Neurology (Quesmed) Flashcards
What are the differentials for Autonomic Dysfunction (Dysfunction of the Involuntary Nervous System- things that happen whether we want them to or not- vasoconstriction, sensation etc.)?
Autonomic Neuropathies
Neurodegenerative Diseases (Parkinson’s, Multiple System Atrophy, Autonomic Failure)
Drug Induced Autonomic Dysfunction- Antihypertensives, Anticholinergics, Tricyclic Antidepressants
What are the causes of Autonomic Neuropathies?
Diabetes (Most Common)
HIV, Lyme Disease, Chagas Disease
Autoimmune Diseases (like SLE)
AMYLOIDOSIS
What are some of the common signs of Autonomic Dysfunction?
Dizziness
Feeling FULL early (Early Satiety) + Nausea + Bloating= Gastroparesis
Postural Hypotension (on standing)
What are the 2 types of Brachial Plexus Injuries?
Erb’s Palsy (C5-6)
- Dermatomal Sensory Loss
- Weakness of the Intrinsic Muscles of the hand
- Associated with Shoulder Dystocia and Traumatic Childbirth
Klumpke’s Palsy (C8-T1)
- Dermatomal Sensory Loss
- Weakness of the Intrinsic Muscles of the Hand
- T1 Involvement can lead to Horner’s Syndrome
- Claw Hand
What should be suspected if a patient presents with Horner’s Syndrome and Erb’s/ Klumpke’s Palsy?
SQUAMOUS CELL LUNG CARCINOMA- Pancoast Tumour
What can a mid-humeral fracture result in?
A Damaged RADIAL NERVE
This leads to WRIST DROP due to the EXTENSOR MUSCLES BEING WEAKENED
What is Brown-Sequard Syndrome?
It is the Anatomical Disruption of Nerve Fibre Tracts in one half of the spinal cord
So injury of one side of spinal cord leads to symptoms on that side
What are the signs of Brown-Sequard Syndrome?
Ipsilateral Hemiplegia
Ipsilateral loss of Proprioception and Vibration
Ipsilateral HYPERreflexia and HYPERtonia
CONTRALATERAL Loss of Pain and Temperature (2-3 segments below the level of the lesion)
What are the causes of Brown-Sequard Syndrome?
Cord Trauma (Penetrating Injuries (KNIFE/ GUNSHOT) are the most common)
Neoplasms
Disk Herniation
Demyelination
Infective/ Inflammatory Lesions
Epidural Haematomas
What is the management of Brown-Sequard Syndrome?
Surgical Intervention if PHYSICAL CAUSE
1) Retained Foreign Objects
2) CSF Leakage
3) Infection or signs of Extrinsic Spinal Cord Compression
Medical Management if CHEMICAL/ MICROSCOPIC CAUSE
1) Infective/ Inflammatory causes
2) Demyelinating causes
What is Bulbar Palsy?
Which Cranial Nerves are affected?
It is a “Lower Motor Neurone” Lesion affecting Cranial Nerves 9,10 and 12
This causes impairments in SPEECH and SWALLOWING
What are the signs of bulbar Palsy?
If PSEUDOBULBAR- SPASTIC TONGUE with NO FASCICULATIONS
Absent or Normal JAW JERK REFLEX
Absent GAG REFLEX
Flaccid, Fasciculating Tongue
Nasal Quiet Speech
Signs of the cause (Lower Limb Fasciculations if Motor Neurone Disease)
What are the causes of Bulbar Palsy?
Motor Neurone Disease (the Progressive Bulbar Palsy Variant)
1) Myasthenia Gravis
2) Guillain-Barre Syndrome
Brainstem Stroke (Lateral Medullary Syndrome)
Syringobulbia
How is Progressive Bulbar Palsy (Motor Neurone Disease) diagnosed?
EMG shows DIFFUSE DENERVATION
What are the signs of Cerebellar Syndrome?
DANISH
Dysdiachokinesia (Inability to perform rapid alternating hand movements)
Ataxia (unsteady gait)
Nystagmus (involuntary eye movements)
Intention Tremor (Seen in the finger-nose test)
Slurred Speech
Hypotonia
Cerebellar Vermis- Ataxia (few signs in limbs)
Cerebellar Hemisphere- signs in Ipsilateral Limb
What are the causes of Cerebellar Syndrome?
VITAMIN C (usually Strokes or Multiple Sclerosis)
Vascular causes (Strokes- Ischaemic or Haemorrhagic) that affect the Posterior Circulation)
Infective Causes (LYME Disease)
Inflammatory Causes (Multiple Sclerosis)
Traumatic Causes (trauma to Posterior Fossa)
Metabolic Causes (Alcoholism)
Iatrogenic Causes- PHENYTOIN and CARBAMAZEPINE
Neoplastic Causes- Tumours
Congenital/ Hereditary- Friedrich’s Ataxia/ Spinocerebellar Ataxia
How do you differentiate between Cerebellar and Sensory Ataxia?
Romberg’s sign positive (measure of PROPRIOCEPTION) (can’t stand with eyes closed but can without)- SENSORY
Romberg’s sign negative- CEREBELLAR
They need to SEE to STAND= SENSORY
What is Cervical Spondylosis (Cervical Osteoarthritis)?
It is an Osteodegenerative Disease of the Cervical Spine- due to wear and tear
It is a common and underdiagnosed cause of Falls in the Elderly
(usually seen in 50-59 year olds)
What are the signs of Cervical Spondylosis?
Suspect in Neck Pain/ Lhermitte’s following Osteoarthritis
Suspect in Progressive Upper Limb Weakness and 70 year olds- after OSTEOARTHRITIS
LACK of Bladder/ Bowel issues- unless very SEVERE
Neck pain, Arm Weakness, Following Osteoarthritis in the Elderly basically
Neck Pain, Headache, Interscapular Pain
Radiculopathy (due to compression of NERVE ROOTS/ injury of nerve roots at the site of the Foraminal Exit)- leading to FLACCID UPPER LIMB PARESIS
Myelopathy- due to the dynamic stretch of the spinal cord over impinging Spinal Osteophytes (Injury to spinal cord caused by COMPRESSION of SPINAL CORD itself)
Variable Sensory Changes (like the Lhermitte Phenomenon)- Pain on Neck Flexion
Spastic Paraparesis
- Variable involvement of the upper limbs depending on site of lesion and degree of Radiculopathy
How can you differentiate between Motor Neurone Disease and Cervical Myelopathy?
Motor Neurone Disease has NORMAL SENSATION
Cervical Myelopathy does not (because it is compressed so sensation is also affected)
When does Multiple Sclerosis present age-wise?
20-40 years of age (so UNLIKELY in children in people over middle-aged)
What is the key sign of Mononeuritis Multiplex?
Why does it occur?
Asymmetrical, Painful, Progressive, Multifocal Neuropathy of SENSORY and MOTORNERVES
Of MULTIPLE NERVE AREAS
Weakness, Atrophy, Cramping, Hyporeflexia
It is ASYMMETRICAL
It occurs due to an autoimmune reaction to a protein on motor neurone cells
More local than Multiple Sclerosis, which is symmetrical
What are the signs of Cubital Tunnel Syndrome?
It affects the ULNAR NERVE and Medial Half of the hand
Lateral Half- Carpal Tunnel Syndrome
What is Charcot-Marie-Tooth Syndrome (HEREDITARY SENSORIMOTOR NEUROPATHY- HSMN)?
What is the inheritance?
When does it usually present?
It is a HEREDITARY MOTOR and SENSORY NEUROPATHY
It is the MOST COMMON group of Genetic Peripheral Neuropathies
The most common gene associated is the !!!!Autosomal Dominant mutation of the PMP22 gene
There are 2 types-
1) Type 1= Demyelinating (more common)
2) Type 2= Axonal
The disorder affects both the Sensory and Motor Nerves of the Peripheral Nervous System- though MOTOR NERVES are MORE affected
Patients usually present in PUBERTY with !!!!!!!!!! symptoms affecting the feet which progresses to involve the hands
What are the signs of Charcot-Marie-Tooth Syndrome?
