Male Family history Head injury Smoking Drinking
What are the signs and symptoms of cluster headaches?
Occur in clusters lasting 4-12 weeks
Occur at night, 1-2hrs after sleeping`
What are the symptoms of cluster headaches?
Occurs rapidly around 10 mins Intense, sharp and penetrating pain Centred around eye, temple or forehead Unilateral affects same side Lasts 30-45 mins Find it difficult to stay still and will pace around and bang their heads on things
What are the associated symptoms of cluster headaches?
Ipsilateral features:
- Ipsilateral lacrimation
- Rhinorrhoea
- Nasal congestion
- Eye lid swelling
- Facial swelling and flushing
- Conjunctival infection
- Partial Horner’s syndrome
What triggers cluster headaches?
Alcohol
Exercise and solvents
Sleep disruption
What are the investigations of cluster headaches?
Clinical diagnosis based on history
Neurological examination may be useful
What is Epilepsy?
Recurrent tendency to spontaneous, intermittent, abnormal electrical activity in part of the brain, manifesting as seizures (paroxysmal synchronised cortical electrical discharges)
What are the types of seizures?
Partial - Complex - Simple Generalised - Tonic-clonic - Abscence - Myoclonic - Atonic
What are the structural causes of epilepsy?
Cortical scarring Developmental Space-occupying lesion Stroke Hippocampal sclerosis Vascular malformations
What are the non-epileptic causes of seizures?
Trauma Stroke Haemorrhage Raised ICP Alcohol or Benzodiazepin withdrawal Liver disease Infection High temperature Drugs (Cocaine, tramadol, theophylline, tricyclics) Metabolic disturbance
What are the ‘other’ causes of epilepsy?
Tuberculous sclerosis
Sarcoidosis
SLE
PAN
What are common things that look like seizures?
Syncope
Migraine
Non-epileptiform seizure disorder (e.g. dissociated disorder)
What are the types of frontal/partial seizure epilepsy?
Frontal lobe focal motor seizure
Temporal lobe seizure
Frontal lobe complex partial seizure
What are the signs and symptoms of frontal lobe focal motor seizure?
Motor convulsions
Jacksonian march
Post-ictal flaccid weakness (Todd’s paralysis)
What is a jacksonian march?
Muscular spasm spreads from distal limb to ipsilateral face
What are the signs and symptoms of temporal lobe seizure?
Aura (Visceral or psychic symptoms)
Hallucinations (Usually olfactory or affecting taste)
What are the signs and symptoms of frontal lobe complex partial seizure?
Loss of consciousness
Involuntary actions/disinhbition
Rapid recovery
What are the types of generalised seizures?
Tonic-clonic (Grand mal)
Abscence (Petit mal)
Non convulsive status epilepticus
What are the signs and symptoms of grand mal seizures?
Vague symptoms before attack (irritability)
Tonic phase (generalised muscle spasm)
Clonic phase (Repetitive synchronous jerks)
Faecal/urinary incontinence
Tongue bitings
Post-ictal phase: Impaired consciousness, lethargy, confusion, headache, back pain, stiffness
What are the signs and symptoms of petit mal seizures?
Onset in childhood
Loss of consciousness but maintained postures
The patient will appear to stop talking and stare
NO post-ictal phase
What are the signs and symptoms of non-convulsive status epilepticus?
Acute confusional state
Often fluctuating
Difficult to distinguish from dementia
What are the investigations for Epilepsy?
FBC U&E LFTs Glucose Calcium Magnesium ABG Toxicology screen Prolactin EEG CT/MRI Other
How do you manage newly diagnosed epilepsy?
Start anti-convulsant treatment after >2 unprovoked seizures
Drugs change depending on type of epilepsy
- Generalised: Sodium valproate or Lamotrigine
- Abscence: Sodium valproate, lamotrigine or ethosuximide
- Partial: Carbamezapine
What is status epilepticus?
A seizure lasting >30 mins or repeated seizures without recovery and regain of consciousness in between
How do you manage status epilepticus?
ABC approach Check glucose IV lorazepam or IV/PR Diazepam If they recur, use IV phenytoin - need ECG monitor If fails consider general anaesthesia Treat cause Check plasma levels of anticonvulsants
What are the complications of epilepsy?
Fractures from tonic-clonic seizures Behavioural problems Sudden death Complications of drugs: - Gingival hypertrophy (Phenytoin) - Neutropenia and osteoporosis (Carbamezapine) - Stevens-Johnson syndrome (Lamotrigine)
What is an extradural haemorrhage?
Bleeding and accumulation of blood in the extradural space -between dura mater and skull
What causes an extradural haemorrhage?
Trauma
- Usually due to fracture of the pterion area - rupturing the middle meningeal artery.
What are the risk factors for extradural haemorrhage?
Age
Bleeding tednency (E.g. haemophilia)
Drugs (warfarin)
What are the symptoms of extradural haemorrhage?
Temporary loss of consciousness /drowsiness
Then Lucid interval (resolved consciousness levels)
Then progressive deterioration in GCS as ICP rises
Increasing severe headache/vomiting/confusion/ fits +/- hemiparesis with brisk reflexes and upgoing plantars
Ipsilateral pupil dilation, coma deepens, b/l limb weakness
What are the signs of extradural haemorrhage?
