Neurology Flashcards
What are the main causes of ischemic strokes
Thrombosis
- atherosclerotic plaque stenosis/ rupture
usually occurs at branch points → carotid artery stenosis & middle cerebral artery in the circle of Willis
Embolism
cardio embolic- atrial fibrillation
Hypoperfusion
What is the main cause of haemorrhagic stroke
weakening of cerebral vessels leading to cerebral vessel rupture and haematoma formation.
aneurysms - saccular/ berry, Charcot-bouchard
vascular rupture, resulting in intra-parenchymal and/or subarachnoid haemorrhage. 2/3 = intracerebral haemorrhage, 1/3 = subarachnoid haemorrhage
-cerebral amyloid angiopathy
- high BP
- Av malformation, surge in high blood pressure
- cocaine/ amphematime, cavernoma
- Clinical deficit is caused directly by neuronal injury and indirectly by cerebral oedema (this peaks at day 5 following symptom onset).
Ischemic stroke RFs
9 strong
5 weak
- strong = age, male sex, personal/ family history of ischaemic stroke/ TIA, hypertension, smoking, diabetes mellitus, and atrial fibrillation, carotid artery stenosis
- weak = hypercholesterolaemia, obesity, poor diet, oestrogen-containing therapy, and migraine, sickle cell disease
Haemorrhagic stroke RFs
7 strong
4 weak
- strong = age, male sex, family history of haemorrhagic stroke, haemophilia, cerebral amyloid angiopathy/hypertension, anticoagulation therapy, illicit sympathomimetic drugs (such as cocaine and amphetamines), and vascular malformations (particularly in younger patients).
- weak = non-steroidal anti-inflammatories, heavy alcohol use, and thrombocytopenia.
- Moreover, Asians have a higher rate of intracerebral haemorrhage compared with other ethnic groups
What is the criteria for total anterior circulation infarct? (TACI)
- Contralateral hemiplegia or hemiparesis, AND
- Contralateral homonymous hemianopia, AND
- Higher cerebral dysfunction (e.g. aphasia, neglect)
A TACI involves the anterior AND middle cerebral arteries on the affected side.
What is the criteria for a partial anterior circulation infarct (PACI)
2 of this:
- Contralateral hemiplegia or hemiparesis, AND
- Contralateral homonymous hemianopia, AND
- Higher cerebral dysfunction (e.g. aphasia, neglect)
- OR isolated higher cerebral dysfunction alone.
A PACI involves the anterior OR middle cerebral artery on the affected side.
Anterior Cerebral Artery stroke features
- contralateral Hemiparesis (motor cortex), Hemisensory loss (sensory cortex) of the lower limb
- cognitive and personality change - frontal lobe symptoms
Middle Cerebral Artery stroke features (most common site of stroke)
- contralateral Hemiparesis (motor cortex), Hemisensory loss (sensory cortex) of the upper limb
- homonymous hemianopia
- contralateral lower face spastic paralysis (UMN so spastic, also forehead sparing)
- neglect (parietal lobe) → if right lobe
- Receptive or expressive dysphasia (due to involvement of Wernicke’s and Broca’s areas)→ if left lobe
What is a lacunar infarct
- A LACI affects small deep perforating arteries, typically supplying internal capsule or thalamus.
- pure motor - internal capsule
- pure sensory - thalamus
- sensori-motor - thalamus, internal capsule, basal ganglia
- ataxic hemiparesis - base pons, internal capsule
- There should be NO: visual field defect, higher cerebral dysfunction, or brainstem dysfunction.
secondary to hyaline arteriolosclerosis, a complication of benign hypertension or diabetes or smoking.
A posterior circulation infarct (POCI) is defined by:
- Cerebellar dysfunction, OR
- Conjugate eye movement disorder, OR
- Bilateral motor/sensory deficit, OR
- Ipsilateral cranial nerve palsy with contralateral motor/sensory deficit, OR
- Cortical blindness/isolated hemianopia.
A POCI involves the vertebrobasilar arteries and associated branches (supplying the cerebellum, brainstem, and occipital lobe).
