Neurological Flashcards
Spina Bifida
Occurs when a portion of the neural tube fails to close properly, typically in the lower back. This is a defect or absence of the vertebral arches due to a failure of the mesoderm to organise over the defect.
Can be Myelomeningocele (meninges and spinal cord), Meningocele (meninges), or Spina Bifida Occulta (no protrusion).
C: Unknown, folate deficiency, obesity, poorly controlled diabetes, medications that interfere with folate metabolism e.g. anti-seizure medication.
D: Prenatal: increased level of alpha fetoprotein in the mother’s serum, Blood tests: human chorionic gonadotropin, inhibin A, estriol, ultrasound, amniocentesis
T: Prenatal surgery to close myelomeningocele, postnatal surgery within days of birth, additional intervention - urinary catherization, wheelchair/crutches in the case of paralysis.
Microcephaly
Thebraindoes not develop properly, resulting in a shorter-than-normalhead.
C: Genetic mutations, external toxins during pregnancy (like alcohol), vertically transmitted infections.
S: Small head and a receding forehead, and a loose, often wrinkledscalp. Whole body is often underweight and dwarfed. Intellectual disability, poor motor function, poor speech, and seizures.
D: Clinical diagnosis, head circumferance, ultrasound in pregnancy, CT scan.
T: No cure. Supportive and symptomatic treatment. Treatment with amino acids may reduce seizures and motor function delays.
Macrocephaly
The human head is abnormally large; this includes thescalp, thecranialbone, and the contents of the cranium.
C: Megalencephaly(enlarged brain),hydrocephalus(abnormally increasedcerebrospinal fluid), cranialhyperostosis(bone overgrowth), genetic causes, infection, haematoma, subdural effusion and arachnoid cysts during pregnancy.
S: Developmental delay, a fast-growing head, autism, mental disorders.
D: CT, ultrasound, MRI
T: No need for treatment in benign familial macrocephaly.
Hydrocephalus
A build-up of fluid in the brain. The excess fluid puts pressure on the brain and can be fatal.
C: Congenital (spina bifida, mumps/rubella infection), acquired by illness or injury, normal pressure (develops in the elderly).
S: Congenital (learning disabilities, speech, memory, organisational, coordination and vision problem, short attention span, epilepsy). Acquired (vomiting, headache, blurred vision, difficulty walking), Normal pressure (dementia, mobility problems, urinary incontinence).
D: MRI and CT
T: Shunt to drain away excess fluid, endoscopic third ventriculostomy (ETV) to make a hole in the floor of the brain.
Bell’s palsy
Weakness or paralysis of the muscles on one side of the face due to damage to the facial nerve.
C: Unknown. Viral infections like HSV, EBV and VZV or the bacteria borrelia burgdorferi which causes Lyme disease.
S: Absence of the nasolabial fold, drooping of the eyelid and the mouth, dryness in affected eye or mouth, hypersensitivity to loud noises, loss of sensation to the anterior 2/3 of the tongue.
D: Clinical diagnosis. Serology for HSV-1 and VZV.
T: Lubricating eye drops and an eye patch for nightime, If present within 72 hours give prednisolone for 10 days, antivirals are controversial, referral to ENT if it doesn’t resolve.
Horner syndrome
Problem with the sympathetic nerve supply to one side of the face.
C: damage along the oculosympathetic pathway due to lung cancer, schwannoma, trauma, damage to aorta, surgery.
S: Results in miosis, ptosis, anhidrosis. The symptoms occur on the ipsilateral (same side) as the damaged nerve.
D: Eye drop test using cocaine or apraclonidine (no dilation)
T: Depends on underlying cause. Surgical intervention in the cause of syringomyelia, tumours or carotid artery dissection.
Motor neuron disease
Group of progressive neurodegenerative diseases, characterised by selective loss of neurones in the motor cortex, cranial nerve nuclei, and anterior horn cells (UMN and LMN).
C: Unknown. Smoking, genetics, age, heavy metal and pesticides might increase risk.
S: Progressive weakness of the muscles throughout the body, affecting the limbs, trunk and face, stumbling spastic gait, foot drop, weakened grip and shoulder abduction, dysarthria and dysphagia.
D: Clinical diagnosis, El Escorial diagnostic criteria,
MRI of the spinal cord can rule out structural causes; LP can exclude inflammatory causes.
