Case 7: MS, Parkinsons, spinal cord compression Flashcards
MS: managing symptoms
- Fatigue:can be overwhelming and disabling: Cooling, Pacing activities, CBT, Mindfullness, Amantadine
- Mood:Depression is common in MS: CBT, SSRIs, Duloxetine
- Cognition:Cognition deteriorates as disease becomes advanced: Social support, rule out sleep issues/pain/depression
- Spasticity:This can lead to pain and discomfort: Physiotherapy, Baclofen, BoTox
- Pain:Typically neuropathic type pain: CBT, Amitriptyline, Gabapentin, Pregabalin
- Neurogenic bladder issues:Urinary frequency, urgency, nocturia. Frequent UTIs: Fluid intake control, regimented toilet regime, Oxybutinin, BoTox injection, intermittent self-catherterisation
- Constipation:Difficulty opening bowels, or mobilising to toilet: Good diet and fluid, regular laxatives, bowel care, assistedevacuation, good hygiene
Parkinson’s symptoms: motor
- Slowness
- Gait alteration (arm-swing less on affected side, shuffle, reduced stride length)
- Loss of dexterity
- Resting tremor ( pill rolling coarse, 4Hz, re-emergent)
- Postural alteration (antecollis, Pisa- lean to one side, camptocormia- bent double).Reduced arm swing and hunched appearance
- Falls and imbalance
Parkinson’s symptoms: non-motor
- Depression/anxiety
- Constipation, urinary symptoms, postural hypotension
- Shoulder pain
- REM behaviour disorder: move a lot in sleep, can occur years before motor symptoms
- Restless leg syndrome/Periodic leg movement in sleep
- Hypersalivation
- Subjective memory issues
- Anosmia: often present years before diagnosis
- Parkinson’s Disease Dementia seen in patients with motor symptoms for 10+ years. Causes afluctuatingcognitive deficit, impaired visuospatial function and frequent visual hallucinations as well as memory problems.
Parkinson’s medication
- CDMT inhibitor: preserve levodopa and dopamine
- Levodopa: replaces dopamine
- Dopamine agonists i.e. ropinirole: mimic dopamine. Side effects lack of impulse control. Can be given by transdermal patch
- MAO-B inhibitors i.e. Rasigilline: preserve existing dopamine
- Non-oral: Apomorphine infusion (levodopa is continuously released under the skin) , deep brain stimulation, Duodopa (PEG tube which releases levodopa continuously- best for fluctuating symptoms), Cueing and physiotherapy
- Combine Levodopa with carbidopa pr benserazide creating co-careldopa and co-beneldopa
Names of Parkinson’s medications
- Levodopa (+ Carbidopa/Benserazide)= Madopar, Sinemet
- Dopamine Agonist= Rotigotine, Ropinirole, Pramipexole (given for restless leg)
- Stronger dopamine agonist: Apomorphine- given by SC infusion
- MAO-B inhibitors = Rasagiline, Seligiline
- COMT inhibitors= Entacapone, Epicapone
Cause of Parkinson’s
Caused by loss of dopaminergic neurones in the substantia nigra in the basal ganglia. The basal ganglia is essential for coordinating movement. Due to accumulation of alpha synuclein in the form of ‘lewy bodies.’
Braak stages in Parkinson’s
- Braak stage 1 and 2: autonomic and olfactory disturbances
- Braak stages 3 and 4: sleep and motor disturbances
- Braak stage 5 and 6: emotional and cognitive disturbances
Symptoms of Parkinson’s
- Bradykinesia- slow movement
- Tremor- rest, postural
- Rigiditity- cog wheeling, lead pipe
- Postural instability- late feature
- Early features- loss of sense of smell, REM sleep behaviour disorder, constipation, depression and anxiety
- Late complications- bladder and blood pressure problems, pyschosis and dementia
Differentials for Parkinson’s
- Essential tremor
- Drug induced parkinsonism
- Dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration
- Alzheimer’s and multiple cerebral infarctions
Clinical examination in Parkinson- gait
- Stooped posture
- Forward flexion of the trunk and the knees
- Arms flexed at the elbows and wrists
- Known as shuffling gait due to small and fast steps
- Difficulty initiating turning around
Clinical examination in Parkinson’s- tremor
- Resting tremor
- Pill rolling, looks like the patient is trying to roll a pill between their thumb and index finger
- 4-6Hz
- Asymmetrical, worse at rest
- Improves with movement
- No change with alcohol
UK Parkinsons disease society brain bank clinical diagnostic criteria
Step 1- diagnosis of Parkinsonian syndrome
- Bradykinesia
- At least one of muscle rigidity, resting tremor, postural instability
Step 2- exclusion criteria for Parkinson’s disease:
- History of strokes/ head injury/ encephalitis
- Oculogyric crisis
- Neuroleptic treatment at onset of symptoms
- Sustained remission
- Cerebrallar signs
- Cerebral tumour
Step 3- supportive positive criteria for Parkinson’s disease
What is the supportive positive criteria for Parkinsons
- Three or more are required alongside step one
- Unilateral onset
- Resting tremor present
- Disease is progressive
- Good response to levodopa
Parkinson’s- investigations
DAT scan- less dye is taken up. Reduced uptake in the substantia nigra, particularly in the posterior part of the putamen. Motor symptoms begin in stage 4
MRI/CT- to rule out other things
Bedside- physical examination, anosmia testing
Bloods
Management plan in Parkinsons