Scalp traumas or fracture Signs of raised ICP (dilated, unresponsive pupils) Cushing's response - Hypertension - Bradycardia - Irregular breathing
What are the investigations for extradural haemorrhage?
Urgent CT scan
- Check for haematoma, may get a contracoup injury on opposite side
- Look for features of raised ICP e.g. midline shift
What is Guillain-Barre syndrome?
GBS is a type of acute inflammatory neuropathy.
Clinically defined syndrome associated with motor difficulty, absence of deep tendon reflexes, paraesthesias without sensory losses and an increase in CSF albumin without cellular reaction
What are the subtypes of GBS?
Acute inflammatory demyelinating polyardiculoneuropathy
Acute motor and sensroy axonal neuropathy
Acute motor axonal neuropathy
Acute sensory neuropathy
Acute pandysautomnia
Which Antibodies are the types of GBS associated with?
AIDP - none
AMSAN - GM1, GM1b & GD1a
AMAN - GM1, GM1b, GD1a, GalNac-GD1a
Acute sensory neuropathy - GD1b
What are the risk factors for GBS?
History of GI or respiratory infections (1-3 weeks after)
Association with zika virus
Vaccinaitons
Malignancies (lymphomas)
Pregnancy (Decreases during pregnancy and increases after delivery)
What are the symptoms of GBS?
Weakness Pain Reflexes Sensory symptoms Autonomic symptoms
What are the signs of GBS?
Hypotonia Demonstrate altered sensation or numbness Reduced or absent refelxes Fasciculation may occasionally be absent Facial weakness (asymmetrical) Autonomic dysfunction Respiratory muscle paralysis
What investigations are done for GBS?
Electrolytes Lumbar puncture (Raised CSF protein, no raised CSF cells, not until weakness) Antibody screen Spirometry Nerve conduction studies ECG
What is the management of GBS?
Plasma exchange IV immunoglobulin DVT prophylaxis Admission to ICU Pain relief
What are the complications of GBS?
Persistent paralysis
Respiratory failure requiring ventilation
Hypotension or Hypertension
Thromboembolism, pneumonia, skin breakdown
Cardiac arrhythmia
Ileus
Aspiration pneumonia
Urinary retention
Psychiatric problems e.g. depression, anxiety
What is Horner’s syndrome?
Disruption of sympathetic nerves supplying the eye
What are the three things of Horner’s syndrome?
Partial ptosis (upper eyelid drooping) Miosis (Pupillary constriction leading to anisocoria) Hemifacial anhidrosis (abscence of sweating)
What causes horner’s syndrome?
Cranial nerve lesions
Preganglionic nerve lesions
Postganglionic nerve lesions
What causes cranial nerve lesions?
MS Cerebrovascular accidents Pituitary or basal skull tumours Basal meningitis Neck trauma Syringiomyelia Anrold-chiari malformation Spinal cord tumours
What causes pre-ganglionic nerve lesions?
Apical lung tumours (Pancoast tumour) Lympadenopathy Lower brachial plexus trauma or rib Aortic aneurysm Trauma or surgical injury Neuroblastoma Mandibular dental abscess
What causes post-ganglionic nerve lesions?
Cluster headaches or migraines Herpes zoster infectoin Internal carotid artery dissection Raeder's syndrome Carotid-cavernous fistula Temporal arteritis
What investigations should be done in Horner’s syndrome?
CXR
CT/MRI
CT angiography/Carotid ultrasound
What is the pharmacological testing for Horner’s?
- Cocaine eye drops normally cause dilatation of eyes but wont in horners
- Apraclonidine is an alternative to cocaine - dilates a horner’s pupil but not a normal one
- Hydroxyamfetamine causes dilatation in first and second order lesions but not third
What is the management of horner’s
Treat underlying cause
What is Huntington’s disease?
Autosomal dominant trinucleotide repeat disease (CAG repeat on chromosome 4)
What characterises huntington’s disease?
Progressive chorea and dementia
Typically starts in middle age
What causes huntington’s disease?
The huntingtin gene codes for huntingtin
In the huntingtin gene there is a trinucleotide repeat expansion that results in toxic gain of function
Causes atrophy and neuronal loss of striatum and cortex
Autosomal dominant
Earlier age of onset with each generation
What are the symptoms of huntington’s disease?
Family history Insidious onset in middle age Progressive Early symptoms Late symptoms Drug history (cocaine, antipsychotics)
What are the early symptoms of huntington’s disease?
Lability Dysphoria Irritability Incoordination Fidgeting Clumsiness Mental inflexibility Anxiety Develops in dementia
What are the late symptoms of huntington’s disease?
Rigid Involuntary, jerky, duskinetic movement soften accompanied by grunting and dysarthria (chorea) Dementia Fits Akinetic Bed-bound Death
What are the signs of huntington’s disease?
Chorea Dysarthria Slow voluntary sacades Supranuclear gaze restriction Parkinsonism Dystonia MMSE shows cognitive and emotional deficits
What are the investigations for huntington’s disease?