There is a FAMILY HISTORY of this condition
PROGRESSION and INHERITED
1) Thickening and Enlargement of the Nerves themselves
2) Symmetrical Distal Muscular Atrophy (Champagne Bottle Legs and !!!!!Claw Hands)
3) Pes Cavus (High Arched Feet) due to ankle wasting and they therefore have a HIGH STEPPING GAIT to compensate
Therefore TRIPPING as well
What is needed for the diagnosis of Charcot-Marie-Tooth Syndrome?
Nerve Conduction Studies and Genetic Testing
Type 1- Reduced Conduction Velocity
Type 2- Normal Nerve Conduction
NO CURE but majority of them require WALKING AIDS
What are the signs of Huntington’s Disease?
DCD
PRESENT AFTER 35 YEARS OLD
Dominant Inheritance
Choreoathetosis (Chorea= Uncontrollable Twitches-Jerky or Dancelike unlike Tremors, Athetosis= Slow ongoing movements that prevent stable posture)
Dementia
What investigations should be ordered in Huntington’s Disease?
What is needed for DEFINITIVE Diagnosis?
MRI and CT show
1) LOSS of STRIATAL VOLUME
2) INCREASED SIZE of the FRONTAL HORNS of the Lateral Ventricles
Big Ventricles and Small Striata
DEFINITIVE DIAGNOSIS made by GENETIC TESTING
What is the management of Huntington’s Disease?
What is given for the chorea?
What other 2 symptoms are treated?
What is done for the progression?
Chorea- Tetrabenazine
Depression- SSRIs
Psychosis- ATYPICAL Antipsychotics
No drugs can prevent the PROGRESSION
What is the prognosis of Huntington’s Disease?
Increase in Chorea and eventually Dementia
Death usually occurs from Physical Decline leading to things like !!!!!PNEUMONIA
What actually happens in Huntington’s??
What is shown in MRI?
MRI would show ATROPHY of the CAUDATE NUCLEUS and the PUTAMEN
What are the core features of Dementia with Lewy Bodies?
Fluctuating Cognition
Parkinsonism
Hallucinations
What investigations are used to confirm Dementia with Lewy Bodies?
Usually Clinical
But Dopamine Uptake Scanning may be used
What is the Pathophysiology of Dementia with Lewy Bodies?
Alpha-Synuclein Cytoplasmic Inclusions (Lewy Bodies) in the Substantia Nigra, Paralimbic and Neocortical Areas
What are the 7 differentials for Diplopia?
What suggests a Surgical Lesion in Third Nerve Palsy and what are the examples of Surgical/ Medical Lesions?
What can aid the diagnosis of a squint?
The Nerve Palsies, The 2 Ms and Squint/ Thyroid
1) Third Nerve Palsy
- Oculomotor Nerve Affected
- Controls all Ocular Muscles except Superior Oblique and Lateral Rectus Muscle
- Looking DOWN and OUT
- Other features= Ptosis (Upper Eyelid Drooping), Proptosis (Bulging) and Fixed Pupil Dilation (which suggests Surgical Lesion rather than Medical Lesion)
- Surgical Lesions= Compressive lesions like Posterior Communicating Artery Aneurysm
- Medical Lesions= Non Compressive lesions like Multiple Sclerosis or Vascular Causes= Diabetes
2) Fourth Nerve Palsy
- Trochlear Nerve
- Controls Superior Oblique Muscle
- Looking IN and UP (Patient may tilt head to compensate
- Ocular Trauma and Diabetes Mellitus are the most common causes
3) Sixth Nerve Palsy
- Abducens Nerve
- Controls the Lateral Rectus Muscle
- Looking MEDIALLY
- It is easily compromised when the ICP is raised
- !!!! Common causes= Diabetic Neuropathy, Stroke, Infection, Trauma
4) Myasthenia Gravis
- Diplopia and Ptosis WORSEN throughout the day and when watching TV
5) Strabismus (Squint)
- Common cause in CHILDREN
- !!!! Esotropia (Convergent Squint) is the most common- The malaligned Eye Diverges INWARDS towards the midline)
- !!!!The CORNEAL REFLEX Test and the COVER Test aid diagnosis
6) Multiple Sclerosis
- Symptoms may disappear between attacks
7) Thyroid Eye Disease
What is Dystonia?
Prolonged, Painful Muscle Contraction
What are the primary causes of Dystonia?
Idiopathic
- Idiopathic Generalised Dystonia- Flatau-Sterling Syndrome is an AUTOSOMAL DOMINANT condition that starts in the LOWER LIBS and spreads to involve the rest of the body
- Idiopathic Focal Dystonia- limited to one part of the body- Musician’s Cramp or Spasmodic Torticollis (Cervical Dystonia)
What are the secondary causes of Dystonia?
2 drugs and 3 common symptoms
Usually Anti-dopaminergic drugs (Antipsychotics or Antiemetics- Metoclopramide)
May present with Oculogyric Crisis (Spastic movement of eyes into a FIXED POSITION), Torticollis (Cervical Dystonia) or Trismus (Lockjaw)
What are the 3 types of Focal Seizures?
Hwre do they commonly occur?- which part of the brain?
1) With impairment of consciousness (Complex)
- Loss of consciousness occurs either on Seizure onset or after an aura. These most commonly occur at the TEMPORAL LOBE. Post Ictal Symptoms are common (like Confusion if Temporal Lobe)
2) Without impairment of consciousness (Simple)
- There are NO Post Ictal Symptoms
3) Evolving to a Bilateral, Convulsive Seizure (Secondary Generalised). The generalised seizure is usually Tonic-Clonic. This occurs in 2/3s of patients with Focal Seirzures
What are the signs of Focal Seizures based on the lobe?
1) Temporal Lobe
- Automatisms (Lip-Smacking)
- Deja Vu or Jamais Vu
- Emotional Disturbance (Sudden Terror)
- Olfactory, Gustatory or Auditory Hallucinations
2) Frontal Lobe
- Motor Features (Jacksonian features- twitching/ tremor on muscles of one side of the body, Dysphasia (aka Aphasia) or Todd’s Palsy- seizure followed by temporary paralysis)
3) Parietal Lobe
- Sensory Symptoms (Tingling, Numbness) and Motor Symptoms (due to the spread of electrical activity to the precentral gyrus of the frontal lobe)
4) Occipital Lobe
- Visual Symptoms
What is the treatment of Focal Seizures?
First line- LAMOTRIGINE or LEVETIRACETAM
Second-line- CARBAMAZEPINE, OXCARBAZEPINE or ZONISAMIDE
What are the 4 types of Generalised Seizures? How are they treated?
1) Absence Seizures- Treat with ETHOSUXIMIDE then Sodium Valproate (men) or Levetiracetam/Lamotrigine (women)- AVOID CARBAMAZEPINE as it makes the seizure worse
2) Tonic-clonic Seizures- They lose consciousness, stiffen and fall, and then start jerking. Treat with Sodium Valproate (Men) or Lamotrigine/ Levetiracetam (Women)
3) Myoclonic Seizures- A SUDDEN JERK. Treat with Valproate (unless they are a female of childbearing age- then LevetiracetaM (or Topiramate) instead. AVOID CARBAMAZEPINE
4) Atonic Seizure- Sudden loss of muscle tone and they fall. Treat with Valproate (men) or Lamotrigine (women)- same for JUST Tonic Seizures
What are the 4 complications of Epilepsy?
1) Status Epilepticus (>5 minutes)- treat with IV Lorazepam, Buccal Midazolam then Phenytoin if that does not work
2) Depression
3) Suicide
4) Sudden Unexpected Death in Epilepsy- Thought to be due to excess electrical activity that causes Arrhythmia and Death
What are the rules of thumb of Antiepileptics?