First line treatment- levodopa. Addition of a dopamine agonist/ MAO-B inhibitor as an adjunct if motor symptoms are not controlled
Supportive therapies- include physiotherapy/ occupational therapy/ speech and language therapy/ diet advice
Can give deep brain stimulation: surgical option, works best for dopamine responsive tremor
Levodopa
Usually combined with carbidopa to prolong action (peripheral inhibitor of dopamine metabolism). Side effects include dyskinesia when doses are high, impulsive behaviour, NV, loss of appetite, hypotension. Becomes less effective over time
Dyskinesia with levodopa
- Excessive motor activity when the dose is too high
- Dystonia - excessive muscle contraction leading to abnormal postures and movements
- Chorea - abnormal involuntary movements that are jerky and random
- Athetoid - involuntary twisting or writhing movements typically of the fingers / feet / hands
As the levodopa wears off
- Occurs as more dopaminergic cells die off- tend to be 5 years after treatment
- Delayed ‘on’ - where patients have to wait longer for dopamine levels to rise following their medication
- Wearing ‘off’ - where symptoms return earlier when the next dose is due
Dopamine agonists
- Stimulate dopamine receptors in the basal ganglia
- Side effects include pulmonary fibrosis / dizziness / drowsiness / tachycardia / dry mouth / NV / memory, concentration and confusion problems
- Can cause: excessive daytime somnolence, postural hypotension and visual hallucinations
- Usually used to delay the use of levodopa and then used in combination with levodopa to reduce the dose needed
- They increase impulsivity, shows as disinhibited behaviours i.e. gambling and hypersexuality
- g. pramipexole, ropinirole, bromocriptine
Monoamine oxidase B inhibitors
- These block the affects of monoamine oxidase B (an enzyme which breaks down dopamine)
- Used to delay the use of levodopa and used to reduce the required dose
- For example, Selegiline, Rasagiline
COMT inhibitors
- COMT metabolises levodopa, the inhibitors slow this down and extends the effect of levodopa
- For example, Tolcapone, entacapone
Multiple system atrophy
- Progressive degeneration of the neurones in multiple brain areas e.g. basal ganglia, cerebellum, autonomic and peripheral nervous system
- Basal ganglia degeneration leads to parkinsonian presentation but there are also other symptoms e.g. a lot of autonomic dysfunction causing hypotension/incontinence/impotence/sexual dysfunction etc
- Also profound cerebellar dysfunction
- Parkinsons with autonomic disturbance e.g. atomic bladder/postural hypotension points towards MSA
Progressive supranuclear palsy
- Rapidly progressive neurological disease characterised by accumulation of tau protein in basal ganglia, brainstem, prefrontal cortex, and cerebellum
- Symptoms include difficulty moving eyes, mood changes, dysphagia, backwards falls, slurred speech, memory loss, apraxia, resting tremor
- Involves psudeobulbar palsy (dysarthria and dysphagia)
- Bradykinesia, Backwards falls, Slow vertical saccades, Loss of a vertical gaze
Dementia with Lewy bodies
- Dementia associated with features of Parkinson’s disease - the parkinsonian features develop after the memory loss
- Other features = visual hallucinations / delusions / disorders of REM sleep / fluctuating consciousness
Neuroepileptic malignant syndrome
Life threatening muscle rigidity, fever and rhabdomyolysis in response to antipsychotics
Signs and symptoms of spinal cord compression
- Can start with back pain
- Patients will have approximately symmetrical weakness below the spinal level of compression and reduced sensation below this level
- There may be asensory levelthat can be detected andfocal spinal tenderness
- There may be Lower Motor Neurone signs at the level of lesion butUpper Motor Neuronesigns below the level
Red fags with back pain
- Sciatica (pain radiating down back of leg)
- Leg weakness
- Saddle paraesthesia (numbness or changed sensation to the perineum)
- Urinary symptoms (difficulty initiating urination, reduced sensation of urination, urinary incontinence)
- Bowel symptoms (faecal incontinence)
- Recent trauma
- Fevers/Night sweats
- Weight loss
- Pain that disturbs sleep
- History of intravenous drug use
- Osteoporosis
- Prolonged use of steroids (raises risk of osteoporosis)
Cauda equina pathophysiology
- the spinal cord (UMN) ends at L1/L2 in adults at the conus medullaris. Below this the nerves are peripheral and exhibit LMN signs (cauda equina)
- Causes: Disk herniation (most common), Haematoma, Malignancy, Spinal abscess, Stenosis, vertebral fracture
Cauda equina presentation
- Urinary symptoms (incontinence, reduced desire to void urine and reduced ability to pass urine), loss of perianal sensation
- Lower motor neurone problems (lower limb weakness, hyporeflexia and down going plantars)
Cauda equina examination
- Digital rectal examination: reduced anal tone and peri-anal sensation
- Post void bladder scan: urinary retention
- MRI spine: gold standard- would show compression at L1/L2 where the cauda equina starts
Suspected cauda equina syndrome
Any of the following - bilateral sciatica pain, leg weakness, saddle paraesthesia, bladder disturbance, bowel disturbance.