What artery supplies the midbrain
PCA
Posterior cerebral artery stroke features
Contralateral homonymous hemianopia with macular sparing
Visual agnosia
Memory problems
may also get Thalamic syndrome: contralateral sensory loss: face, arms, legs, proprioception defect, and chronic pain on resolution of stroke (PCA supplies later thalamus)
Weber’s syndrome/medial midbrain syndrome stroke features
(branches of the posterior cerebral artery that supply the midbrain)
(paramedian branches of the upper basilar and proximal posterior cerebral arteries)
causes an ipsilateral oculomotor nerve palsy
contralateral hemiparesis of upper and lower extremity
What artery supplies the lateral and medial pons
lateral -> AICA
medial -> Basillar artery
Basilary artery stroke?
‘Locked-in’ syndrome or ventral pontine syndrome
- loss of corticospinal and corticobulbar tracts → bilateral paralysis (quadriplegia) → of arms, legs and face
- Patient can only blink
(similar to central pontine myelinolysis - demyelination of central pontine axis due to rapid correction of hyponatraemia)
Anterior inferior cerebellar artery stroke features?
results in lateral pontine syndrome,
CN 8 vestibular nuclei damage: nystagmus, vertigo and N&V
CN 8 Cochlear nuclei: Deafness
CN 7 Facial nucleus: Ipsilateral facial droop, loss of corneal reflex, loose Taste on anterior tongue
CN 5: ipsilateral face pain/ temperature
spinothalamic tract: contralateral loss of pain and temperature sensation
sympathetic tract- causes ipsilateral Horner’s syndrome
Posterior Inferior Cerebellarartery stroke features
Wallenberg’s syndrome (lateral medullary syndrome)
- 9 and 10 nucleus: nucleus ambiguous (horseness, dysphagia, decreased gag reflex)
- spinothalamic tract: contralateral loss of pain and temperature sensation
- causes ipsilateral Horner’s syndrome
- CN 8 vestibular nuclei damage: nystagmus, vertigo and N&V
- spinal 5 nucleus: ipsilateral loss of pain and temperature sensation on the face
(ipsilateral deficits in face and contralateral sensor in body )
Anterior spinal artery stroke features
can affect medulla or spinal cord
midline structure
medulla level = corticospinal, medial lemniscus and CN12 damage = contralateral hemiparesis, contralateral loss of proprioception/ vibration, flaccid paralysis of tongue (deviation to side of lesion)
spinal level = bilateral motor and sensory deficit, sparing posterior dorsal columns (vibration & proprioception)
Superior cerebellar artery stroke fetaures
- mostly exhibit all cerebellar symptoms DANISH
(dysdiadochokinesis
ataxia
nystagmus
intention tremor
scanning dysarthria
heel-shin test positivity) - Ipsilateral cerebellar ataxia
- put hand on palm and flip it over → cannot on ipsilater side
- nose to finger → cannot of ipsilateral side
- Nausea and vomiting
Retinal/ophthalmic artery stroke
Amaurosis fugax - is a temporary loss of vision in one or both eyes due to a lack of blood flow to the retina.
Putaminal Hemorrhagic stroke
- Aputaminal hemorrhageis the most common form ofintracranial hemorrhage because theputamen is the most common site involved with hypertensive intracranial hemorrhage.
- contralateral hemiparesis (internal capsule)
- hemisensory loss (thalamus)
- Gaze deviation toward the side of the bleed (frontal eye field)
- watch for: left paralysis & sensory loss. Eyes deviated to the right.
What are 2 screening tools used for stroke?
Use a validated tool to aid recognition: use ROSIER (Recognition of Stroke in the Emergency Room) in the emergency department; use FAST (Face Arm Speech Test) in the community.
Initial ABCDE management of acute stroke
- Airway protection
- (in patients presenting with depressed consciousness)
- endotracheal intubation for patients who are unable to protect their airway or for those presenting with a depressed level of consciousness (Glasgow Coma Scale score ≤8).
- supplemental oxygen only if oxygen saturation drops below 93%. (target 94%-96%)
- aspiration precautions (in patients presenting with swallowing impairment) are very important.
- antihypertensive treatment only if there is a hypertensive emergency
- hydrate
- Bloods
- Exclude hypoglycaemia and hyperglycaemia before giving thrombolysis;
- hypoglycaemia is a stroke mimic and hyperglycaemia is associated with intracerebral bleeding and worse clinical outcomes in patients treated with intravenous thrombolysis.