1) Lamotrigine, Levetiracetam or Valproate are good for ALL Seizures
2) Carbamazepine, Gabapentin and Phenytoin are better for Focal Seizures
3) Ethosuximide is the drug of choice for ABSENCE Seizures
4) Carbamazepine may worsen Absence and Myoclonic Seizures
5) Valproate should be avoided in Pregnancies due to risk of Neural Tube Defects- use Lamotrigine instead
What is a Jacksonian March?
It is a twitching that goes up from the Legs to the Arms and is associated with Frontal Lobe Seizures
What are the signs of a Psychogenic Seizure?
there are 3 signs that point to a psychogenic seizure
Asynchronous Limb Movements, TRUNK Movements and Closed Eyes
Treat by Verbally Reassuring them that they are fine
What is the investigation of choice in Seizures?
EEG
What are the 4 side effects of Lamotrigine?
Lamotrigine affects the SKIN
1) Large blistering rash throughout the body
2) Steven-Johnson Syndrome (Stevens-Johnson syndrome is a rare condition arising from ‘over-reaction’ of the immune system to a trigger such as a mild infection or a medicine, leading to blistering and peeling of the skin and surfaces of the eyes, mouth and throat)
3) Toxic Epidermal Necrolysis- severe version of SJS- affects TRUNKS as well (not just face) and >30% of the body (SJS is <10%)
4) Hypersensitivity Syndrome (if Fever, Multiorgan Failure occurs)
What are the signs of Essential Tremor?
What is the Inheritance?
When is it worse? and what makes it better?
Which limbs does it usually affect?
HALF of the cases are INHERITED Autosomal DOMINANTLY
1) Mainly involves Distal Upper Limbs and is Postural (holding hand out) or Kinetic (with movement)
2) The Tremor Amplitude INCREASES with time and they experience DIFFICULTY in Writing, Eating, Holding objects and doing Fine Motor Tasks
3) Issues with DRESSING and SPEAKING as well
4) Tremor increases with ANXIETY and STRESS
5) It is IMPROVED with ALCOHOL??!?! and REST
What is the management of Essential Tremor?
Remember the First Line Medical and the alternative medical
- Behavioural Techniques and Physical Therapy
- Medical Therapy- Propanolol, Primidone, then (Topiramate, Gabapentin, Clonazepam)
- Surgical- Deep Brain Stimulation, Focused Ultrasound Thalamotomy and Radiosurgical Thalamotomy (Gamma Knife)
What are the possible causes of Facial Nerve Palsy?
3 Infections
What is it usually due to?
What kind of Lesions cause it?
What infections and lesions can cause Facial Nerve Palsy?
1) Usually due to Bell’s Palsy
2) Physical Lesions of the Cerebellopontine Angle (such as Acoustic Neuroma (Benign Brain Tumour))
3) Basal Meningitis (often bilateral and may be infective or inflammatory- Lyme Disease or Sarcoidosis)
4) Ramsay Hunt Syndrome (Herpes Zoster of the Facial Nerve)
5) Trauma
6) Middle/ Inner Ear Infection
7) Mononeuritis Multiplex
What is Ramsay Hunt Syndrome?
What are the 3 signs and how is it managed?
The Reactivation of Varicella Zoster
Symptoms
- Facial Nerve Palsy
- Vertigo
- Sensorineural Hearing Loss with VESICULAR RASH over the affected ear
So manage with Prednisolone and Acyclovir
What is the Onset like for Bell’s Palsy and an Acute Stroke?
Which one has forehead sparing?
Acute Stroke- Sudden Onset- Unusual for Bell’s Palsy
Also Forehead Sparing= Stroke not Bell’s Palsy
What type of motor neurone lesions may be seen following Parotidectomies?
LOWER Motor Neuron Lesions if distal portion of temporal or zygomatic branches is affected
UPPER if proximal portion of mandibular or cervical branches
(Forehead Sparing (Which Includes Eyebrows) is an UPPER Motor Neuron Sign)
What is Creutzfeldt-Jakob Disease?
Myoclonus and Mental Deterioration
It is a group of Neurodegenerative Conditions caused by PRIONs (Misshaped Proteins)
It presents as Progressive Dementia, Myoclonus and Psychiatric Impairment
How is Creutzfeldt-Jakob Disease diagnosed?
Supportive Signs
- EEG- Biphasic, High Amplitude Sharp wave Complexes
- MRI- Basal Ganglia Hypertrophy
- Lumbar Puncture- Abnormal Proteins BUT usually normal
Also
Tissue Biopsy- TONSIL/ OLFACTORY Biopsy
How is Creutzfeldt-Jakob Disease treated?
Symptom control and Palliative Care
What is Fibromuscular Dysplasia?
It is a group of Non-Atherosclerotic, Non-Inflammatory Arterial Diseases that most commonly involve the Renal and Carotid Arteries
They account for 10% of Renal Vascular Diseases, the other 90% is Renal Artery Stenosis due to Atherosclerosis
Also 90% of cases are in WOMEN
What are the 3 core signs of Fibromuscular Dysplasia?
What is the main investigation done to confirm?
What is the main differential?
Three main subtypes
- Intimal, Medial and Perimedial- these are HISTOLOGICAL types
STRING of BEADS Appearance- seen in CATHETER ANGIOGRAPHY in the CAROTID or RENAL Arteries
If String of Beads with only GENERIC INFLAMMATORY SYMPTOMS- Polyarteritis Nodosa
Reno-vascular Hypertension is the most common sign of Renal Artery FMD
Other signs
- CKD that isn’t helped by ACE Inhibitors
- FLASH Pulmonary Oedema
- Renovascular Hypertension
What are the possible complications of Fibromuscular Dysplasia?
Head-related Vascular Complications
Dissection with Headache
Stroke
Horner’s Syndrome
Intracerebral Aneurysms with a risk of Subarachnoid or Intracerebral Headache
What is the management of Fibromuscular Dysplasia?
1) If Renovascular Hypertension- Antihypertensives
2) Percutaneous Angioplasty of Severe Stenoses
3) Reconstructive Surgery if it extends to Segmental Arteries
Whcih Nerve is affected in Foot Drip?
The Common Peroneal Nerve
What are the 4 causes of Foot Drop?
1) L4 Lesion (Radiculopathy)- Loss of INVERSION (which is a TIBIAL Function and is NOT lost if the Lesion is on the Common Peroneal Nerve itself)- Sciatica type Leg Pain also present
2) Distal Motor Neuropathy- Glove and Stocking Sensory Disturbance and loss of movements of the foot
3) Small Cortical Lesions- There may be OTHER Upper Motor Neuron features
4) Uncommonly- Intrinsic Cord Disease, Partial Sciatic Nerve Disease and Myopathy can mimic Foot Drop
What are the reflexes like in Motor Neurone Disease?
Reflexes would NOT be normal in Motor Neuron Disease
What signs are expected to be seen in Cauda Equina?
Loss of Bladder and Bowel Function
What is a risk factor for Foot Drop?
Plaster Cast Compression
What is Friedrich’s Ataxia?
What are the 4 signs?
Teenagers with Lower Limb Weakness and Gait Abnormalities (as it is INHERITED)
Also T1DM, Deafness and Low Visual Acuity
It is an Autosomal Recessive Trinucleotide Repeating Condition- so diagnosed via Genetic Analysis
It is a neurodegenerative disorder
Signs
1. Bilateral spastic paresis
2. Bilateral loss of proprioception and vibration sensation
3. Bilateral limb ataxia
4. Intention Tremor
- May be Cerebellar Signs (due to Spinocerebellar Tracts affected)
- Also- Hypertrophic Obstructive Cardiomyopathy, Reduced Visual Acuity, Type 1 Diabetes and Deafness
There will also be
1) High-arched Palate
2) Pes Cavus
3) Kyphoscoliosis
What is he management of Friedrich’s Ataxia?
It is Supportive
What are Typical Absence Seizures?
Up to 20 seconds of Daydreaming- Seizures begin and end suddenly
If it is >10 seconds, there may be motor signs (changed tone)
These should resolve by 18 years old
Usually provoked by HYPERVENTILATION
EEG= 3Hz Spike-wave Discharge
What are Atypical Absence Seizures?