Genetic analysis
- Diagnosed if >39 CAG repeats
- Reduced penetrance leads to intermediate number of CAG repeats
Imaging
- Brain MRI/CT show symmetrical atrophy of striatum and butterfly dilation of the lateral ventricles
Bloods
What is Hydrocephalus?
Enlargement of the cerebral ventricular system due to accumulation of CSF.
Too much CSF produced, blocked CSF flow or insufficient CSF reabsorbing
What are the types of CSF?
Non-obstructive / communicating
Hydrocephalus ex vacuo
Obstructive / non-communicating hydrocephalus
What is a communicating hydrocephalus?
Impaired CSF reabsorption into the subarachnoid villi
What is a hydrocephalus ex vacuo?
Apparent enlargement of the ventricles as a compensatory change due to brain supply
What is a non-communicating hydrocephalus?
Blockage of CSF within the ventricles
What are risk factors for hydrocephalus?
Congenital: - Premature birth causing bleeding in ventricles - Uterus infection - CNS abnormal development Acquired: - CNS infeciton - Bleeding in brain from trauma/stroke - Age >65 years - Vascular disease
What are the symptoms of obstructive/communicating hydrocephalus?
Acute drop in GCS
6th nerve palsy causing diplopia
What are the symptoms of normal pressure hydrocephalus?
Dementia
Gait disturbance
Urinary incontinence
What are the signs of obstructive hydrocephalus?
Low GCS
Papilloedema
6th nerve palsy
Why is the 6th cranial nerve most susceptible to raised ICP?
It has the largest intracranial path
What are the signs of obstructive jaundice in neonates?
Increased head circumference Sunset sign (downward cojugate deviation of the eyes)
What investigations are done for Hydrocephalus?
CT head (First line)
CSF
- Ventricular drain or LP, indicate pathology
LP (Contraindicated in raised ICP, therapeutic in normal pressure hydrocephalus)
What is a migraine?
Chronic, episodic, neurological disorder that presents in early-to-mid life
What causes migraines?
Brains are hyperexcitable to a variety of stimuli
What are the risk factors for migraines?
Family history of migraine High caffeine intake Exposure to barometric pressure Female sex Obesity Habitual snoring Stressful life events Overuse of headache movements Lack of sleep Low socio-economic status Allergies or asthma Hypertension Hypothyroidism Diet
What are the symptoms for migraine?
Visual or other aura
Aura without headache
Episodic severe headaches without aura, often premenstrual, U/L, with allodynia
What are the signs for migraine?
None
What are the criteria for migraines without aura?
>/=5 headaches lasting 4-72 hours Nausea / vomiting Photo/phonophobia With 2 of: - Unilateral - Pulsating - Abnormalities suggesting another cause
What are the investigations for Migraines?
ESR LP CSF culture CT head MRI brain
What are red flags seen on examination for migraines?
Papilloedema
New seizure
Abnormal neurological signs
New-onset cluster headaches
What are the orange flags seen on examination for migraines?
New headache and no other clear diagnosis
Headache aggravated by exertion or valsalva
Headache with vomiting
New headache in 50+
Changed pattern of headaches
What are the yellow flags for migraines?
Migraine
Memory loss
What is the management for migraines?
Relieve symptoms (NSAIDs > Triptans) - if not, paracetamol
Anti-emetics for N&V
High flow O2 via non-rebreather mask
Reduce frequency and severity of migraine attacks
Identify trigger factors to avoid them
Prophylaxis if occurs >2/month
What can be done as prophylaxis for migraines?
Start low-dose and re-evaluate after trial
Non-pharmaceutical help: CBT, Biofeedback- assisted relaxation
Treat for 4-6 months
Menstrual migraine > hormonal therapy to suppress menses
Main drugs: Anticonvulsants (topiramate), tCAs and Beta blockers
CCBs for hemiplegic migraine and migraine with brainstem aura
What are the complications for migraines?
Status migrainosus Migrainous infarction Migraine-triggered seizures Depression Chronic migraine Persistent aura without infarciton
What is motor neuron disease?
A progressive neurodegenrative disorder of cortial, brainstem and spinal motor neurons (LMN and UMN) - this is selective loss of neurons in more cortex, cranial nerve nuclei and anterior horn cells!
- No sesnory loss
What are the subtypes of MND?
Amyotrophic lateral sclerosis (ALS)
Progressive muscular atrophy variant
Progressive bulbar palsy variant
Primary lateral sclerosis variant
What is ALS?
Lou gherig’s disease
Combined degeneration of upper and lower motor neruoens resulting in a mix of LMN and UMN signs
What is progressive muscular atrophy variant MND?
Only LMN signs
Better prognosis
What is progressive bulbar palsy variant MND?
Only affects CN IX-XII Dysarthria Dysphagia Wasted fasciculating tongue Brisk jaw jerk reflex
What is primary lateral sclerosis variant MND?
Loss of Betz cells in motor cortex UMN pattern of weakness Brisk reflexes Extensor plantar responses No LMN signs
What causes motor neuron disease?
Unknown
Free radical damage and gluatamate excitotoxicity have been implicated
Associated with frontotemporal lobar dementia
What are the risk factors for MND?
Genetic predisposition/family history
Age > 40
Smoking
Athleticism/ family history
What are the symptoms of MND?