T: No cure. Nutritional support, psychosocial support, physiotherapy/exercise programs, and occupational therapy. Riluzole extends survival. Symptomatic relief - opiates, antimuscarinic drugs for saliva, quinine for muscle cramps, Baclofen, tizanidine, dantrolene sodium or gabapentin can be used to treat symptoms of muscle stiffness, spasticity, or increased tone.
Multiple Sclerosis
Chronic, progressive, inflammatory condition that involves demyelination of myelinated neurones in the CNS.
C: Unknown. May be triggered by EBV, genetic changes, low vit D, smoking, obesity, environmental factors, SLE, T1DM, autoimmune thyroid disease, rheumatoid arthritis.
S: relapsing-remitting - optic neuritis, diplopia, hemianopia, ophthalmoplegia, Bell’s palsy, Horner’s syndrome, limb paralysis, incontinence, trigeminal neuralgia, paraesthesia, Lhermitte’s sign, loss of coordination, proprioceptive sense and cerebellar control, erectile dysfunction, positional vertigo, seizures.
D: Clinical diagnosis with McDonald criteria.
T: Lifestyle changes, Dimethyl fumarate (for mild/moderate RRMS), Monoclonal antibodies (alemtuzumab/natalizumab), baclofen/gabapentin and physiotherapy for spasticity, botulinum toxin A injections for tremor, self-catheterisation for urinary incontinence, CBT, mindfulness, exercise programs for fatigue, amitriptyline or gabapentin for neuropathic pain.
Conductive hearing loss
The movement of sound is blocked or does not pass into the inner ear due to a problem in the ear canal or middle ear.
C: earwax, foreign bodies, ottitis externa, ottitis media, perforated eardrum, cholesteatoma, otosclerosis, eustachian tube dysfunction.
S: Sudden or unexpected hearing loss in one or both ears, sound is muffled, pain, clear or yellow drainage.
D: Whispering in the patient’s ear, Weber’s test (if conductive, the sound will be heard best in the affected ear) Rinne’s test (bone conduction is better than air conduction, and the sound is not heard when the tuning fork is placed adjacent to the canal). Formal audiography.
T: Removing ear wax with ear drops, irrigation, microsuction. Surgery if congenital defect or tumours. Conventional hearing aid, bone-conduction hearing aid, or a surgically implanted, osseointegrated device. Antibiotic or antifungal medications are used to treat chronic ear infections, or chronic middle fluid.
Sensorineural hearing loss
The hair cells in the cochlea or the auditory nerve is not functioning correctly so some or all of the sounds are not being sent to the brain.
C: Age, noise damage, severe head injury, measles, mumps, meningitis, TB, VZV, Gentamycin, Meniere’s disease, MS, encephalitis, acoustic neuroma, stroke.
S: Can be mild, moderate or profound and affect one or both ears.
D: Whispering in the patient’s ear, Weber’s test (if sensorineural, the sound will be heard best in the unaffected ear) Rinne’s test (air conduction is better than bone conduction, so the sound is heard for longer when the tuning fork is placed adjacent to the canal). Formal audiography.
T: Cannot be treated with surgery or medicine. Conventional hearing aids or cochlear implants.
Cerebellopontine Angle Syndrome
A neurological syndrome of deficits due to the closeness of thecerebellopontine angleto specificcranial nerves.
C: Tumours within the nerve canaliculi or that extend into the CPA. Commonly a vestibular schwannoma affecting cranial nerve VII or a meningioma. The cause of the tumours is usually unknown.
S: Unilateral sensorineural hearing loss, unilateral tinnitus, disequilibrium, ataxia depending on how far it extends into the brainstem. Cranial nerve V, VII, VIII dysfunction.
D: Radiography, auditory brainstem response audiometry and adjunct tests, MRI
T: Acoustic neuromas are managed by either surgery, radiation therapy, or observation with regular MRI scanning (because most are benign, slow growing or non-growing, and non-invasive).
Jugular foramen syndrome
Characterised by paresis of theglossopharyngeal,vagal, andaccessory(with or without thehypoglossal) nerves.
C: Glomus tumours, meningiomas, schwannomas, metastatic tumours, trauma, fracture of occipital bone, infections, nasopharyngeal cancer.
S: Hoarseness, soft palate drooping, deviation of the uvula, decrease in theparotid glandsecretion, loss of gag reflex, sternocleidomastoidandtrapezius musclesparesis.
D: Gadolinium enhanced MRI for vestibular schwannoma, normal MRI, NCCT for occipital bone fracture.
T: Safe resection in schwannomas, meningiomas, non-infiltrative paragangliomas, and other benign tumours.