Complete cauda equina syndrome
Urinary retention is present. Overflow urinary incontinence may be present. Loss of anal tone and peri-anal sensation. Faecal incontinence is a sign of advanced disease. Saddle paraesthesia is more widespread and complete.
Incomplete cauda equina syndrome
Urinary retention is not present. Subjective reduced urination sensation, difficulty passing urine, poor stream, reduced urge to void bladder. Anal tone and peri-anal sensation might be normal. Saddle anaesthesia may be present in a more patchy/unilateral way.
Cauda equina management
- Refer to Neurosurgery team- may require emergency surgical decompression. Surgery should happen within 48 hours of presentation
- High dose steroids to reduce swelling around site of nerve compression
- If disk herniation: laminectomy or discetomy
- Adequate analgesia for pain control
- Blood tests (including Group and Save and Coagulation) to prepare for surgery
- Urethral catheter if in urinary retention or difficulty passing urine
- Check if patient needs to be Nil By Mouth for surgery
Cauda equina long term complications
Pain, reduced motor function, bladder/bowel disturbance and sexual dysfunction
Peripheral nervous system (PNS) disease and LMN lesions
Peripheral nervous system (PNS) disease- The sensory symptoms are symmetrical, or in the territory of a sensory dermatome or the cutaneous distribution of a peripheral nerve.
Lower motor neurone lesions- atrophy, fasciculations, reduced tone, diminished reflexes
Ascending peripheral polyneuropathy
- Symptoms started distally and progressed proximally
- LMN signs
- Affecting motor and sensory systems
Guillain Barre syndrome
- Acute demyelinating polyneuropathy
- May be post infectious- camylobacter gastroenteritis, viral
Lesion vocabulary
- Plegia- severe weakness, complete/ nearly complete
- Paresis- mild weakness
- Para- both legs
- Hemi- one half of the body
- Quadri- all 4 limbs
Upper motor neurone lesion
- No atrophy or fasciculations
- Spastic tone
- Brisk reflexes
Cervical cord compression
- Bilateral UMN signs (brisk reflexes and increased tone) below the level of the lesion
- Slowly progressive spastic parapesis
Damage to different cervical roots
- C5 root- upper lateral arm, never below the elbow
- C6 root- lower lateral arm, into thumb if pain goes through the hand
- C7 root- deep pain in triceps area. Front and back of forearm and into the middle finger especially
- C8 root- pain in the medial forearm and into the two medial finger
- T1 root- deep pain in the axilla and shoulder with some radiation down the inside of the arm
Cervical spondylosis with myelopathy
- osteoarthritis is a risk factor
- Nerve root problem with compression of the cord
Myopathic neurological defect
- Proximal, fatigable weakness
- Minimal atrophy
- No fasciculations
- Normal/reduced tone
- Normal reflexes
Myasthenia gravis
- Disorder of neuromuscular transmission
- Positive anti-AChR antibodies
- Abnormal EMG
Cranial nerve abnormalities
- CNIII (oculomotor)- right eye down and out (MR, IR, SR, IO), Mydriasis (pupillary constrictor), Ptosis (levator palpebrae superioris)
- VI (abducens)- diplopia on right gaze (LR)
- Ophthalmic division of V (trigeminal)- altered sensation to right forehead.
Cavernous sinus thrombosis
- Central dural sinus
- Receives venous drainage from facial veins
- Structures passing through include ICA and several cranial nerves
- Infections from face, nose, and tonsils can easily spread here
Differentials for bilateral leg weakness
- Guillain-Barré syndrome
- Cauda-Equina syndrome (rule out early)
- Stroke (if unilateral, worth keeping in mind)
- Transverse myelitis
- Epidural abscess
- ‘Off legs’ (general reduced mobility)
Tracts which run through the spinal cord
- Voluntary movement is controlled by the corticospinal tract (descending).
- Pain and temperature sensation travels through the lateral spinothalamic tract (ascending).
- Vibration sensation and proprioception travel though the dorsal column (ascending).
Types of lesions in the spinal cord
- Brown-sequard syndrome-left side of the spinal cord affected
- Anterior spinal artery syndrome (ASA): anterior 2/3 of spinal cord affected
- Central cord syndrome: central spinal cord affected with peripheries unaffected
