Neurology Flashcards
Name 5 features of neuroleptic malignant syndrome?
Occurs just after start of treatment (first 10 days) Pyrexia Rigidity Elevated CK Renal failure Tachycardia
Tx with Bromocriptine or dantrolene
What is neuroleptic malignant syndrome
Rare SE of antipsychotics, mortality of around 10%
How is neuroleptic malignant syndrome managed?
Stop antipsychotic
IV fluids (prevent renal failure)
Dantrolene or bromocriptine
Neurofibromatosis and tuberous sclerosis are inherited via what pattern?
Autosomal dominant
Name 3 common features of neurofibromatosis T1?
Cafe au lait spots (>6 and around 1.5cm diameter) Axillary/ groin freckles Peripheral neurofibroma's Iris haematomas Scoliosis Pheochromocytomas
Name 2 features of neurofibromatosis T2?
Bilateral acoustic neuromas
Multiple intercranial schwannomas, meningiomas and ependymomas
What is the incidence of neurofibromatosis T1 and T2?
T1 = 1 in 4000 T2 = 1 in 100,000
So T1 is much more common!
What is the main differential when considering alternatives to carpal tunnel syndrome? How could you distinguish?
Degenerative cervical myelopathy (DCM)
- 50% are incorrectly initially diagnosed as CTS
Hoffman’s sign (flick middle finger, watch for flexion of index/ thumb = UMN lesion) can distinguish as CTS is LMN
Which gender is most affected by cluster headaches, name a risk factor and a common trigger?
M3:1F
Smoking is a big RF
Alcohol commonly triggers attacks
What drug is used as prophylaxis for cluster headaches?
Verapamil
What is the management of acute cluster headache?
100% oxygen
Subcut triptan
A 67-year-old man presents to his GP with numbness in his feet and recurrent falls over the past month. He describes difficulty walking. His GP recently started him on folate for a macrocytic anaemia. He takes no other regular medications. He does not drink alcohol. On examination, he has an ataxic gait and Romberg’s test is positive. Examination of his lower limbs demonstrates an increase in tone and bilateral weakness. He has absent ankle and knee jerks with upgoing plantars. Light touch and vibration sense is reduced bilaterally. MLD and cause?
Subacute combined degeneration of spinal cord
- Always replace vitamin B12 before folate - giving folate to a patient deficient in B12 can precipitate subacute combined degeneration of the cord
(Note this is not ALS or other MND as sensory involvement)
What is charcot-marie-tooth disease and how does it present?
A hereditary sensory and motor peripheral neuropathy
UMN signs are not present
Patients can present with lower motor neurone signs in all limbs and reduced sensation (more pronounced distally)
What is Lambert-Eaton myasthenic syndrome?
A rare autoimmune disorder involving the neuromuscular junction, associated with small-cell lung cancer.
- Similar to myasthenia gravis but movements IMPROVE with exercise, in MG they get WORSE with exercise
What is Cervical Spondylotic Myelopathy?
Cervical spondylosis is the term used for osteoarthritis of the spine and can result in compression of the spinal cord. This is more likely to result in LMN signs at the level of the compression (ie. upper limb if the lesion is below C5) with UMN signs below (in the lower limb). Patients usually complain of neck pain and stiffness.
What is first line treatment of trigeminal neuraligia?
Carbamazepine
When should a patient with trigeminal neuralgia be referred? (2)
Failure to respond to treatment or atypical features (e.g. < 50 years old) should prompt referral to neurology
Antipsychotics worsen the symptoms of which common neurological disease?
Parkinsons
What visual defect is seen with pituitary tumours?
Bitemporal hemianopia
Following head trauma, what is the quickest and easiest way to test fluid to determine if it is CSF?
Check glucose of the fluid
How long must a seizure last to be diagnosed as status epilepticus?
> 5mins
What visual defect would be caused by a parietal lobe lesion?
Contralateral inferior quadranopia
What visual defect would be caused by a temporal lobe lesion?
Contralateral superior quadranopia
What visual defect is caused by a pituitary tumour?
Lower bitemporal hemianopia
What medication is used to treat sleep paralysis?
Clonazepam
Which anti-emetic acts on D2 receptors and so can cause parkinsonian side effects?
Metoclopramide
What is the most common type of MND?
ALS
How does MND present?
Male >50 with FHx is typical presentation
Presents with progressive weakness of limb/ thoracic/ abdo muscles
- UL tends to be first = dropping objects, wrist drop, fasiculations etc
Name some common signs seen in MND?
Often asymetrical
UMN (brisk reflexes) with LMN (wasting) signs
No sensory or pain symptoms
What is the only drug proven to have disease modifying abilities in MND?
Riluzole (a neuroprotective glutamate-release inhibitor) is the only drug of proven disease-modifying efficacy
A patient with MND is struggling with drooling, name a drug tx which could improve this?
Hyoscine
A patient with MND is suffering with muscle cramps, name 4 medications which could be trialled to relieve these symptoms?
Diazepam Baclofen Tinzanidine Phenytoin Quinine
What is the pathophysicological cause of Myasthenia Gravis?
IgG1 Antibodies to acetylcholine receptors
How does myasthenia gravis commonly present?
Muscles fatigued after exercise
- Eyes often first (some can remain just eyes for years)
- Proximal muscles tend to be most affected
What is a myasthenic crisis?
Affects 25% MG px, often in first year. Can be first presentation.
Complication where muscle weakness results in respiratory failure and a need for mechanical ventilation
(Tx = intravenous immunoglobulin, plasma electrophoresis)
What signs would be seen on neurological exam in myasthenia gravis?