Begin and End gradually
As they usually occur in children with Global Cognitive Impairment, it may be difficult to distinguish from Normal Behaviour
May go into Adulthood
Not provoked by Hyperventilation
EEG= <2.5Hz Slow Spike-wave
What are the features of Tonic Clonic Seizures?
Loss of Consciousness associated with an Initial Tonic Phase followed by Clonic Jerking
This may be followed by Transient Agitation
EEG= Generalised Polyspikes but usually diffused by Muscle Artefacts from the twitching
What is Guillain-Barre Syndrome?
It is an ASCENDING Inflammatory Demyelinating Polyneuropathy which typically occurs 1-3 weeks AFTER an Infection (Campylobacter, Mycoplasma, EBV)
What are the signs of Guillain-Barre Syndrome?
What type of motor neurone signs are seen in Guillain Barre
What is seen if it is severe?
Ascending Limb Weakness
May be Paraesthesia before this
If Severe- Respiratory Muscles may be affected and Type 2 Respiratory Failure
!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!There will be LOWER MOTOR NEURONE signs in the Lower Limbs- Hypotonia, Flaccid Paralysis, Areflexia
What investigations should be ordered in Guillain-Barre Syndrome?
What is seen on Spirometry, Lumbar Puncture, Blood Tests (ABG and Normal) and Nerve Conduction Studies?
Spirometry- Reduced FVC
Bloods- ABG (Type 2 Respiratory Failure) and Anti-Ganglioside Antibodies (Anti-GM1)
Lumbar Puncture- High Protein, Normal Cell Counts and Glucose (Albuminocytological Dissociation)
Nerve Conduction- DECREASED Nerve Conduction (Like Type 1 Charcot-Marie-Tooth)
What is the management of Guillain-Barre Syndrome?
What are the 3 features of Miller Fisher?
Monitoring Ventilation (Serial Spirometry and ABG)
Reduce risk of VTE
Protection of Pressure Areas
MEDICAL MANAGEMENT- IV Immunoglobulin, otherwise Plasmapheresis
Miller-Fisher Syndrome- Ataxia, Ophthalmoplegia and Areflexia (Eyes are affected first)- it is also PROXIMALLY PREDOMINANT
What causes Haemorrhagic Strokes?
They are caused by the weakening of cerebral vessels which leads to cerebral vessel rupture and haematoma formation
This leads to Neuronal Injury and Cerebral Oedema which causes the symptoms seen
What are the risk factors of a Haemorrhagic Stroke?
what are the 3 weak risk factors?
Strong Risk Factors
1) Age
2) Male
3) Family History of Haemorrhagic Stroke
4) Haemophilia
5) Cerebral Amyloid Angiopathy
6) Hypertension
7) Illicit Drug Use
8) Anticoagulation Therapy
9) Vascular Malformations
Weak Risk Factors
1) NSAIDs
2) Heavy Alcohol Use
3) Thrombocytopaenia
What is the management of Haemorrhagic Stroke?
Remember ABC
1) Anticoagulant Reversal (Stop them and give the reversal agents:
- For WARFARIN= Vitamin K, Prothrombin Concentrate
- For Dabigatran= Idarucizumab)
2) BP Control (only if MORBID, otherwise no need)
3) Control Neurosurgery
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- Neurosurgical and Neurocritical Care Evaluation due to the potential for Surgical Intervention (Decompressive Hemicraniectomy)
- Admission to Neuro ICU or Stroke Unit (patient may require Intubation and Ventilation or Invasive monitoring of ICPs)
- Aim to keep Blood Pressure <140/80 as poor Blood Pressure control in the Acute Stage is associated with Poor Outcomes
What is Hereditary Spastic Paraparesis?
Which tract is affected?
Which fibres as well?
Which limbs are affected?
It is a group of Inherited Disorders characterised by Lower Limb Spasticity
There is Axonal Degeneration of the Corticospinal Tracts (and sometimes in the Dorsal Column Pathways)
The Corticospinal Tracts to the Lower Limbs and the Fasciculus Gracilis Fibres (which is part of the Dorsal Column) from the lower limbs are the most severely affected, meaning the Lower Limbs are affected more severely than the Upper Limbs
What are the signs of Hereditary Spastic Paraparesis?
What is the power like?
What is the additional sign seen in these patients (think of the toes)
Progressive Gait Disturbance- SCISSOR GAIT
Spasticity of the Lower Limbs and Hyperreflexia of the Lower Limbs
Extensor Plantar Responses (Toe goes up)
POWER is USUALLY NORMAL/MILDLY Reduced
How is Hereditary Spastic Paraparesis diagnosed?
Clinical Findings and Genetic Analysis of the Known Mutations
How is Hereditary Spastic Paraparesis treated?
(Like Cerebral Palsy)
Symptomatic Relief- Baclofen, Botox and Physiotherapy
What is Internuclear Ophthalmoplegia?
It is an Ocular Movement Disorder caused by a lesion of the Medial Longitudinal Fasciculus which blocks the connection between the (1) Contralateral Sixth Nerve Nucleus and the (2) Ipsilateral Third Nerve Nucleus which affects the HORIZONTAL GAZE
What is the Medial Longitudinal Fasciculus responsible for?
It transmits information that is vital for Coordination of different eye movements
A lesion of the Medial Longitudinal Fasciculus impairs Horizontal Eye Movements
What are the signs of Internuclear Ophthalmoplegia?
There is Double Vision!!!!!!!!!!
Ipsilateral Impaired Eye Adduction
Nystagmus in the Abducting (Contralateral) Eye
If there is dissociation of the medial rectus function Horizontal Saccades and Convergence, this confirms a Medial Longitudinal Fasciculus Lesion
What are the 2 causes of Internuclear Ophthalmoplegia?
Vascular (Stroke)
Demyelination (Multiple Sclerosis)- More Commonly Bilateral
What is Intracranial Venous Thrombosis?
What are the 3 core signs (and others)
What kinds of risk factors would be present?
Intracranial venous thrombosis refers to occlusion of venous vessels in the cranial cavity
Signs (Throbbing headache, reduced consciousness and nausea for 1 day)
- Headache, Confusion, Impaired Vision, Nausea/ Vomiting
- Other Signs- Seizures, Reduced Consciousness, Focal Neurological Deficits, Cranial Nerve Palsies, Papilloedema
Risk factors- the same as DVT/ PE
What investigations should be ordered in Intracranial Venous Thrombosis?
MRI Venogram- Filling Defect (The Empty Delta Sign)
Non-contrast CT is mostly normal but may show Hyperdensity in the Affected Sinus (usually Superior Sagittal)
D-Dimer is usually raised
What is the management of Intracranial Venous Thrombosis?
LMWH
What are the signs of Extradural Haemorrhage?
What is seen on CT?
Trauma
Which artery is affected and how is it treated?
Between Skull and Dura Mater of the Meninges
Tearing of the Middle Meningeal Artery
1) Headache
2) CONTRALATERAL Hemiplegia
3) Biconvex Haematoma on CT
Management- Ligation of the Bleeding Artery
What are the signs of Subdural Haemorrhage?
What is seen on CT
What is the management?
Old Age/ Alcohol/ Gradual
Between Dura Mater and Arachnoid Mater
Presents MORE GRADUALLY than Extradural Haemorrhage
1) Headache
2) Confusion
3) Usually in OLDER PATIENTs
4) Crescent Haematoma on CT
Management- Burr hole Craniostomy
What are the signs of Subarachnoid Haemorrhage?
What are the guidelines on imaging?
What is the management?