- Maintain a blood glucose concentration between 4 and 11 mmol/L.
- serum urea and creatinine- renal failure may be a potential contraindication to some stroke interventions.
- serum electrolytes- To exclude electrolyte disturbance (e.g., hyponatraemia) as a cause for sudden onset neurological signs.
- FBC - To exclude anaemia or thrombocytopenia prior to possible initiation of thrombolysis, anticoagulants, or antithrombotics.
- prothrombin time and PTT (with INR) - To exclude coagulopathy. Don’t delay thrombolysis (e.g., by waiting for test results) if the patient has no history of anticoagulant use, coagulopathy, or a condition that may lead to coagulopathy.
- Exclude hypoglycaemia and hyperglycaemia before giving thrombolysis;
Imaging for acute stroke
After ABCDE management:
non-enhanced CT head - distinguish ischaemic from haemorrhagic stroke
- ideally in the next available time slot and definitely within 1 hour of arrival at hospita
- (most sensitive test for confirming ischaemic infarct is a diffusion weighted MRI. used if the diagnosis is unclear but not normally possible in the emergency setting)
acute management: ischemic stroke
abcde
non-contrast ct
3) hyper-acute treatments - If presenting within4.5 hours of symptom onset - Thromboysis with Alteplase (tissue plasminogen activator)
- contraindications: recent head trauma, GI or intracranial haemorrhage, recent surgery, BP>185/110, platelet count, INR > 1.7, elevated PTT, glucose <50mg, stroke or head trauma in the past 3 months
- Otherwise, Mechanical Thrombectomy
- in patients with anterior circulation strokes within 6 hours of symptom onset
- in posterior circulation strokes up to 12 hours after onset
4) Aspirin 300 mg orally once daily for two weeks
- If hyper-acute treatments are offered, aspirin is usually started 24 hours after the treatment following a repeat CT Head that excludes any new haemorrhagic stroke.
5) Consider decompressive hemicraniectomy (which should be performed within 48 hours of symptom onset) for people with acute stroke who meet all of the following criteria:
- Clinical deficits that suggestinfarction in the territory of themiddle cerebral artery (with a score >15 on the National Institutes of Health Stroke Scale (NIHSS))
- Decreased level of consciousness,
- Signs on CT of an infarct of at least 50% of the middle cerebral artery territory
acute management: Haemorrhagic stroke
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non-contrast CT
3) Neurosurgical and neurocritical care evaluation due to the potential for surgical intervention (e.g. decompressive hemicraniectomy).
- Craniotomy is done to relieve pressure and stereotactic aspiration to aspirate blood using CT scan for guidance and relieve pressure.
4) Admission to the neuro ICU or stroke unit (the patient may require intubation and ventilation or invasive monitoring of ICPs).
5) Aim to keep blood pressure <140/80 as poor blood pressure control in the acute stage is associated with poorer outcomes later on
- labetalol or nimodipine
6) Urgently reverse abnormalities of clotting, particularly in patients taking anticoagulants
how to reverse anticoagulation
Warfarin/ vit K antagonist:
Dabigatran:
Factor Xa inhibitor reversal:
Warfarin/ vit K antagonist: prothrombin complex concentrate/fresh frozen plasma and phytomenadione (vit K)
Dabigatran: idraucizumab
Factor Xa inhibitor reversal: prothrombin complex concentrate
Stroke investigations (post-acute)
Investigations in the post-acute phase aim to further define the cause of the stroke and to quantify vascular risk factors.
Further investigations to determine the cause of the stroke include, for example:
- In ischaemic stroke:
- carotid doppler ultrasound (to identify critical carotid artery stenosis)
- CT/MR angiography (to identify intracranial and extracranial stenosis)
- 12h ECG & echocardiogram (if a cardio-embolic source is suspected)
- In young patients further investigation e.g. a vasculitis screen or thrombophilia screen may be necessary.
- In haemorrhagic stroke:
- serum toxicology screen (sympathomimetic drugs e.g. cocaine are a strong risk factor for haemorrhagic stroke).