Weakness of limbs (proximal myopathy)
Speech disturbance
Swallowing disturbances (choking on food)
Behavioural changes
What are the signs of MND?
Combo of UMN and LMN signs LMN - Muscle wasting - Fasciculations - Flaccid weakness - Hyporeflexia UMN - Spastic weakness - Extensory plantar response - Hyperreflexia Sensory exam is normal Frontotemporal dementia occurs in 25%
What are the investigations for MND?
Clinical diagnosis
EMG: Diffused/ongoing/chronic denervation seen
Nerve-conduction studies
MRI to exclude cord compression/brainstem lesions
Bloods
- Mild elevation of CK
- ESR (Raised)
- Anti GM1-ganglioside antiboides (Negative)
What is multiple sclerosis?
Inflammatory demyelinating disease of the CNS - discrete plaques of demyelination occur at multiple CNS sites, from T-cell mediated immune response
What are the types of MS?
Relapsing-remitting MS
Clinically isolated syndrome
Primary progressive MS
Marburg variant
What is relapsing-remitting MS?
Commonest form
Clinical attacks of demyelination with poor healing in between attacks
What is Clinically isolated syndrome?
Single clinical attack of demyelination
The attack itself doesn’t count as MS
10-50% progress to MS
What is Primary progressive MS?
Steady accumulation of disability with NO relapsing-remitting MS
What is Marburg variant MS?
Severe fulminant variant of MS leading to advanced disability or death within weeks
What causes MS?
Unknown
AI bases with potential environmental trigger in genetically susceptible individuals
Immune-mediated damage to myelin sheaths results in impaired axonal conduction
What are the risk factors of MS?
EBV exposure
Prenatal vitamin D levels
What are the symptoms of MS?
Varies on site Usually monosymptomatic Optic neuritis (most common) Sensory Motor Autonomic Psychological/cognitive Sexual GI Cerebellum Early on, relapses then remits to full recovery Uhthoff's sign Lhermitte's sign
What are the optic neuritis symptoms?
Unilateral deterioration of visual acuity and colour perception
Pain on eye movement
Rapid loss of centrla vision
What are the sensory symptoms of MS?
Pins and needles
Numbness
Burning
What are the motor symptoms of MS?
Limb weakness
Spasms
Stiffness
Heaviness
What are the autonomic symptoms of MS?
Urinary urgency
Hesitancy
Incontinence
Impotence
What are the psychological/cognitive symptoms of MS?
Depression
Psychosis
Amnesia
Reduced executive fucntioning
What are the sexual symptoms of MS?
Erectile dysfunction
Anorgasmia
What are the GI symptoms of MS?
Swallowing disorders
Constipation
What are the cerebellar symptoms of MS?
Trunk and limb ataxia
Intention tremor
Scanning speech falls
What is Uhthoff’s sign?
Worsening of neurological symptoms as body gets overheated from hot weather, saunas, hot tubs etc
What is Lhermitte’s sign?
Electrical sensation that runs down the back and into the limbs when neck is flexed
What are the signs of MS?
Optic neuritis Visual field testing RAPD Internuclear ophthalmoplegia Paraesthesia UMN signs Limb ataxia Dysdiadochokinesia Ataxic wide-based gait Scanning speech Central scotoma Loss of coloured visions Impaired visual acuity
What are the investigations for MS?
Diagnosis based on finding of 2 or more CNS lesions with corresponding symptoms, seperated in time and space - Mcdonald criteria!
Lumbar puncture
- Microscopy (exclude infection/inflammatory causes)
- CSF electrophoresis shows unmatched oligoclonal bands
MRI brain
- Plaques identifies
- Gadolinium enhancement shows active lesions
Evoked potentials
- Visual, auditory and somatosensory evoked potentials may show delayed conduction velocity
What is Myasthenia gravis?
An AI disease affecting the neuromuscular junction producing weakness in skeletal muscles
What causes myasthenia gravis?
Antibodies against the nicotininc acetylcholine receptor which interferes with the neuromuscular transmission via depletion os post-synaptic receptor sites
Can be paraneoplastic
What is Lambert-Eaton syndrome?
Paraneoplastic subtype of MG caused by auto antibodies against pre-synaptic calcium channels, leading to impairment of acetylcholine release
What are the risk factors for MG?
AI conditions
What are the symptoms of MG?
Muscle weakness that worsens with repetitive use or towards the end of the day - fatiguability Ocular symptoms - Drooping eyelids - Diplopia Bulbar symptoms - Facial weakness (myasthenic snarl) - Disturbed hypernasal speech - Difficulty smiling, chewing or swallowing
What is the order of muscles that become weakened?
Extraocular > Bulbar > Face > Neck > Limb girdle > Trunk
In lambert-eaton syndrome fatiguability improves with use
What are the signs of MG?
May be generalised May be bulbar (relating to medulla oblongatea) May be ocular Eye signs - Ptosis - complex opthalmoplegia - ocular fatigue Ice on eyes test (putting ice packs on eyelids for 2 mins improves transmission and reduces ptosis) Bulbar signs (reading aloud causes dysarthria or nasal speech) Limbs - Test power - tendon reflexes are normal
What are the investigations for MG?