There may also be PUPIL SIGNS
Haemorrhage is underneath the Arachnoid Mater
1) Severe Headache- Thunderclap Occipital
2) Seizures, Decreased Consciousness, Neurological Deficits
3) MENINGISM and NAUSEA and VOMITING
4) Spontaneous or cos of Trauma, Spontaneous is usually caused by Burst Aneurysm of a Vessel in the Circle of Willis
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1) FIRST- NON CONTRAST CT- a White Area in the Centre of the Brain (basal cistern and sulci) expanding bilaterally
- if CT is Negative AFTER 6 HOURS, LP should be done 12 HOURS AFTER Symptoms- Blood in CSF or Yellowing of CSF if Older Bleeds (XANTHOCHROMIA)
- if CT is Negative WITHIN 6 hours of symptoms, do NOT do an LP
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Management- Endovascular Coiling and Nimlodipine/ Surgical Clipping so REFER to NEUROSURGERY ASAP
What should be done with regards to Warfarin Therapy in the event of a Head Injury?
Stop the Warfarin
Give Vitamin K 5mg IV
Prothrombin Complex Concentrate
What can help to manage a raised ICP?
Hyperventilation
This increases CO2 and this causes Vasoconstriction of the Cerebral Arteries and this decreases ICP
What is the most common cause of Intracranial Haemorrhage?
Hypertension
Why is Nimlodipine given for Subarachnoid Haemorrhages?
To Prevent Vasospasm-induced Cerebral Ischaemia
What is the most common type of Stroke?
85% are Ischaemic Strokes
15% are Haemorrhagic Strokes
What are the main causes of Ischaemic Strokes?
50% of them are caused by Large Vessel Atherosclerosis- Carotid Artery Stenosis
20% are Cardio-embolic- Atrial Fibrillation
What defines a Total Anterior Circulation Infarct (TACI) Stroke?
What arteries does it involve?
1) Contralateral Hemiplegia or Hemiparesis AND
2) Contralateral Homonymous Hemianopia AND
3) Higher Cerebral Dysfunction (Aphasia, Neglect)- a TACI involves the Anterior AND Middle Cerebral Arteries on the affected side
What defines a Partial Anterior Circulation Infarct (PACI) Stroke?
2 of the TACI signs OR
1) Higher Cognitive Dysfunction ALONE
2) Can involve the Anterior OR Middle Cerebral Arteries on the affected side
What defines a Lacunar Anterior Circulation Infarct (LACI) Stroke?
What 3 things should not be present?
Pure Motor Stroke, Pure Sensory Stroke, Sensorimotor Stroke or Ataxic Hemiparesis
So LESS SEVERE version of the other strokes
There should be NO
1) Visual Field Defect
2) Higher Cerebral Function
3) Brainstem Dysfunction
LACI affects small deep perforating arteries which typically supply the Internal Capsule or Thalamus
What defines a Posterior Circulation Infarct (POCI) Stroke (5)?
Which arteries are involved?
1) Cerebellar Dysfunction OR
2) Conjugate Eye Movement Disorder (Two eyes don’t move in sync) OR
3) Bilateral Motor/ Sensory Deficit OR
4) Ipsilateral Cranial Nerve Palsy with Contralateral Motor/ Sensory Deficit
5) Cortical Blindness/ Isolated (as in no other symptoms) Homonymous Hemianopia
Involves the Vertebrobasilar Arteries and the associated branches
What are the 4 types of Posterior Stroke Syndromes?
1) Basilar Artery Occlusion- Likely to present with Locked In Syndrome (Quadriparesis with Preserved Consciousness and Ocular Movement), Loss of Consciousness or Sudden Death
2) Wallenberg’s Syndrome (Lateral Medullary Syndrome) causes Ipsilateral Horner’s Syndrome, Ipsilateral Loss of Pain and Temperature on the Face and Contralateral Loss of Pain and Temperature in the Body
3) Lateral Pontine Syndrome due to Anterior Inferior Cerebellar Artery Stroke. This is similar to Lateral Medullary Syndrome with involvement of Pontine Cranial Nerve Nuclei
4) Weber’s Syndrome/ Medial Midbrain Syndrome (Paramedian branches of the Upper BASILAR and Proximal POSTERIOR CEREBRAL Arteries)- this causes an IPSILATERAL Oculomotor Nerve Palsy and CONTRALATERAL Hemiparesis
What is the Acute Management of Ischaemic Stroke?
Which 2 particular contraindications to Thrombolysis should you bear in mind?
First Things to do-
1) ABCDE first
2) Depressed Consciousness= Airway Protection
Swallowing Impairment= Aspiration Precaution
Haemorrhagic or Ischaemic?
1) The Subsequent management depends on whether it is ISCHAEMIC or HAEMORRHAGIC. Do a NON-CONTAST CT Scan first when they arrive to differentiate between these
2) The Most Sensitive test to figuring out what kind of stroke it is is a Diffuse Weighted MRI- this is used if the diagnosis is unclear or CT is not possible
For ISCHAEMIC-
1) Alteplase (activates tissue plasminogen) if it is WITHIN 4.5 HOURS of symptom onset and if there are NO CONTRAINDICATIONS to Thrombolysis (including >50% of Artery affected as this can become Haemorrhagic/ also including a GCS drop)
2) Do DECOMPRESSIVE HEMICRANIECTOMY if they are <60 years old with severe symptoms of MCA Stroke (severe Swelling)/ reduced consciousness
3) Mechanical Thrombectomy if:
- Anterior Circulation Stroke presenting WITHIN 6 HOURS as long as they have a good baseline functional status and lack of Significant Early Infarction on CT
- Posterior Circulation Stroke presenting within 12 HOURS
4) Aspirin-
= If Hyperacute treatment is not offered- 300mcg once every day for 2 weeks (so NOT Long Term)
= If Hyperacute treatment is offered- start 24 Hours after treatment following a repeat CT Head to rule out Haemorrhagic Stroke
What investigations should be ordered straight after an Acute Stroke?
Used to define the cause of the stroke and to quantify the vascular risk factors
-Ischaemic Stroke-
1) Carotid Ultrasound to Identify Carotid Artery Stenosis
2) CT/MR Angiography to Identify Intracranial and Extracranial Stenosis
3) Echocardiogram if a Cardio-embolic Source is suspected (AF)
4) In YOUNG PATIENTS- Vasculitis Screen/ Thrombophilia Screen
-Haemorrhagic-
1) Serum Toxicology Screen= Sympathomimetic Drugs like Cocaine are strong Risk Factors for Haemorrhagic Stroke
Further Investigations (Vascular Risk Factors)-
1) Serum Glucose (screen for Diabetes)
2) Serum Lipids (Cholesterol)
What is the Chronic (Long Term) Management of Stroke?
HALTSS
- Hypertension- There is NO BENEFIT in lowering the blood pressure acutely (as this may IMPAIR CEREBRAL PERFUSION) unless there is MALIGNANT HYPERTENSION (>180). Start Antihypertensives 2 WEEKS after a stroke
- Antiplatelets- 75mg of Clopidogrel Once a Day. If patients have Ischaemic Stroke due to ATRIAL FIBRILLATION, Start Warfarin or a DOAC 2 WEEKS after the stroke
- Lipid-Lowering Therapy- 20-80mg Atorvastatin once a night (whatever their cholesterol level is, give this)
- Tobacco- stop smoking
- Sugar- Screen for Diabetes
- Surgery- If Ipsilateral Carotid Artery Stenosis, Refer for CAROTID ENDARTERECTOMY
Also Rehab and Therapy
In a Stroke, which brain areas are associated with which Cranial Nerve Palsies?
Mid brain- CN 1-4
Pons- CN 5-8
Medulla- CN 9-12
All CRANIAL NERVE LESIONS are IPSILATERAL to the Stroke Location, APART from the TROCHLEAR NERVE which is Contralateral (Right Midbrain lesion= Left Trochlear Lesion)
What are the signs of a Middle Cerebral Artery Stroke
What is seen if the left MCA is affected?
1) Contralateral Hemiplegia (Upper affected more than Lower)
2) Contralateral Homonymous Hemianopia with NO MACULAR Sparing
3) Contralateral Facial Weakness
4) Wernicke’s Dysphasia (if LEFT MCA)- Slurred Speech
MCA supplies the LATERAL PART of the CEREBRAL HEMISPHERE
What are the signs of a Posterior Cerebral Artery Stroke?
