Neurology: Other Common Conditions Flashcards
What 3 presentations does diabetic foot disease include?
- Diabetic neuropathy
- Ischaemia e.g. absent pulses, reduced ABPI, intermittent claudication
- Complications e.g. calluses, ulceration, Charcot’s arthropathy, osteomyelitis, gangrene
*See Yr3 medicine endocrinology FC for more
Describe the typical presentation of peripheral diabetic neuropathy
- Typically sensory loss (motor loss to lesser extent)
- Glove & stocking distribution affecting lower legs first
- Neuropathic pain
Discuss the management of peripheral diabetic neuropathy
- Improve glycaemic control (diet, medications)
- Neuropathic pain medications:
- First line: amitriptyline, duloxetine, gabapentin, pregabalin
- Second line: try one of others above
- Topical capsaicin for those who don’t want or tolerate oral treatments with localised neuropathic pain
- Education about diabetes and diabetic neuropathy
- Advise to check feet regularly
- Annual screening
Patients with diabetes can also get autonomic neuropathy, state some symptoms associated with this (categorise based on organ system affected)
- GI symptoms: metoclopramide, domperidone, erythromycin (prokinetic)
- Gustatory sweating: antimuscarinics
- Erectile dysfunction: phosphodiesterase-5 inhibitors
For Brown-Sequard syndrome, discuss:
- What it is
- Tracts affected
- Presentation
- Injury to half of spine (hemi section of spinal cord)
- Tracts affected on one side:
- Lateral corticospinal tract
- Dorsal columns
- Lateral spinothalamic tract
- Presentation:
- Ipsilateral motor loss at and below lesion
- Ipsilateral segmental anaesthesia
- Ipsilateral loss of DC modalities (proprioception, vibration, fine touch, two point discrimination) below lesion
- Contralateral loss of ST modalities (crude touch, pain, temperature) below lesion
For subacute combined degeneration of the spinal cord, discuss:
- What it is
- Tracts affected
- Presentation/clinical features
- A neurological complication of vitamin B12 deficiency. It is characterized by degeneration of the posterior and lateral spinal cord due to demyelination
-
Tracts affected:
- Dorsal columns
- Lateral corticospinal tracts
- Spinocerebellar tracts
- Presentation/features:
- Bilateral motor weakness/spastic paresis
- Bilateral loss of DC modalities (proprioception, vibration, fine touch, two point discrimination)
- Bilateral limb ataxia
For syringomyelia, discuss:
- What it is
- Tracts affected
- Presentation/clinical features
- Fluid filled cyst forms on spinal cord. Usually occurs in cervicothoracic cord
- Tracts affected depend where the cyst is, but if near central canal then:
- Lateral spinothalamic
- Ventral horns
- Presentation/clinical features:
- Loss of pain & proprioception in cape like distribution
- Flacid paresis (typically in hands)
For anterior spinal artery occlusion, discuss:
- What it is
- Tracts affected (main ones for you to be aware of)
- Presentation/clinical features
- Occlusion of anterior spinal artery which supplies anterior ⅔ of spinal cord leading to damage to parts of cord supplied by this artery
- Tracts affected:
- Lateral corticospinal
- Lateral spinothalamic
- Presentation/clinical features:
- Muscle weakness/spastic paresis
- Bilateral loss of ST modalities (pain, temp, crude touch)
Which part of spinal cord does neurosyphilis affect?