CK (Exclude myopathies) Serum acetylcholine receptor antibody If seronegative - TFTs - Anti-voltage gated calcium channel antibodye Tensilon test (Not used) Nerve conduction study EMG CT thorax/CXR - visualise thymoma or lung malignancies
What is Neurofibromatosis?
An Autosomal dominant genetic disorder affecting cells of neural crest origin, resulting in the development of multiple neuroctaneous tumours
There are 2 types
What is type 1 neurofibromatosis?
Von Recklinghausen’s disease
- Peripheral and spinal neurofibromatosis
- Multiple cafe au lait spots
- Freckling (axillary/inguinal)
- Optic nerve glioma
- Lisch nodules (on iris)
- Skeletal deformities
- Phaeochromocytomas
- Renal artery stenosis
What is Neurofibromatosis type 2?
A condition characterised by:
- Schwannomas
- Meningiomas
- Gliomas
- Cataracts
What causes neurofibromatosis?
Associated with mutations in tumour suprression genes NF1 and NF2
What are the risk factors for neurofibromatosis?
Severe crush trauma
Parent with neurofibromatosis
What are the symptoms of type 1 neurofibromatosis?
Family history Skin lesions Learning difficulties (40%) Headaches Disturbed vision (optic gliomas) Precoccious puberty (lesions of pit. gland from an optic glioma)
What are the symptoms of type 2 neurofibromatosis?
Family history Hearing loss Tinnitus Balance problems Headache Facial pain Facial numbness
What are the signs of type 1 neurofibromatosis?
5+ cafe au lait macules of >5mm (prepubertal)
5+ cafe au lait macuels of >15mm (Post-pubertal)
Freckling in armpit or groin
Lisch nodules (hamartomas on the iris - slit lamp)
Spinal scoliosis
Short stature
What are the signs of type 2 neurofibromatosis?
Few or no skin lesions
Sensorineural deafness with facial nerve palsy or cerebellar signs (if schwannoma is large)
What are the investigations for neurofibromatosis?
Full body exam for skin lesions Ophthalmological assessment Audiometry MRI brain and spinal cord Skull X-ray (sphenoid dysplasia in NF1) Genetic testing
What is Parkinsons’s disease?
A neurodegenerative disorder
What is the triad of parkinsonism?
Tremor
Rigidity/increased tone
Bradykinesia/hypokinesia
What causes primary parkinson’s disease?
Genetic component, subsequent environmental factors/exposure contributing to evolution of clinical disease
What causes secondary parkinson’s disease?
Neuroleptic therapy (e.g. schizophrenia)
Vascular insults (e.g. in basal ganglia)
MPTP toxin from illicit drug contamination
Post-encephalitis
Repeated head injury
Manganese of copper toxicity (wilson’s diseae)
HIV
What are the risk factors for parkinson’s disease?
Increasing age
History of familial PD in younger-onset disease
Mutation in gene encoding glucocerebrosidase
Metal exposure
Male
Head truama
Toxin exposure
Occupation (Teacher, HCP, construction worker, carpenter or cleaner)
What are the key diagnostic features of signs and symptosm?
Presence of risk factors Bradykinesia Resting tremor Rigidity Postural instability
What are the other diagnostic features of parkinson’s disease?
Masked facies Hypophonia Hypokinetic Micrographia Stooped posture Shuffling gait Conjugate gaze disorders Fatigue Constipaiton Depression Anxiety Dementia
What are the uncommon features of parkinson’s disease?
Exposure to Neuroleptics or antiemetics
Featrues of atypical parkinsonism (Acute onset, rapid progression, cognitive impairment)
What are the investigations for Parkinson’s disease?
Dopaminergic agent trial (improved symptoms)
MRI brain (normal)
Functional neuroimaging
Olfactory testing
Brain pathology
Genetic testing
Lewy bodies and positive reactivity to synuclein with immunohistochemical staining
What is Radiculoapthy?
Spinal nerve root compression
What are the most common types of radiculopathy?
Lumbar and cervical radiculopathy
What are the causes of radiculopathy?
Herniated disc Spinal stenosis Degenerative disc disease Osteoarthritis Facet joint degeneration/hypertrophy LIgamentous hypertrophy Spondylolisthesis (vertebra moves and rests on vertebrae below) Rarer: Radiation, DM, Neoplastic disease, lyme meningitis
What are the risk factors for radiculopathy?
Age Overweight Poor posture Improper lifting technique Family history of degenerative bone conditions
What are the signs and symptoms of radiculopathy?
Lumbar or sacral radiculopathy (primary sciatica)
Pain/electrical sensations in arms or fingers or legs at level of compression
Dull reflexes
Dynatomal sensory disturbance (numbness, tingling, reduced pain and temperature sensation)
LMN weakness and eventual muscle wasting
UMN signs below affected level (Spasticity, weakness, brisk reflexes and upgoing plantars)
Position and vibration sense may be lost
What are the investigations for Radiculopathy?
Full Neurological exam MRI X-ray and MRI EMG to test nerve function Nerve conduction studies
What is Raised intracranial pressure?