- to quantify vascular risk factors include:
- serum glucose (all patients with stroke should be screened for diabetes with a fasting plasma glucose or oral glucose tolerance test)
- serum lipids (to check for raised total cholesterol/LDL cholesterol).
Stroke management of ischemic stroke (chronic)
The key steps in secondary stroke prevention can be remembered by the mnemonic HALTSS:
- Hypertension:
- Anti-hypertensive therapy should, however, be initiated 2 weeks post-stroke.
- no benefit in lowering the blood pressure acutely (as this may impair cerebral perfusion) unless there is malignant hypertension (systolic blood pressure >180 mmHg).
- Antiplatelet therapy:
- patients should be administered Clopidogrel 75 mg once daily for long-term antiplatelet therapy (after 2 weeks of aspirin).
- If clopidogrel CI, then aspirin &dipyrimidole
- In patients with ischaemic stroke secondary to atrial fibrillation, and chadVASc ≥2, warfarin (target INR 2-3. or a direct oral anticoagulant (such as Rivaroxaban or Apixiban) is initiated 2 weeks post-stroke.
- patients should be administered Clopidogrel 75 mg once daily for long-term antiplatelet therapy (after 2 weeks of aspirin).
- Lipid-lowering therapy:
- patients should be prescribed high dose atorvastatin 20-80 mg once nightly (irrespective of cholesterol level this lowers the risk of repeat stroke).
- Do not start statin treatment immediately. There is consensus that it is safe to start statins after 48 hours
- Tobacco:
- offer smoking cessation support.
- Sugar:
- patients should be screened for diabetes and managed appropriately.
- Surgery:
- patients with ipsilateral carotid artery stenosis more than 50% should be referred for carotid endarterectomy.
- (carotid endarterectomy also indicated for non-stroke patients with 70% occlusion)
- patients with ipsilateral carotid artery stenosis more than 50% should be referred for carotid endarterectomy.
- Feeding assessment and management
- swallow
- All patients presenting with acute strokemust be screened for safe swallowing function prior to further oral intake, as dysphagia is common after stroke
- if unsafe: NG tube feeding, if not tolerated → nasal bridle tube/gastrostomy instead
CHADS VASc score
used for AF stroke risk
CHF +1
Hypertension +1
Age >= 75 +2
Diabeter +1
Stroke/TIA/VTE +2
Vasc disease +1
Age 65-74 +1
Sex (female) +1
1- oral anticoagulant should be considered
2 or oral - anticoagulant is recommended
Complete vs incomplete spinal cord injury & presentation
complete = complete loss of function (motor power, sensory input, reflexes)→ may enter spinal shock
- caused by complete transection of the cord or compression
- loss of involuntary movement & sensation if parts enervated by segment
- spinal shock
- irreversible
Incomplete = some preservation of function below level of injury
- often from contusions produced by pressure on the cord
- clinical picture unpredictable
- some function is present below site.
What are the types of incomplete spinal cord injury (6)
- Brown-Sequard Syndrome
- Anterior Cord Syndrome
- Posterior Cord Syndrome
- Central cord syndrome
- Cauda Equina Syndrome
- Conus Medullaris Syndrome
What is autonomic dysreflexia? cord level?
a lesion affecting T6 or higher.
triggered by excessive autonomic response to stimuli below the level of the lesion, such as a faecal impaction or blocked catheter.
The abnormal response produces autonomic imbalance with sympathetic overactivity.
results in:
unbalanced physiological response: extreme hypertension, flushing and sweating above the level of the cord lesion
- agitation
- in untreated cases severe consequences of extreme hypertension have been reported, e.g. haemorrhagic stroke.
how to assess spinal cord injury?
history
- Nature and mechanism
- is it Complete, incomplete, absent SCI at scene
- was Methylprednisoloone given/not given/
- Concomitant injuries.
Examination
- Initial Assessment
A Airway and cervical spine immobilisation.
B Breathing: adequacy of oxygenation and ventilation.
C Circulation: Fluid resuscitation(in trauma patients hypotension should always be considered to be secondary to blood loss, not spine shock).
D Disability: GCS, pupil size.
- Associated injuries
- Level of spinal injury: Radiological and clinical. (level= Most distal uninvolved nerve root segment with normal function)
complete or incomplete
tetraplegia or paraplegia?
tetraplegia vs paraplegia?