1) Contralateral Homonymous Hemianopia
MACULAR SPARING
PCA supplies the OCCIPITAL LOBE
What are the signs of a Posterior Inferior Cerebellar Artery Stroke?
Lateral Medullary Syndrome (Brainstem Infarct)
Signs
- ATAXIA
- Ipsilateral Horner’s Syndrome/CRANIAL NERVES/ Facial Numbness
- Contralateral limb sensory loss
- Vomiting, Vertigo, Nystagmus, Soft Palate Paralysis
What are the signs of an Anterior Cerebral Artery Stroke?
1) Contralateral Leg Weakness
2) Contralateral MILD Arm Weakness
Not seen= Dysphasia, Visual Defects
What kind of Stroke should be suspected if the patient has DANISH signs (Dysdiadochokinesia, Ataxia, Nystagmus, Intention Tremor, Slurred Speech, Hypotonia) and if there is VOMITING?
Cerebellar Stroke
due to a Posterior Circulation Infarction
How can BPPV and Menieres be differentiated?
BPPV- vertigo <1 minute, Menieres- vertigo up to 1 hour
BPPV is triggered by MOVEMENT- like rolling in bed
Meniere’s- Hearing Loss may be there
What is the treatment of BPPV?
Epley Manouevre (Dixie-Hallpike is only for assessment)
Brandt-Daroff Exercises
Antihistamines
What should be done if a patient has a Crescendo TIA? (2 TIAs in 1 week)
Aspirin 300mcg and review in TIA Clinic in 24 hours
What is the most common Ischaemic Stroke?
Middle Cerebral Artery Stroke
What are the signs of an Internal Capsule Stroke?
MAINLY MOTOR (and maybe Sensory Symptoms) Contralateral
What is a patient’s modified Rankin score if they can’t walk?
4-5
What are some contraindications to Thrombolysis?
INR>1.7
Unknown Time of Symptom Onset
What type of Stroke leads to LOCKED IN SYNDROME (tetraplegia)?
Basilar Artery Stroke
What are the signs of Third Nerve Palsy?
Paralysis of the Oculomotor Nerve
- Eye Looking Down and Out
- DOUBLE VISION
- Ptosis/ Proptosis/ Fixed Pupil Dilatation (if the lesion is Surgical and not Medical)
Surgical- Compressive Lesions (Posterior Communicating Artery Aneurysm)
Medical- Non-compressive (Multiple Sclerosis, Vascular causes (Diabetes or Hypertension)), Vasculitis
What are the causes of Fourth Nerve Palsy?
What is the Diplopia like?
Paralysis of the Trochlear Nerve (Superior Oblique Muscle)
- Eye looking Up and In
- Double Vision in VERTICAL PLANE and Hypertropia
Two Commonest Causes= Ocular Trauma and Diabetes Mellitus
What are the causes of Sixth Nerve Palsy?
Paralysis of Abducens Nerve (Lateral Rectus Muscle)
- Double Vision in HORIZONTAL PLANE
- It is EASILY compromised if High ICP
Commonest Causes
- Diabetic Neuropathy
- Stroke
- Infection
- Trauma
What is Jugular Foramen Syndrome?
CONTRAlateral CN 9, 10 and 11 Palsies with
1) Soft Palate
2) Uvula Deviation
3) Weakness of Head Twisting
4) Weakness of Shoulder Shrugging
Causes-
- Tumours (Neurofibroma, Meningioma, Glomus Jugulare)
- Infiltration (CNS Lymphoma, Carcinomatosis)
- Sarcoidosis
- Spreading of Middle Ear Infection
What is seen in Lesions in the Cerebellopontine Angle?
Like Acoustic Neuroma (Vestibular Schwannoma)
CN 5, 7 and 8 lesions
CN5- absent corneal reflex (CORNEAL REFLEX)
CN7- facial palsy
CN8- vertigo, unilateral sensorineural hearing loss, unilateral tinnitus (Ear Stuff)
What kind of Spinal tumours lead to Unilateral Bulbar Palsies due to the compression of CN 12?
Foramen Magnum Tumours (which is the exit of the spinal cord)
What is Lambert Eaton Myasthenic Syndrome?
It is an AUTOIMMUNE CONDITION against Presynaptic Voltage Gated Calcium Channels
These channels cause an Influx of Calcium ions which causes ACh release into the synapse
What are the risk factors for Lambert-Eaton Myasthenic Syndrome?
1) Underlying Malignancy (seen in HALF of the patients- usually the malignancy seen in SMALL CELL LUNG CANCER)
2) Underlying Autoimmune Conditions (seen in a QUARTER of patients)
What are the 5 signs of Lambert-Eaton Myasthenic Syndrome?
What is it usually associated with?
How can this be differentiated from Myasthenia Gravis (2)?
Limb Weakness (But NORMAL SENSATION) and Autonomic Features
Associated usually with SMALL CELL LUNG CANCER (also breast and ovarian cancer)
Weakness IMPROVES after Exercise- unlike Myasthenia Gravis. Also the EYES are usually not affected here
Legs open up after Walking
- Weakness starting with LOWER LIMBS
- Xerostomia (Dry Mouth)
- Orthostatic Hypotension
- Impotence (Erectile Dysfunction) AND CONSTIPATION
- HYPOREFLEXIA
On Examination
- REDUCED or ABSENT Reflexes and there will be brief contractions of the relevant muscles
What investigations should be ordered if Lambert-Eaton Myasthenic Syndrome is suspected?
Bloods for Anti-VGCC Antibodies
Nerve Conduction studies= DOUBLING of Muscle Action Potential Amplitude with Exercise
Chest Xray- rule out Lung Cancer
What is the management of Lambert-Eaton Myasthenic Syndrome?
Mainstay- manage the Underlying cause- Small Cell Lung Cancer
1) IMMUNOSUPPRESSION (PREDNISOLONE and AZATHIOPRINE)
2) Amifampridine- blocks Presynaptic POTASSIUM Channels which Prolongs the action potential and prolongs the opening time of the VGCC
2) If severe respiratory impairment or Bulbar Weakness-
- Intubation
- Ventilation
- Plasma Exchange/ IV Immunoglobulin
What causes Lyme Disease?
Borrelia Burgdorferi- transmitted via hard ticks in Wooded Areas
Birds and Deer are Reservoirs for the disease
What are the 3 stages of Lyme Disease?
Stage 1- Localised Disease lasting several weeks (Flu, Skin, Lymph nodes)
1) Tick Bite (remembered in 75% of patients)
2) Flu Like Symptoms- Fatigue, Headache, Anorexia, Myalgia
3) Regional Lymphadenopathy
4) Erythema Migrans (BULLS EYE RASH) (Painless but may itch- CIRCULAR TARGET SHAPED)
5) Borrelia Lymphocytoma (Blue Patch on the Earlobe, Nipple or Scrotum)
Stage 2- Weeks to months after the bite (Head, Heart)
1) Continued Flu-like symptoms
2) Neuroborreliosis- Facial Nerve (single or bilateral) and other Cranial Nerve Palsies, Aseptic Meningitis, Encephalitis Polyradiculitis
3) Cardiovascular- Myocarditis, Heart Block and other Arrhythmias, Pericarditis
4) Early painful Arthritis
Stage 3- Months to years after bite (Joints, Nerves, Fatigue, Swelling)
1) Arthritis- Usually LARGE joints like the Knee- usually non-destructive
2) Late Neurological Disorders- Polyneuropathy, Chronic Encephalomyelitis, Dementia, Psoriasis
3) Acrodermatitis Chronica Atrophicans- Blue-Red Discoloration and Swelling at Extensor Surfaces- may be associated with Peripheral Neuropathy
4) Controversial cause of Fibromyalgia and Chronic Fatigue Syndromesma
How is Lyme Disease diagnosed?