The volume inside the cranium is fixed so any increase in the contents can lead to raised ICP/ Can be mass efect, oedema or obstruction to fluid outflow
Normal ICP in adults is <15mmHg
What causes raised intracranial pressure?
Primary or metastatic tumours Head injury Haemorrhage Infection (Meningitis, encephalitis, brain abscesses) Hydrocephalus Cerebral oedema Status epilepticus Aneurysms
What are the symptoms of a raised ICP?
Headache (worse on coughing and leaning forward, worse in morning) Vomiting Altered GCS History of trauma Poor vision
What are the signs of a raised ICP?
Altered GCS
Falling pulse and rising BP (Cushing’s response)
Cheyne-Stokes respiration
Pupil changes
Reduced visual acuity
peripheral visual field loss
Papilloedema (unreliable sign but venous pulsaiton of disc may be absent)
What is Cheyne-stokes respiration?
Progressively deeper and sometimes faster breathing followed by a gradual decrease that results in a temporary stop in breathing
What are the investigations for Raised ICP?
U&Es FBC LFT Glucose Serum osmolality Clotting Blood culture Consider toxicology screen CXR CT head Consider LP if safe
What is spinal cord compression?
Pressure injury to the spinal cord with Neurological symptoms dependent on site and extent of the injury
What is Cauda equina syndrome?
Symptoms due to damage to the bundle of nerves below the end of the spinal cord (L2 down)
What is conus medullaris syndrome?
Tapered, lower end of the spinal cord i the conus medullaris.
Conus medullaris syndrome is compression at L1/L2
What causes spinal cord compression?
Most causes: Trauma and tumours Tumours are more frequently metastases Spinal abscess TB (Pott's disease) Haematoma (warfarin) Intrinsic cord tumour Atlanto-axial sublulxation Myeloma Congenital lumbar disc disease
What are the risk factors for spinal cord compression?
Trauma Malignancy Osteoporosis Metabolic bone disease Vertebral disc disease
What are the symptoms of spinal cord compression?
History of trauma or malignancy Pain Weakness Sensory loss Disturbance of bowel and bladder function
What are the signs of spinal cord compression?
Normal above lesions Diaphragmatic breathing Reduced anal tone Hyporeflexia Priapism Spinal shock Sensory loss (level of lesion) Motor - Weakness or paralysis - Downward plantars (acute) - UMN signs below lesions - LMN signs at level of lesion
What is Brown-Sequard syndrome?
Seen with hemisection of spinal cord
What are the investigations for spinal cord compression?
Radiology
- Lateral radiographs of spine to look for loss of alignment, fractures etc
- MRI
Bloods
- FBC, U&E, Calcium, ESR, Immunoglobulin electrophoresis (Multiple Myeloma), syphilis serol, LFTs, PSA
- CXR for lung malignancy, TB
- Urine (Bence Jones proteins - multiple myeloma)
What is a stroke?
Acute neurological deficit of two different types
What are the types of stroke?
Ischaemic or Haemorrhagic
What is an ischaemic stroke?
Acute neurological deficit lasting more than 24 hours and caused by cerebrovascular aetiology caused by vascular occlusion or stenosis.
Resulting in intraparenchumal and/or subarachnoid haemorrhage
What is a Haemorrhagic stroke?
Acute neurological deficit caused by cerebrovascular aetiology. Due to a rupture of a cerebrospinal artery, resulting in intraparenchymal subarachnoid and intraventricular haemorrhage
What causes an ischaemic stroke?
Transient of permanent critical reduction in cerebral blood flow due to arterial occlusion, Hypotension, vasculitis or cocaine
How does thrombosis cause ischaemic strokes?
Lacunar infarcts (Small vessels)
Can occur in larger vessels (middle cerebral artery)
Can arise in prothrombotic states (e.g. dehydration, thrombophilia)
How does an embolus cause ischaemic stroke?
Carotid dissection
Carotid atherosclerosis
AF
What causes a haemorrhagic stroke?
Hypertension Charcot-Bouchard microaneurysm rupture Amyloid angiopathy Arteriovenous malformaitons Anticoagulant therapy Trauma Tumours Vasculitis
What are the risk factors for ischaemic stroke?
Older age Family history History of stroke Hypertension Smoking Diabetes mellitus Atrial fibrillaiton Comorbid cardiac conditions Carotid artery stenosis Sickle cell disease Dyslipidaemia People with lower levels of education Obesity Elevated CRP Aortic arch plaques
What are the risk factors for haemorrhagic stroke?
Hypertension Advanced age Male sex Asian, black and/or hispanic Family history of heamorrhagic stroke Asian, black or hispanic Haemophilia Smoking Illicit sympathomimetic drugs Leukaemia Thrombocytopeina AD mutaitons Hereditary haemorrhagic telangiectasia Leukaemia Moyamoya disease NSAID
What are the symptoms of ischaemic stroke?
Sudden onset Vision loss or visual field deficit Impaired co-ordination Loss of function Diplopia Sensory loss Vertigo/dizziness Nausea and / or vomiting Confusion altered sensation Headahce Neck/facial pain
What are the signs of ischaemic stroke in the anterior cerebral artery?