Paraplegia is a paralysis starting in the thoracic (T1-T12), lumbar (L1-L5) or sacral (S1-S5) area, while tetraplegia is caused by damage in the cervical area (C1-C8). Persons with paraplegia possess good functioning of the arms and hands.
investigations in spinal cord injury
- Routine Bloodsand ABG- FBC, U&Es,calcium, ESR, immunoglobulin electrophoresis (multiple myeloma)
-
Radiology
- Trauma series for trauma: First CT cervical spine, then CT of whole spine to look for injury somewhere else in the spine. Imaging of cervical spine is especially important
- Refer urgently to a neurosurgeon or spinal surgeon any patient with clinical signs of aspinal cord injury(i.e., an abnormal neurological exam). An MRI of the spine is indicated (in addition to the CT) in these patients. ( T2 weighted saggital MRI scan )
- Patient with clinical features suggestive of spinal cord compression or cauda equina syndrome should have an urgent WHOLE spine MRI, with an aim (in appropriate cases) to surgically decompress within 48 hours.
- Monitor: pulse oximetry, ecg, BP, twice daily VC, UO with catheter. ICU monitoring is needed.
- Urine- look for Bence Jones proteins (multiple myeloma)
Management of spinal cord injury
- ABCDE
- Maintain SpO2 >98% and if C3 injury or above then do invasive ventilation/tracheostomy. IF T6 and above do non-invasive ventilation or bird respirator.
- Maintain cardiovascular system and prevent/treat spinal shock - prevent hypotension (SBP >90mmHg).
- Patient may need ICU monitoring (cardiac, haemodynamic, respiratory monitoring to detect any dysfunction) and they should be transferred to specialist centres.
- full in-line spinal immobilisation if needed
- Pain management:
- In acute phase of trauma: intravenous morphine as the first-line analgesia. If intravenous access has not been established, consider diamorphine or ketamine via the intranasal route (follow local protocols)
- For chronic spinal cord injury
- Nociceptive pain: physiotherapy and simple analgesia
- Neuropathic: Gabapentin/ pregabalin/ amitriptyline (first line). Oxycodone/ tramadol/ venlafaxine (second line)
- If progressive neurologic deficit: urgent assessment.
- Surgical decompression in 48 hours if needed- if there is presence of retained foreign objects, CSF leakage, infection or signs of extrinsic spinal cord compression.
- If progressive neurology is secondary ischemia or inflammation- no surgery.
- Treat underlying autonomic dysreflexia if present, treated by resolving: urinary retention (catheter) , rectal examination (check for and remove faecal impaction. If doesn’t resolve vasodilator to lower BP (sublingual nifedipine or glyceryl trinitrate )
- Supportive care: resp, pressure sores, bladder & bowel management
- spinal shock is autonomic function loss
- Blood pressure should be maintained
- DO NOT give steroids in acute stage - in patients where malignancy is demonstrated on MRI, or in patients where clinical suspicion is high, administration of dexamethasone 16 mg daily in divided doses (with PPI cover) is indicated.
- Immoblisation to prevent further injury
- refer to specialised centres
- Physiotherapy and mobilisation
Brown-sequard syndrome
(what is it, cases, spinal cord tracts, affected, presentation)
lateral hemisection or loss of half of the spinal cord
trauma (knife or gunshot), tumour
- Lateral corticospinal tract
- Dorsal columns
- Lateral spinothalamic tract
Below the lesion:
- ipsilateral weakness below lesion (UMN motor signs= spastic paresis )
- ipsilateral loss of proprioception and vibration sensation
- contralateral loss of pain and temperature sensation
At the level of the lesion:
→ Ipsilateral: complete sensory, (vibration, pain, proprioception, temperature) and motor loss (LMN lesion sign)
if lesion is above T1 → Horner’s syndrome: Constricted pupil, eyelid droop
Anterior cord syndrome
(what is it, cases, spinal cord tracts, affected, presentation)
2nd most common
- Usually caused by cervical flexion injury , which occludes anterior spinal artery and/ or damages anterior spinal column
- damage to the aorta, including both aneurysm repair and dissection.