1) Depends on the Stage of the Disease-
- If Early Disease- Erythema Chronicum Migrans (diagnosis can be made Clinically) (so NO NEED FOR INVESTIGATIONS- just give antibiotics)
- If Stage 2 or 3- Serology is needed as Borrelia Species can not be cultured
2) ELISA and Western Blot can be done for Stage 2 and 3 but they can be negative
3) If it is negative initially, but the symptoms are still there, retest 3-4 weeks later
4) If they have arthritis, a Synovial Fluid Sample can be obtained for PCR Borrellia DNA Testing
What is the management of Lyme Disease?
Make sure the tick is removed and the bite area is not infected
Treat with DOXYCYCLINE for 2 WEEKS (can give CEFUROXIME or AMOXICILLIN as well). 3-4 weeks if there are Neurological, Cardiac or Arthritis
There may be Post-treatment Lyme disease Syndrome- occurs in 5-15%- where symptoms persist for months after the treatment and gradually improve over 6 months to 1 year
What are signs of Neuroborreliosis in Lyme Disease?
Pain and Weakness in an arm, followed by Headache following Rural Trip
Also p much any Nervous Symptom
What is Meralgia Paresthetica?
Motor Examination is NORMAL
It is a Syndrome caused by the Compression of the Lateral Cutaneous Nerve of the Thigh Underneath the Inguinal Ligament
NO Motor Weakness
There will be SHOOTING PAINS going up the UPPER LEG- may be replicated by palpating above the ASIS
- and NUMBNESS and loss of HEAT and TOUCH SENSATION
What is the management of Meralgia Paresthetica?
Usually self limiting
but Weight Loss and Loose fitting underwear can help
If Refractory- Steroid Injections
What are the signs of a Migraine?
A Unilateral Throbbing Headache preceded by an Aura such a VISUAL (lines, zigzag) or SENSORY (paresthesia spreading from fingers to face)
Associated with Photophobia and Phonophobia
How is Migraine diagnosed?
Aura helps confirm the diagnosis
Otherwise-
At least FIVE Headaches lasting 4-72 hours with
- Nausea/ Vomiting or Photophobia/ Phonophobia AND 2 of
1) Unilateral Headache
2) Pulsing Headache
3) Impaired or Worsened by Activities
What is the management of Migraines?
What are the 5 different medications that can be given (3 are for prophylaxis, 2 are for attacks)
What must be avoided
1) Avoid the triggers
Prophylaxis-
- Propanolol for prophylaxis UNLESS ASTHMA
- Topiramate (avoid in young females looking to get pregnant) or Amitriptyline for Prophylaxis
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Acute-
- Oral Triptan like Sumatriptan for an ACUTE ATTACK UNLESS ISCHAEMIC HEART DISEASE)- take ONCE the headache starts and NOT during the Aura phase- PLUS Paracetamol or NSAID
- Otherwise Antiemetic like Metoclopramide/ Prochlorperazine can be given
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6) Stop COCP as this increases risk of ISCHAEMIC STROKE
What is Mononeuritis Multiplex?
Basically 2 or more unrelated nerves are inflamed
It is sometimes called Polyneuritis Multiplex
SUSPECT IN DIABETICS
It is a type of Peripheral Neuropathy which is characterised by Simultaneous or Sequential involvement of Individual Non-Contiguous Nerve Trunks
It evolves over days to years and typically presents with Acute or Subacute loss of Sensory and Motor Function of Individual Nerves
What are the signs of Mononeuritis Multiplex?
Also look for Multiple Incidents of Neuropathy in a Diabetic Patient, accompanied by gradual recovery and affecting both SENSORY and MOTOR FUNCTION
MANAGEMENT Ensure TIGHT GLUCOSE CONTROL in Diabetics/ treat the underlying cause
Usually Asymmetric, however it becomes SYMMETRICAL as it progresses so it is difficult to differentiate from Polyneuropathy
Basically any two unrelated nerves being affected (where they also recover over time) in the context of diabetes usually
What are the causes of Mononeuritis Multiplex?
1) Diabetes mellitus
2) Vasculitis: polyarteritis nodosa, granulomatosis with polyangitis, and eosinophilic granulomatosis with polyangitis
3) Immune-mediated diseases, such as rheumatoid arthritis and systemic lupus erythematosus
4) Infections: Leprosy, Lyme disease, Parvovirus B19, HIV
5) Sarcoidosis
6) Cryoglobulinaemia
7) Reactions to exposure to chemical agents, including trichloroethylene and dapsone
8) Rarely, following the sting of certain jellyfish, such as the sea nettle
What are the 4 types of Amyotropic Lateral Scleorosis (ALS) Motor Neurone Disease?
Spinal ALS (the classic MND Syndrome)
Bulbar ALS (Early Tongue and Bulbar Involvement)
Progressive Muscular Atrophy (only Lower Motor Neuron features)
Primary Lateral Sclerosis (only Upper Motor Neuron features)
What are the signs of Motor Neuron Disease?
What 3 things suggest a Poor Prognosis?
What suggests a better chance of survival?
What are the Upper and Lower Motor Neurone Signs?
What is NOT seen in Motor Neurone Disease? (3)
1) Chronic and Progressive Bilateral Weakness and Scissor Gait
2) Poor Prognosis if: Early Bulbar and Respiratory Muscle Involvement, and older age of onset. More prominent Lower Motor Neuron features= more protracted survival
3) Combination of Upper and Lower Motor Neuron signs
- Standard Upper Motor Neuron signs= Spasticity, Hyperreflexia (Brisk), UPGOING Plantars (Babinksi response) (but usually going down in MND), SCISSOR Gait
- Standard Lower Motor Neuron signs= Fasciculations and Later Atrophy
4) Usually the Eye and Sphincter Muscles are spared until late in the disease
5) There is NO SENSORY DISTURBANCE
How is Motor Neuron Disease treated (2)?
What is given for the Drooling in MND and the Spasticity and Contractures in MND?
1) DMARD- Riluzole- Antiglutamatergic (only extends life expectancy by a few months)
2) Non-invasive Ventilation (BIPAP at NIGHT)if Type 2 Respiratory Failure
3) Treatment focus is Supportive (like PEG) and best coordinated via MDT
4) Simple Analgesia
5) Spasticity and Contractures= Baclofen and Botox
6) Drooling= give Anticholinergics (TCAs), and then Supportive Feeding via NG/ PEG can be indicated
What is Multiple Sclerosis?
It is a disease that MOSTLY AFFECTS WOMEN- usually 30 years old
It is a CD4 mediated Destruction of Oligodendronal Cells and there is also a Humoral response to Myelin-binding Protein
What are the 2 types of Multiple Scleoris?
Relapsing-remitting (which becomes secondarily progressive)
Primary Progressive
- Relapsing-Remitting MS makes up to 80% of cases
What are the signs of Multiple Sclerosis?
Transient Nerve symptoms- relapsing remitting
Bear in mind, unlike a Cerebrovascular Disease, Multiple Sclerosis can have MULTIPLE POINTS OF DISEASE (Multifocal)
Also it is a disease of the CENTRAL NERVOUS SYSTEM- so signs pointing to peripheral disease make MS unlikely (like Lower Motor Neuron Signs)
1) LETHARGY and Sensory Disease (Patchy Paresthesia)
2) Optic Neuritis (Loss of Central Vision and Painful Eye Movements)
- Eye pain especially in Eye Movement
- Blurred Vision
- Red desaturation
3) Internuclear Ophthalmoplegia (Lesion in the Medial Longitudinal Fasciculus of the Brainstem- which helps CN3 and CN4 talk to each other to keep in sync)
- Seen in BOTH EYES (one eye indicates a stroke)
- Eyes stop talking to each other so are out of sync when looking left and right
4) Subacute Cerebellar Ataxia (usually on a RELAPSE) and TREMOR
5) Spastic Paraparesis (transverse Myelitis, including Lhermitte’s sign)
- Stiffness in the legs
- Reduced power in legs
How is Multiple Sclerosis diagnosed?