Hemiparesis
Confusion
Disturbance of intellect, executive function, judgement and social behaviour
What are the signs of ischaemic stroke in the middle cerebral artery?
Facial weakness Hemiparesis Hemisensory loss Apraxia Hemineglect Aphasia if left sided Hemineglect Quadrantopia
What are the signs of ischaemic stroke in the posterior cerebral artery?
Homonymous hemianopia, visual agnosia
What are the symptoms of haemorrhagic stroke?
Neck stiffness History of AF History of liver disease Altered sensation weakness Headache Sensory loss Aphasia Dysarthria Ataxia Vertigo Nausea/vomiting Confusion Gaze paresis Altered consciousness
What are the signs of haemorrhagic stroke?
Meningism Severe headache Coma within hrs Carotid bruit AF previous TIA IHF
What investigations are done for ischaemic stroke?
FBC Serum glucose (normal) Serum electrolytes (normal) Serum U&Es (Normal) Cardiac enzymes (normal) PTT/aPTT (Normal/coagulopahty) Toxicology screen ESR Carotid doppler US CT head MRI brain ECG CXR CT cerebral angiogram
What are the investigations for haemorrhagic stroke?
Chemistry panel (normal) CBC (exclude thrombocytopenia) Clotting test Platelet function test LFT Non-infused CT ECG CT angiography and venography MR angiography and venography MRI brain with diffusion-weighted imaging adn gradient echo sequence
What is the management for stroke?
<4.5 hrs since onset:
- CT Head to exclude haemorrhage
- IV alteplase
- Aspirin 24 hrs later
> 4.5hrs since onset;
- 300mg Aspirin (heparin if high emboli recurrence)
- Swallow assessment
- Thromboprophylaxis
Secondary:
- clopidogrel / aspirin 75mg
- warfarin if paroxysmal AF
- control risk factors
Surgical:
Carotd endarterectomy
What are the complications of stroke?
Aspiration pneumonia Cerebral oedema Immobility Infections DVT Cardiovascular events Death
What is a subarachnoid haemorrhage?
Arterial haemorrhage into the subarachnoid space
What causes a subarachnoid haemorrhage?
Rupture of a saccular aneurysms at base of brain (berry aneurysm) - 85%
Perimesencephalic haemorrhage - 10%
Arteriovenous malformations, bleeding diathesis, vertebral artery dissection - 5%
What are the risk factors for a subarachnoid haemorrhage?
Hypertension Smoking Excessive alcohol intake Bleeding disorders Family history of SAH Saccular aneurysms
What are saccular aneursysms associated with?
PKD Coarctation of he aorta Marfan's syndrome Ehlers-Danlos syndrome SLE
What are the symptoms of SAH?
Sudden-onset worst headache ever Nausea/vomiting Collapse Seizures Neck stiffness Photophobia Reduced level of consciousness
What are the sign’s of SAH?
Meningism (Neck stiffness, kernig’s sign, pyrexia)
GCS - Check for deterioration
Signs of raised ICP
- Papilloedema, CN IV/III palsies, HTN, bradycardia, focal neurological signs
What investigations are done for SAH?
FBC (Leucocytosis) INR (Raised) PTT (Raised) Troponin (High) CT scan asap if inconclusive - LP (increased pressure, incerased red cells, blood then xanthochromia) ECG: 50% have an abnormal ECG
What is a Subdural haemorrhage?
A collection of clotting blood that forms in the subdural space
What are the 3 classifications of subdural haemorrhage?
Acute
Subacute (3-7 days after)
Chronic (2-3 weeks after)
Who are the at risk groups of SDH?
Infants
Elderly
Alcoholics
People on anticoagulation treatment
What causes SDH?
Trauma
Less commonly: Rupture of aneurysm or vascular malformations
What are the risk factors for SDH?
Recent trauma
Coagulopathy and anticoagulant use
Advanced age (>65)
What are the symptoms of acute SDH?
History of trauma
Reduced consciousness level
What are the symptoms of subacute SDH?
Worsening headache 7-14 days after injury
Altered mental state
What are the symptoms of chronic SDH?
Headache Confusion Cognitive impairment Gait deterioration Focal weakness Seizures Sleepiness
What are the signs of acute SDH?
Reduced GCS Ipsilateral fixed dilated pupil Pressure on brainstem (reduced consciousness + bradycardia) Evidence of trauma Papilloedema
What are the signs of chronic SDH?
Neurological examination may be normal
Focal neurological signs (3rd nerve palsy)
Evidence of trauma
Papilloedema
What investigations are done for SDH?
FBC U&Es LFTs Thrombocytopenia Coagulation screen Cross-match/Group and save CT head C-spine imaging
What is the management of acute SDH?
ALS protocol Watch out for C-spine injury Raised ICP consider osmotic diuresis Conservative if small Surgical (for irrigation/evacuation) - Burr twist drill and burr hole craniotomy - Craniotomy
What is the management for chronic SDH?
Burr hole or craniotomy and drainage
Assess any causes of trauma
Children - treat with percutaneous aspiration via open fotanelle
What are complications of SDH?
Neurological deficits Come Stroke Surgical-site infection Epilepsu Recurrence of SDH postoperatively
What is a tension headache?