Dorsal columns
Loss of proprioception, vibration, fine touch & 2 point discrimination
Define radiculopathy
Conduction block in axons of spinal nerve or it its root; results in weakness (when impacts of motor neurones) and parasthesia or anaesthesia (when impacts on sensory neurones)
Define radicular pain
Radicular pain= pain deriving from damage or irritation of the spinal nerve tissue- particularly dorsal root ganglion
*NOTE: different from radiculopathy wich can be thought of as a state of neurologial loss and may or may not be associated with radicular pain
Define myelopathy
Myelopathy is neurological signs & symptoms due to pathology of the spinal cord
*NOTE: must be compressing spinal cord e.g. therefore not cauda equina
State some potential causes of radiculopathy
Most commonly a result of nerve compression which can be due to:
- Intervertebral disc prolapse (lumbar spine most common)
- Degenerative diseases of spine which lead to neuroforaminal or spinal canal stenosis (cervical spine most common as it is most mobile)
- Fracture (trauma or pathological)
- Malignancy (most commonly metastatic)
- Infection (e.g. extradural abscesses, osteomyelitis, herpes zoster)
Describe clinical features of radiculopathy
- Sensory features: parasthesia, numbness
- Motor features: weakness
- Radicular pain (deep, burning, strap like pain. Can be intermittent)
- Red flag symptoms
Discuss the general principles of the management of radiculopathy
- Depends on underlying cause.
- Main one to identify quickly/rule out is CES as it requires emergency surgical treatment.
For cervical spondylosis, discuss:
- What it is
- Presentation
- Managment
- Cervical spondylosis is an age-related degeneration (‘wear and tear’) of the bones (vertebrae) and discs in the neck.
- Presentation:
- Neck pain
- Neck stiffness
- Headaches
- Pins and needles
- Clumsiness of hands/difficulty fine movements of hands
- Management:
- Analgesia (paracetamol, NSAIDs, weak opioids e.g. codeine, neuropathic pain meds)
- Physiotherapy
- Advise to use firm supportive pillow when sleeping
Remind yourself of the following for facial nerve:
- Path
- Function
*
- Exits brainstem at cerebellopontine angle, travels through temporal bone and parotid gland. Then divides into 5 branches: temporal, zygomatic, buccal, marginal mandibular, cervical
- Function:
- Motor: muscles of facial expression, stapedius, posterior digastric, stylohyoid and platysma
- Sensory: taste anterior ⅔ tongue
- Parasympathetic: submandibular, sublingual and lacrimal glands
State some potential causes of facial nerve palsy (think about UMN &LMN causes)
UMN
- Stroke
- Tumours
- MND (would cause bilateral palsy- rare)
LMN
- Bell’s palsy
- Ramsey-Hunt syndrome
- Infection:
- Otitis media
- Malignant otitis media
- Systemic disease
- MS
- Guillian-Barre syndrome
- Diabetes
- Tumours
- Acoustic neuroma
- Parotid tumourr
- Cholesteatoma
- Trauma
- Direct
- Damage in surgery
- Base of skull fractures
For Bell’s palsy, discuss:
- What it is
- Presentation
- Management
- Prognosis
- Acute, unilateral, idiopathic, facial nerve paralysis
- Presentation:
- LMN lesion (forehead involved)
- Altered taste
- Dry eyes
- Hyperacusis
- Management:
- If present within 72hrs of symptoms, give oral corticosteroids
- 50mg prednisolone for 10 days
- Or 60mg for 5 days followed by 5-day reducing regime of 10mg per day
- Lubricating eye drops
- Tape eye closed at bedtime
- Advised that if they develop pain in eye need to go to eye casualty as may be exposure keratopathy
- If the paralysis shows no sign of improvement after 3 weeks, refer urgently to ENT
- If present within 72hrs of symptoms, give oral corticosteroids
- Prognosis:
- Majority recover within 3-4 months
- 15-30% left with some residual weakness
Describe the typical presentation of carpal tunnel syndrome
- Pain, numbness, parasthesia in median nerve sensory distribution (NOTE: palm often spared)
- Symptoms worse at night
- Symptoms relieved by handing arm over side of bed or shaking
- Weakness of thumb abduction & wasting of thenar eminence
- Positive Tinel’s test or positive Phalen’s test
What is carpal tunnel syndrome?
Symptoms due to compression of median nerve within the carpal tunnel
State some risk factors for carpal tunnel syndrome
- Female
- Age (45-60yrs)
- Pregnancy
- Obesity
- Previous injury to wrist
- Repetitive hand or wrist movements
- Hypothyroid
- RA
- Diabetes
What investigations may be done for suspected CTS?