Weakness (especially after fatigue)
- Normal tone
- Normal sensation
- Normal reflexes
- No muscle wasting or fasiculation
What is the first investigation that should be performed for myasthenia gravis if a patient has ptosis?
Crushed ice in a latex glove is applied to the eye for three minutes. In MG this leads to improvement of ptosis and it has a sensitivity and specificity of over 90%
What is the first investigation that should be performed for myasthenia gravis?
Serum anti-acetylcholine receptor (ACh-R) antibody testing is the first-line investigation for non-urgent patients.
What is the mainstay of treatment for MG?
Pyridostigmine
(Acetylchlinesterase inhibitor)
Immunosuppresion: Steroids, azathioprine
What is the most common form of MS?
Relapsing-remitting (80%)
Also can have primary progressive (10%) or secondary progressive (follows R-R about 50% within first 10yrs)
What are the RF’s for MS?
About 2% of those who have a first degree relative will develop
More common in caucasian, northern latitude
What criteria are needed to diagnose MS?
Dissemination in time AND space (doesn’t NEED MRI for diagnosis)
Name 5 common presenting symptoms of MS?
Optic neuritis/ blindness etc Disorder of eye movement (jerking nystagmus) Facial weakness (Bell's palsy) Deafness Transverse myelitis
What investigations should be done for MS before neurology referral?
Before referring to a neurologist, exclude differential diagnoses by checking FBC, Inflammatory markers, U&E, LFT, TFT, glucose, HIV serology, calcium and B12 levels.
What should be the first line investigation for MS?
Visual evoked potentials
Later followed by MRI
How should an MS relapse be treated?
Oral/ IV methylprednisolone (with gastric protection) - 5 days
What DMARDS are used in MS?
Interferon beta (can give flu like symptoms for 24hours after injection - used ibuprofen) (Needs 2 relapses in last 2 years + able to walk 100m unaided)
Glatrimer (needs at least 2 relapses in last 2 years)
Dimethyl fumarate/ teriflumomide/ alemtuzumab
On neuro exam, what would be found in an MS patient for the 5 neuro domains?
Inspection
(Walking aids, although MS is UMN may have wasting in late disease)
Tone - Increased (UMN)
Power - Weakness
Reflexes- Brisk (UMN)
Co-ordination- Possibly impaired
Gait- Spastic gait suggests UMN, ataxic gait suggests cerebellar
What is guillian barre syndrome?
A disorder which causes demyelination and degeneration causing progressive neuropathy -= weakness, paraesthesia and hyporeflexia
Many subypes but 95% are AIDP
What are the RF’s of Guillan Barre syndrome?
Hx of GI/ resp infection in preceeding weeks
Recent vaccination
Associated with lymphoma and post-partum
What is a common presentation of guillian barre syndrome?
Ascending, progressive, symetrical weakness starting in LL, 3 weeks after viral infection
Max severity after 2 weeks, stops progressing after 5
Possible neuropathic pain, paraesthia and sensory loss
What signs would be seen on a neuro exam for GBS?
I: T: Low (LMN) P: Weakness in affected areas (LMN) R: Reduced or absent (LMN) C: Variable S: Altered sensation
What would be shown on an GBS lumbar puncture?
Very high level of protein with no elevation in CSF cell counts
May not be seen until 1-2 weeks after weakeness onset
How is GBS managed?
IV IG
or Plasma exchange
+/- Steroids
+ DVT prophylactic stockings
What is the prognosis for a patient with GBS?
80% recover in 6 months
10% neuro deficit
10% severely disabled
How does TN commonly present?
Sudden, unilateral, brief, stabbing, episodic, recurrent pain in one or more branches of 5th nerve
Pain in paroxysms from secs-2mins
Possible preceding syx, tingling/ numbness
Name 5 common triggers for TN attacks
Vibration Contact (washing/ shaving) Brushing teeth Oral intake Exposure to wind
What red flags must you look out for with TN?
Sensory changes
FHx of MS
Age <40yrs
Opthalmic divison only (suggestive of MS)
Name 3 differentials for TN?
Dental problems TMJ dysfunction Migraine Temporal arteritis (TN rarely affects foreheard alone) MS
How should TN be investigated?
Referral and MRI only for uncertain diagnosis or red flags
What is the mainstay of tx for TN?
Lifestyle advice
Carbamazepine (until remission for one month)
Refer to pain clinic
Surgical options increasinly promising
How do you distingush between Bells Palsy and possible stroke?
Bells Palsy is 7th nerve so LMN = Forehead is affected
Stroke is UMN as bilaterally inervated = Forehead NOT affected
Name the two biggest RF’s for Bell’s palsy:
Pregnancy
Diabetes
Most are idopathic, now thought to be viral (herpes/ varicella)
(Commonly 15-60yrs)
What is the optimum treatment of Bell’s palsy?
Steroids (Pred for 10days then reducing)
Eye care etc
Refer if doubt, recurrent or bilateral (ENT or neuro)
A 72 year old female presents with progressive immobility, poor balance and a resting tremor of the L hand. She has non-velocity dependant hypertonia of all 4 limbs, dysarthria and difficulties moving her eyes in the superior plane. MLD?
Progressive surapnuclear palsy
PD features (rest tremour, rigidity, bradykinesia, instability) etc. but other features (eye movements and dysarthria) make the PD plus disorder PSP most likely
Name 6 features of cerebellar disease?
D- Dysdiadochokinesia A- Ataxia N - Nystagmus I- Intention tremor S- Slurred stacato speech H- Hypotonia