Generalised headache throughout the head with a predilection for involving the frontal and occipital regions.
Pain expressed as being a ‘tight-band’ around the head
TTH can be episodic or chronic
What causes tension headache?
Muscle contraction considered cause of pain
Stress most common trigger
Disturbed sleep patterns can trigger episodic headache
Insomnia and other sleep disorders are associated with chronic headache
What are the risk factors for headaches?
Mental tension Stress Missing meals Fatigue Somatisation Female sex 20-39 years old Lower socioeconomic status Analgesic overuse
What are the signs and symptoms of tension headache?
Pressure or tightness
Related to neck pain
Non-pulsatile head pain
Often photophobia and exacerbation by movement are common
Mild nausea may occur
Head pain, frontal or occipital head pain
What are the investigations for tension headache?
Not needed but can do CT sinus, MRI brain and LP which would all be normal
What is the management for tension headaches?
Reassure positive diagnosis
Attention to stress, anxiety or depression
Physiotherapy for mobilisation
Advice on opioids/medications
Simple analgesia
- Ibuprofen fine, possibly paracetamol or naproxen, aspirin
TCA
- Amitriptyline
What is a TIA?
Episode where someone has signs or symptoms of a stroke but they only last a short amount of time.
Usually last a few minutes - few hours
What are the risk factors for TIA?
Diabetes Age >40 Severely clogged or narrowed arteries Obesity Heart disease Illegal drug use or heavy alcohol use Recent childbirth Sedentary lifestyle/lack of exercse Current or past history of blood clots High blood cholesterol
What are the signs and symptoms of TIA?
Single or many attacks
Similar in the arterial area to a stroke there
Amaurosis fugax if goes to retinal artery
May have limb-shaking
Syncope, dizziness not typical in TIAs
What are the investigations for TIA?
Blood glucose (<3.3) U&Es (Low Na+, K+ or High Ca2+) FBC (possibly raised, polycythaemia, thrombocytopenia) Brain MRI [First line] CXR
How do you manage TIA?
ABCD2 assessment
- If = 3: Aspirin or clopidogrel adn a statin
- If >/= 4: Aspirin or clopidogrel, measure for secondary CVS prevention
Those with AF should be anticoagulated
Cardiac emboli / Af need warfarin
Carotid endarterectomy if >/= 70% stenosis on internal carotid
How does a TIA affect their ability to drive?
Group 1 (Car/motorcycle): - Not drive for a month, no need to tell DVLA for 1 TIA, multiple needs 3 months off
Group 2 (lorry or bus): - Licence refused/revoked for a year
What are the complications for TIAs?
Stroke
Myocardial infarction
What is trigeminal Neuralgia?
Neuralgia involving one or more of the brances of the trigeminal nerves, often causing severe pain
What triggers trigeminal Neuralgia?
Vibration Skin contact Eating Talking Dental protheses Brushing teeth Exposure to wind
What causes trigeminal neuralgia?
Thought to be due to compression of the trigeminal nerve by a loop of artery or vein
Secondary causes (5-10%)
Can be MS or Brainstem lesions but thats rare
Compression of the trigeminal root by anomalous or aneurysmal intracranial vessels or a tumour
What are risk factors for trigeminal neuralgia?
Increased age
MS
What are the signs and symptoms of trigeminal neuralgia?
Sudden u/l stabbing pain in distribution of one or more of the branches of the trigeminal nerve
Recurrent
Lasts from a few seconds to a couple of mins
Periods of reission may vary
Pain is decribed a being shock-like (Face screws with pain)
What are the investigations for Trigeminal neuralgia?
Diagnosis is clinical
MRI needed to exclude secondary diagnosis
What is Wernicke’s encephalopathy?
Presence of Neurological symptoms caused by biochemical lesions of the CNS following exhaustion of Vitamin B reserves
What causes wernicke’s encephalopathy?
Main cause is alcohol consumption which results in thiamine deficiency by causing: - Inadequate nutritional thiamine intake - Decreased thiamine absorption - Impaired thiamine utilisation by cells Other causes: - Eating disorders - Malnutrition - Prolonged vomiting - GI malignancy - Chronic subdural haematoma - AIDS - Hyperemesis gavidarum - Thyrotoxicosis
What is Hyperemesis gravidarum?
Severe nausea, vomiting, weight loss in pregnancy
What are the risk factors for Wernicke’s encephalopathy?
Alcohol dependence AIDS Cancer treatment Malnutrition/eating disorder History of GI surgery
What are the symptoms of wernicke’s encephalopathy?
Vision changes
Loss of muscle coordination
memory loss/change
Hallucinations
What are the signs of Wernicke’s encephalopathy?
Triad - Confusion - Ophthalmoplegia - Ataxia Patients are mentally alert with vocabulary, comprehension, motor skills, social habits and naming ability maintained You will see: - Decreased reflexes/temperature - Rapid pulse Korsakoff's psychosis: - Amnesia - Confabulation (made-up stories fill any gaps in memory)
What investigations are done for wernicke’s encephalopathy?
Clinical diagnosis
- Parenternal thiamine (improvement)
- Renal function
- FBC
- Electrolytes
- Glucose
- Ammonia
- LFTs
- Magnesium (Low)