- Diagnosis= clinical
- May do nerve conduction studies to confirm emdaiin nerve damage (but normal NCS does not rule out CTS)
Discuss the management of carpal tunnel syndrome
Conservative
- Wrist splint (worn at night)
- Physiotherapy
- Corticosteroid injections into carpal tunnel
Surgical
- Carpal tunnel release surgery to decompress carpal tunnel (cut through flexor retinaculum to reduce pressure on median nerve)
State some potential complications of carpal tunnel release surgery
- Persistent CTS symptoms
- Infection
- Scar formation
- Nerve damage
- Trigger thumb
(90% report good outcomes post-surgery)
Explain why the palm is often spared in carpal tunnel syndrome
Palmar cutenaous branch of median nerve branches proximal to flexor retinaculum and passes over carpal tunnel
What is tuberous sclerosis?
Genetic condition in which hamartomas- benign neoplastic growths of the tissue that they originate from- develop in numerous body systems such as: skin, brain, lungs, heart, kidneys, eyes etc…
What is inheritance pattern of tuberous sclerosis?
What genetic defects are present in tuberous sclerosis?
- Autosomal dominant
- Caused by mutation in 1 of 2 genes:
- TSC1 gene (chromosome 9): codes for hamartin
- TSC2 gene (chromosome 16): codes for tuberin
Hamartin & tuberin interact with each other to control the size & growth of cells; hence, abnormalities in either of these genes causes abnormal cell size & growth
Outline the classical presentation of tuberous sclerosis
Child presenting with epilepsy found to have skin features of tuberous sclerosis (but can also present in adulthood)
Skin Signs
- Ash leaf spots are depigmented areas of skin shaped like an ash leaf
- Shagreen patches are thickened, dimpled, pigmented patches of skin
- Angiofibromas are small skin coloured or pigmented papules that occur over the nose and cheeks
- Subungual fibromata are fibromas growing from the nail bed. They are usually circular painless lumps that grow slowly and displace the nail
- Cafe-au-lait spots are light brown “coffee and milk” coloured flat pigmented lesions on the skin
- Poliosis is an isolated patch of white hair on the head, eyebrows, eyelashes or beard
Neurological Features
- Epilepsy
- Learning disability and developmental delay
Other Features
- Rhabdomyomas in the heart
- Gliomas (tumours of the brain and spinal cord)
- Polycystic kidneys
- Lymphangioleiomyomatosis (abnormal growth in smooth muscle cells, often affecting the lungs)
- Retinal hamartomas
Management of tuberous sclerosis is supportive primarily focused on treating complications; true or false?
True
What is neurofibromatosis?
Genetic condition that causes benign neuromas (nerve cell tumours) to develop throughout the nervous system; although tumours are benign, they cause neurological & structural problems.
There are two types of neurofibromatosis; type 1 and type 2. Which is more common?