Subtotal removal may be needed to preserve cranial nerves, vascular structures and the brainstem.
Stereotactic radiosurgery if diameter is <3cm.
Postoperative radiotherapy to control residual disease.
Migraines
A severe headache felt as a throbbing pain at the front or side of the head. Lasts 4-72 hours.
C: Unknown what causes a period of abnormal brain activity. Genetics. Triggers include chocolate, hangovers, orgasms, caffeine/cheese, oral contraceptives, lie ins, alcohol, travel, exercise. Also periods, stress, tiredness, poor posture, bright lights, loud noises, strong smells.
S: Aura (flashing lights, chaotic distorting, pins and needles, dysarthia and ataxia, dysphasia or paraphasia), throbbing pain, aggravated by routine activities.
D: Clinical features. <15 days/month = episodic migraine. ≥15 days/month for >3 months = chronic migraine.
T: Avoid triggers. Warm/cold packs to the head, breathing in to a paper bag, acupuncture, lying in a dark room. Beta blocker (propranolol) or anti-epileptic (topiramate). Acute attack - an oral triptan with an NSAID or paracetamol. Review medication.
Parkinson’s disease
Progressive neurodegenerative disorder where voluntary movement is lost due to a loss of dopaminergic neurons in the substantia nigra.
C: Age, being male increases your risk, genetic predisposition.
S: ‘pill rolling tremor’ in the hands at rest, muscle stiffness, bradykinesia/akinesia, shuffling gait with reduced arm swing, hypomimia (decreased facial expresssion), micrographia, postural instability, cognitive impairment and memory problems, depression and sleep disturbances.
D: Clinical features and examination. DaTSCAN.
T: Levodopa – synthetic dopamine (reduced efficacy over time), given with peripheral decarboxylase inhibitors and COMT inhibitors (entacapone) to prevent breakdown. Dopamine agonists such as bromocriptine, pergolide, carbergoline. Monoamine Oxidase-B inhibitors such as selegiline and rasagiline.
Huntington’s disease
Rare neurodegenerative disease that involves a repeated sequence of 30-120 CAG causing the patient to experience abnormal movements and cognitive function.
C: Autosomal dominant. The mutated protein aggregates within the nerve cells of the caudate and the putamen of the basal ganglia causing neuronal cell death.
S: Chorea - purposeless ‘dance-like’ movement, Athetosis - slow ‘snake-like’ movements, abnormal eye movements, problems with coordination, dementia, personality changes, depression. Difficulty speaking, swallowing, breathing.
D: Genetic testing, clinical features, neurological examination, brain scan.
T: No cure (10-20yrs after diagnosis). Neuroleptics (dopamine receptor antagonists) and tetrabenazine (depletes dopamine) for chorea. Antidepressants and medication for irritability. Speech and language therapy, physiotherapy, occupational therapy, specialist equipment at home.
Ischaemic stroke
Blocked artery in the brain, extent of damage depends on the location and time.
C: Endothelial cell dysfunction leading to atherosclerosis which can become thrombogenic in brain artery OR embolism from another part of the body. Lacunar stroke occur in middle cerebral artery due to atherosclerosis. Watershed cerebral infarctions can be caused by shock.
S: ACA or MCA can cause numbness and sudden muscle weakness, Broca’s area - slurred speech, Wernicke’s area - difficulty understanding speech
PCA can cause problems with vision. Usually facial drooping, arm weakness, slurred speech.
D: CT/MRI, angiography, Flair sequence MRI to distinguish old and new stroke.
T: Lifestyle changes, carotid endarterectomy or stent to treat atherosclerosis, thrombolytic enzymes - tissue plasminogen activator activates the body’s naturally clot busting mechanism, Aspirin - prevents more clots forming, Surgery - physically remove the clot by mechanical embolus or suction removal.
Intracerebral haemorrhagic stroke
Bleeding into the cerebrum - just in the brain tissue (Intraparenchymal) or into the ventricular system too (Intraventricular).
C: Hypertension, arteriovenous malformations, vasculitis, vascular tumours, cerebral amyloid angiopathy, secondary to ischaemic stroke (haemorrhagic conversion).