Lateral corticospinal tracts
Lateral spinothalamic tracts
- Bilateral spastic paresis (motor function lost)
- Bilateral loss of pain and temperature sensation
- Autonomic dysfunction (loss of sympathetic) can manifest as hypotension (either orthostatic or frank hypotension), sexual dysfunction, and/or bowel and bladder dysfunction
(Vibration, proprioception intact )
Central cord syndrome
(what is it, cases, spinal cord tracts, affected, presentation)
Most common spinal cord injury
- hyperextension injury
- usually in elderly with existing stenosis
- syringomyelia or intramedullary cord tumors
- Impairment of the upper limb pain and temperature sensation. U Limb> LL
- patients will walk with bowel/ bladder function preservation
- Usually spinothalmic tract affected
Posterior cord syndrome
(what is it, cases, spinal cord tracts, affected, presentation)
compression by tumor or infarct of posterior spinal artery
- loose function of dorsal column → proprioception, two point discrimination are lost so keep falling over
- Preservation of motor function, pain and temperature sensation
Cauda Equina Syndrome
(what is it, cases, spinal cord tracts, affected, presentation)
Compression of the Cauda Equina, which is a continuation of the spinal nerves inferiorly beyond the Conus medullaris,
L2-sacrum
lumbosacral nerve roots
more gradual and unilateral presentation
more severe
Massive disk rupture
Trauma, tumour
dysfunction of posterior longitudinal ligament, central cord prolapse
Function of Cauda Equina nerve roots
- Motor and sensory to lower extremity
- Pelvic floor/ sphincter innervation
So in Cauda Equina syndrome
- severe low back pain
- severe bilateral sciatica
- Saddle anaesthesia
- loss of anocutaneus reflex (when you touch skin outside anal sphincter, contraction or muscle)
- bilateral sensory anal loss
- Bowel and bladder dysfunction- prevents later
- Normal babinski (Cauda Equina nerves are peripheral nerves so LMN findings seen not UMN findings)
- both knee and ankle jerks affected -> arreflexia
Conus medullaris syndrome
L1-L2
sacral cord segment and roots
acute and bilateral presentation
symmetrical, less marked hypperreflexia
- perianal anaesthesia bilaterally, as opposed to entire saddle area
- Impotence more frequent
- More acute presentation
Epidemiology and RFs of MND
M>F
Mean age of onset: 55 yrs
- 5-10% have afamily historywith autosomal dominant inheritance (genes including SOD1, FUS and C9ORF72)
- 90% sporadic
- Risk factors include genetic predisposition or family history, and age >40 years.
-
Associations
- Frontotemporal lobar dementia
- familial cases → zinc copper superoxide dismutase deficiency → increased free radical damage
- Free radical damage and glutamate excitotoxicity have been implicated
Types of MND
Amyotrophic Lateral Sclerosis (ALS) (50%) (a.k.a. Spinal ALS)
Progressive Bulbar Palsy Variant
Progressive Muscular Atrophy Variant
Primary Lateral Sclerosis Variant
ALS presentation
-
Amyotrophic Lateral Sclerosis (ALS) (50%) (a.k.a. Spinal ALS)
- AKA Lou Gehrig’s disease
- Combined generation of upperANDlower motor neurones resulting a mix of LMN and UMN signs
- Combination of UMN and LMN signs
-
LMN Features (anterior motor horn)
- Muscle wasting
- Fasciculations - e.g. tongue fasiculations
- Flaccid weakness, paralysis
- Hyporeflexia, hypotonia
- negative Babinski
-
UMN Features (lateral corticospinal tract)
- Spastic weakness, paralysis
- Extensor plantar response (babinski sign )
- Hyperreflexia, hypertonia
- Speech disturbance (slurring or reduction in volume)
- Swallowing disturbance (e.g. choking on food, especially solid foods), dysphagia - cranial nerves can be involved
- Behavioural changes (e.g. disinhibition, emotional lability)
- wasting & weakness of the small hand muscles/tibialis anterior is common - early sign
- Sensory examination- should be NORMAL
- • doesn’t affect ocular muscle, no cerebellar signs, no sphincter disturbances
Late symptoms: immpbile, cannot communicate, unable to swallow, drooling, breathing difficulties
Primary Lateral Sclerosis Variant presentation
-
Primary Lateral Sclerosis Variant
- UMN pattern of weakness
- Brisk reflexes
- Extensor plantar responses
- NOLMN signs
- Progressive Muscular Atrophy Variant presentation
-
Progressive Muscular Atrophy Variant
- Only LMN signs
- affects distal muscles before proximal
- carries best prognosis
Progressive Bulbar Palsy Variant presentation
-
Progressive Bulbar Palsy Variant
- palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
- Dysarthria
- Dysphagia
- Wasted fasciculating tongue
- Brisk jaw jerk reflex
- carries worst prognosis
MND Investigations
clinical diagnosis
-
Bloods
- Mild elevation in CK, ESR
- negative Anti-GM1 ganglioside antibodies (multifocal mononeuropathy) & negative acetylcholine receptor antibodies (myasthenia gravis)
- Electromyography (EMG)- reduced number of action potentials with increased amplitude
- Nerve conduction studies- often normal, can help exclude a neuropathy.