Neurofibromatosis type 1
For neurofibromatosis type 1, discuss:
- Inheritance pattern
- Gene mutation
- Features/diagnostic criteria
**HINT: crabbing
- Autosomal dominant
- NF1 gene on chromosome 17 (codes for neurofibromin which is tumour suppressor protein)
- Features/diagnostic criteria is at least 2 of the 7 features:
- C- cafe-au-lait spots (6 or more, ≥5mm in children, ≥15mm adults)
- R- relative with NF1
- A- axillary or inguinal freckles
- BB- bony dysplasia (e.g. bowing of long bone, sphenoid wing dysplasia)
- I- Irish hamartomas/Lisch nodules (yellow spots on iris) (2 or more)
- N- Neurofibromas (2 or more) or 1 plexiform neurofibroma
- G- glioma of optic nerve
Discuss the management of neurofibromatosis type 1
NOTE: no investigations are required to make diagnosis
- No treatment to slow disease progress
- Management is therefore based on symptom control, monitoring the disease and treating complications
State some complications of neurofibromatosis type 1
- Migraines
- Epilepsy
- Renal artery stenosis causing hypertension
- Learning and behavioural problems (e.g. ADHD)
- Scoliosis of the spine
- Vision loss (secondary to optic nerve gliomas)
- Malignant peripheral nerve sheath tumours
- Gastrointestinal stromal tumour (a type of sarcoma)
- Brain tumours
- Spinal cord tumours with associated neurology (e.g. paraplegia)
- Increased risk of cancer (e.g. breast cancer)
- Leukaemia
For neurofibromatosis type 2, discuss:
- Inheritance pattern
- Mutation
- Features
- Autosomal dominant
- Mutation in NF2 gene on chromosome 22; leads to mutation in merlin (a tumour suppressor protein important in Schwann cells)
- Features:
-
Acoustic neuromas (bilateral acoustic neuromas pretty much pathognomonic for NF2)
- Hearing loss
- Tinnitus
- Balance problems
- Can also get other tumours developing elsewhere
-
Acoustic neuromas (bilateral acoustic neuromas pretty much pathognomonic for NF2)
- Management: surgery can be done to resect acoustic neuromas with subsequent brainstem implants
State some types of brain tumour
- Secondary metastases (e.g. lung [most common], breast, renal, melanoma)
-
Gliomas
- Astrocytoma (least malignant)
- Oligodendroglioma
- Ependymoma (most malignant)
- Meningioma
- Pituitary tumours
- Acoustic neuromas
Meningiomas are usually benign; true or false?
True
Broadly outline possible management options for brain tumours
Given brain tumours can be benign to extremely malignant management varies; options include:
- Radiotherapy
- Chemotherapy
- Surgery
- Palliative care
Pituitary tumours can be managed with:
- Trans-sphenoidal surgery
- Radiotherapy
- Bromocriptine (if prolactin secreting tumour)
- Somatostatin (if growth hormone secreting tumour)
Remind yourself of some causes of neuropathic pain
- Postherpetic neuralgia
- Nerve damage from surgery
- Multiple sclerosis
- Diabetic neuralgia
- Trigeminal neuralgia
- Complex Regional Pain Syndrome
Remind yourself of features of neuropathic pain
- Burning
- Tingling
- Pins and needles
- Electric shocks
- Loss of sensation to touch of the affected area
What questionnaire can be used to estimate probability of neuropathic pain?
DN4 questionniare
State some potential treatment options for neuropathic pain, including:
- First line medications recommended by NICE
- Other options
First line medications recommended by NICE
- Amitriptyline (TCA)
- Duloxetine (SNRI)
- Gabapentin (anticonvulsant)
- Pregabalin (anticonvulsant)
Other options
- Tramadol ONLY FOR RESCUE USE/short term control flares
- Capsaicin cream
- Physiotherapy
- Psychological input
- Certain conditions have NICE recommended management e.g. trigeminal neuraliga → carbamezapine
What is complex regional pain syndrome?
Umbrella term for number of conditions in which abnormal nerve functioning causes neuropathic pain and other abnormal sensations; usually triggered by injury to an area
State some features of complex regional pain syndrome
- Neuropathic pain
- Allodynia
- Abnormal hair growth
- Oedema
- Sweating
- Temperature changes
- Skin colour changes
- Motor dysfunction
*Symptoms of oedema, sweating, temp an colour changes can be intermittent
Discuss the management of complex regional pain syndrome
Managed by specialists (pain team):
- Neuropathic pain meds
- Early physiotherapy
What are the rules regarding driving for the following conditions:
- Craniotomy
- Pituitary tumour
- Narcolepsy/cataplexy
- Chronic neurological conditions e.g. MS, MND
- craniotomy e.g. For meningioma: 1 year off driving*
- pituitary tumour: craniotomy: 6 months; trans-sphenoidal surgery ‘can drive when there is no debarring residual impairment likely to affect safe driving’
- narcolepsy/cataplexy: cease driving on diagnosis, can restart once ‘satisfactory control of symptoms’
- chronic neurological disorders e.g. multiple sclerosis, motor neuron disease: DVLA should be informed, complete PK1 form (application for driving licence holders state of health)