S: Headache, nausea, and vomiting, Lethargy or confusion, Sudden weakness or numbness of the face, arm or leg, usually on one side, Loss of consciousness, Temporary loss of vision, Seizures
D: CT or MRI, Angiography to visualise the exact location
T: Anti-hypertensives
Surgery - Craniotomy: part of the skull is removed to drain any blood and relieve pressure
Stereotactic aspiration under CT can be done to aspirate off blood and relieve intracranial pressure
Subarachnoid haemorrhage
Bleeding between the arachnoid and pia mater layers, increasing intracranial pressure.
C: Traumatic injury, berry aneurysms usually in the anterior half of the circle of Willis, arteriovenous malformation.
S: A sudden severe headache unlike anything you’ve experienced before, a stiff neck, feeling and being sick, photophobia, blurred or double vision, stroke-like symptoms – such as slurred speech and weakness on one side of the body.
D: MRI or CT scan, Lumbar puncture - RBCs or yellow colour (xanthochromia)
T: Emergency surgery
- Clip artery - apply pressure
- Use catheter to insert coil to cause clot formation (closing up the aneurysm)
Calcium channel blockers to prevent vasospasm
Meningitis
Inflammation of the leptomeninges (arachnoid and pia mater).
C: Autoimmune disease, adverse reaction to medication e.g. intrathecal therapy (injected into the CSF), infection due to bacteria, virus, or fungi - usually Neisseria Meningitidis or Herpes simplex virus.
S: Neck stiffness (nuchal rigidity), headaches, fever, photophobia, phonophobia, meningoencephalitis can cause an altered mental state or seizures.
D: Kernig’s sign - positive when the thigh is flexed at the hip and knee at 90 degree angles, and subsequent extension in the knee is painful
Brudzinski’s sign - Severeneck stiffnesscauses a patient’s hips and knees to flex when the neck is flexed
Lumbar puncture, PCR for specific viruses.
T: Bacterial: administer steroids and then antibiotics (usually benzylpenicillin) to prevent injury to the leptomeninges
Antivirals, antibacterial, antifungals or antiparasitic can be used depending on the cause
Vaccine can be used against Neisseria Meningitidis or disseminated tuberculosis
Prophylactic antibiotics can be used to manage outbreaks of meningitis
Encephalitis
Acute inflammatory brain disease due to direct invasion/pathogen-initiated immune response.
C: Viral (HSV, arbovirus, enterovirus, VZV, EBV, HIV, influenza), bacteria (Listeria monocytogenes, mycobacteria, spirochetes), parasites (malaria, protozoea), cryptococcus fungi, acute disseminated encephalomyelitis, anti-N-methyl-D-aspartate (NMDA) receptor encephalitis, T-cell lymphoma.
S: Headache, fever, photophobia, neck stiffness, lethargy, irritability, seizures, confusion.
D: CT/MRI, lumbar puncture (CSF PCR and chemistry), bloods, blood glucose, Toxoplasma serology, brain biopsy, EEG.
T: Viral encephalitis
- HSV: Acyclovir
- CMV: Ganciclovir/Foscarnet
- Most viral infections lack specific antiviral agent
Bacterial encephalitis - targeted antibiotics
Tension headache
Primary headache that causing constant bilateral pain that can last from 30 mins to days.
C: Unknown but triggered by stress, squinting, poor posture, tiredness, dehydration, lack of physical activity, bright lights, loud noise, strong smells.
S: Dull, aching head pain, sensation of tightness or pressure across your forehead or on the sides and back of your head, tenderness on your scalp, neck and shoulder muscles.
D: Clinical features. <15 days/month = episodic headache. ≥15 days/month for >3 months = chronic headache.
T: Analgesia (NSAIDs, paracetamol), fluids, good sleep, heat/ice pack, good posture, acupuncture, avoid triggers, regular exercise, physiotherapy, Amitriptyline for chronic tension headaches.
Cerebellar disorders
Lesions causing cerebellar disorders usually present as severe gait and truncal ataxia, but there is a huge variety of causes of cerebellar disorders.
C: Stroke, TIA, space-occupying lesion, thiamine/Vit E/zinc deficiency, coeliac disease, infections caused by bacteria, viruses, parasites, prions, trauma, toxins, drugs, MS, genetic.
S: ipsilateral limb ataxia and tremor, fourth cranial nerve lesions, marked nystagmus, vertigo and vomiting, spluttering staccato speech, dysdiadochokinesia, emotional blunting, depression, disinhibition and psychotic features.
D: Blood tests: FBC, LFTs, cholesterol, protein electrophoresis, copper and caeruloplasmin, immunoglobulins and glycoproteins, EEG, EMG, MRI.
T: Depends on the underlying cause.