- MRIbrain & spine- exclude cord compression, myelopathy and brainstem lesions
- Spirometry- assess respiratory muscle weakness
Management of MND
no cure. The focus of medical care is to provide supportive and palliative intervention, aiming to improve the quality of life for patients
Riluzole (50mg twice daily)
- only drug for modifying the disease course and should be offered to patients at the time of diagnosis.
- decreases glutamate release by neurones
- used mainly in amyotrophic lateral sclerosis
- increases ventilation free survival by just a few months
- monitor LFTs and FBCs monthly for first three months, and then every 3 months thereafter
Supportive care
muscle cramps- tonic water, quinine
spasticity- baclofenac
drooling - atropine, hyoscine patch, botox
pain- amitriptyline, gabapentine. pregabalin
Difficulty expelling mucus secretion - carbocystine
- ongoing counselling regarding prognosis and end-of-life issues, with special emphasis on advance directives.
Respiratory care
- non-invasive ventilation positive pressure ventilation (usually BIPAP) can also prolong survival in patients with type 2 respiratory failure.
- If severe dyspnea - chronic invasive ventilation
- If do not choose ventilation - palliative care is used at
Dysphagia & weight loss
- diety modification
- when dysphagia present → Percutaneous endoscopic gasterstomy (PEG)
speech and language therapy
PEG/RIG
Prognosis of MND
usually fatal in 3-5 years
common cause of death is aspiration pneumonia
Subacute Combined Degeneration: everything
Demyelination of the posterior columns (Vibration/ proprioception loss), and a loss of lateral motor tracts (upper motor lesion symptoms) due to vitamin B12 ( cobalamin) deficiency
investigation
serum B12, folate (exclude folate def.), MRI (identify demyelinated lesion)
management
Vitamin B12
Red flags of back pain indicating cauda equina
- bilateral neurological deficit of the legs
- bowel bladder dysfunction
- Perianal or perineal sensory loss (saddle anaesthesia or paraesthesia).
- Unexpected laxity of the anal sphincter
Red flags of back pain indicating spinal fracture
- Sudden onset of severe central spinal pain which is relieved by lying down.
- history of major trauma (such as a road traffic collision or fall from a height), minor trauma, or even just strenuous lifting in people with osteoporosis or those who use corticosteroids.
- Structural deformity of the spine (such as a step from one vertebra to an adjacent vertebra) may be present.
- may be point tenderness over a vertebral body
Red flags of back pain indicating cancer
- > 50
- gradual onset of symptoms
- Severe unremitting pain that remains when the person is supine, aching night pain that prevents or disturbs sleep, pain aggravated by straining (for example, at stool, or when coughing or sneezing), and thoracic pain.
- Localised spinal tenderness.
- No symptomatic improvement after four to six weeks of conservative low back pain therapy.
- Unexplained weight loss
- Past history of cancer — breast, lung, gastrointestinal, prostate, renal, and thyroid cancers are more likely to metastasize to the spine.
Red flags of back pain indicating infection
- Fever
- Tuberculosis, or recent urinary tract infection.
- Diabetes.
- History of intravenous drug use.
- HIV infection, use of immunosuppressants, or the person is otherwise immunocompromised.