At least 2 of
1) History and Examination
2) Imaging- Juxtacortical and Periventricular White Matter Lesions seen on MRI (Spatially Distributed Gadolinium enhancing plaques)
- Type 2 HYPERDENSE LESION
Also DAWSON’s FINGERS
3) Oligoclonal Bands in the CSF
- These are the various IMMUNOGLOBULINS in the CSF and point to an Autoimmune reaction
- These are sensitive for Multiple Sclerosis but can also be due to Lyme Disease, SLE and Neurosarcoid
What is the Acute Management of Multiple Sclerosis?
What are the 2 options for acute relapses?
What is given to reduce the chances of a relapse?
What is given for the Bladder Issues and the Spasticity?
1) Glucocorticoids- 1g IV Methylprednisolone every 24 hours for 3 days- EXCLUDE INFECTIONS first
2) If there is no response to Glucocorticoids- PLASMA EXCHANGE
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3) DMARDS for Relapse- Natalizumab (Fingolimod, Glatiramer Acetate)
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4) Bladder issues- Ultrasound first then catheterise if significant residual volume, and anticholinergics if not
5) Spasticity- Baclofen and Gabapentin
6) Check Urine Dip and Routine Bloods to rule out any infections
What is the Chronic Management of Multiple Sclerosis (RELAPSING REMITTING, not Primary Progressive)?
Disease Modifying Therapies and Symptomatic Treatment
-Disease Modification
1) Injectables- BETA INTERFERON and GLATIRAMER
2) Oral Agents- DIMETHYL FUMARATE, TERIFLUNOMIDE and FINGOLIMOD
3) Biologics- NATALIZUMAB and ALEMTUZUMAB
- Symptomatic Treatment
1) Physiotherapy
2) Baclofen and Botox for Spasticity
3) Modafinil/ Amantidine and Exercise Therapy for Fatigue
4) Anticholinergics for Bladder Dysfunction
5) SSRIs for Depression
6) Sildenafil for Erectile Dysfunction
7) Clonazepam for Tremor
What is seen in the Lumbar Puncture of Multiple Sclerosis?
What is usually raised in MS?
Distinct bands of IgG on Western Blot (Oligoclonal Bands)
Total Protein may also be mildly raised
What is Myasthenia Gravis?
It is an autoimmune condition where there are antibodies produced against nAChR in Muscle Fibres
What are the 4 signs of Myasthenia Gravis?
Weakness in (Limbs, Eyes, Face, End of the Day)
1) Limbs
2) Extra-ocular Muscles (Drooping Eyelids and Diplopia)
- Bilateral Ptosis- may be unilateral
3) Facial Muscles (Difficulty Smiling or chewing)
- Myasthenic Snarl
- Head Droop
- Bulbar Features (Nasal speech, Dysphagia, Dysarthria, Drooling, SLurring)
4) Symptoms worse after Prolonged Movement/ at the End of the Day
What 5 investigations should be ordered in Myasthenia Gravis?
1) Bedside-
- The Ice Pack Test can help- apply an ice pack to the eyes for 2-5 minutes. If the patient no longer has Ptosis, then this suggests Myasthenia Gravis
2) Blood Tests-
- For Serum AChR Antibody
- Order Muscle-Specific Tyrosine Kinase if AChR is negative but you still think it is Myasthenia Gravis
3) Imaging-
- CT Chest- 65% have Thymic Hyperplasia, some have Thymoma
4) Repetitive Nerve Stimulation (>10% fall in Muscle Action Potential between 1st and 4th Action Potential) (Unlike Lambert Eaton)
5) Tensilon Test- Edrophonium IV helps with weakness
If MYASTHENIC CRISIS- Pulmonary Function Tests- If FVC is 15 or less- then MECHANICAL VENTILATION ASAP
What is the Management of Myasthenia Gravis?
Conservative
- Occupational Therapist, Physiotherapy, Speech and Language Therapy if Dysarthria
Medical
- Acetylcholinesterase Inhibitors (Pyridostigmine or Neostigmine)- Side Effects (Liquid Loss everywhere= Diarrhoea, Salivation, Lacrimation, Urinary Frequency
(+ this next bit is the same as MS Acute)
- Immunosuppression (Steroids)
- If Steroids do NOT WORK- IV Immunoglobulin/ Plasmapharesis
Surgical
- Thymectomy
What drugs worsen Myasthenia Gravis?
Beta Blockers
Lithium
Antimalarials
Antibiotics
Psychiatric Drugs
What is Myotonic Dystrophy?
It is a TRINUCLEOTIDE REPEAT Disorder which affects Muscle-Specific Chloride Channels
It is AUTOSOMAL DOMINANT
What are the 5 signs of Myotonic Dystrophy?
Frontal Balding
Percussion Myotonia- Difficulty letting go when holding on to things
They usually present in their 20s
HEAD to TOE- Face, Speech, Neck, Hands, Others
1) Face- Frontal Balding, Myopathic Faces (Long and Thin face), Bilateral Ptosis, Cataracts
2) Speech- Dysarthria (Due to Myotonic Tongue and Pharyngeal Muscles)
3) Neck- Wasting of Sternocleidomastoid
4) Hands- Distal Wasting and Weakness, Slow Relaxing Grip, Percussion Myotonia (Thumb Flexion on Percussion of the Thenar Eminence)
5) Internal Features= Insulin resistance/ Metabolic Syndrome, Cardiomyopathy/ Arrhythmia, Testicular Atrophy
How is Myotonic Dystrophy diagnosed?
Genetic Analysis
How is Myotonic Dystrophy managed?
Symptom control and complication management
There is no cure at the moment
What Kidney condition can lead to Subarachnoid Haemorrhage due to Berry Aneurysm?
Polycystic Kidney Disease
What are the signs of Neuromyelitis Optica (Devic’s Disease)?
The Triad
1) Optic Neuritis
2) Transverse Myelitis (Muscle Weakness, Bladder and Bowel Problems, Sexual Problems, Unusual sensations and numbness)
3) Positive NMO IgG (antibody against Aquaporin 4)
- It is usually Relapsing-Remitting- similar to multiple sclerosis
What is Normal Pressure Hydrocephalus?
It is a Reversible cause of Dementia that is usually seen in Elderly patients
It is thought to be due to the Impaired Absorption of CSF at the Arachnoid Villi
This can be due to a Bleed or Head Injury
It may also be Idiopathic
What are the signs of Normal Pressure Hydrocephalus?
Dementia with Urinary Incontinence
Triad
1) Urinary Incontinence
2) Dementia
3) Gait Abnormality which develops over several months
What investigations should be ordered in Normal Pressure hydrocephalus?
MRI Head- Enlarged Ventricles
Definitive Diagnosis= Walking and Cognitive Assessment before and after a large volume of CSF Removal through LUMBAR PUNCTURE
- Patients that improve after CSF Removal are candidates for surgery
What is the management of Normal Pressure Hydrocephalus?
Surgery if fit- Ventriculoperitoneal Shunting
Conservative if not- CSF Taps
What is the Epidemiology of Optic Neuritis?
Usually in adult women who live in high LATITUDES
What are the 3 causes and 4 features of Optic Neuritis?
Central Scotoma then Monocular Vision Loss
Pain Behind Eye
Colour Vision affected
RAPD
Causes
1) Demyelinating Conditions
2) Autoimmune Disorders
3) Infectious Conditions
Features
1) Visual Loss
2) Periocular Pain
3) Dyschromatopsia (Colour Blindness)
4) Relative Afferent Pupillary Defect
What is the management of Optic Neuritis?
Intravenous Methylprednisolone
Contraindicated= ORAL PREDNISOLONE ALONE
What are the CORE features of Parkinson’s Disease?
1) Bradykinesia
2) An ASYMMETRIC Pill Rolling Tremor
3) Lead Pipe Rigidity
4) (Leadpipe rigidity + Tremor= COGWHEELING)
5) Parkinsonian Gait (Small, Shuffling Steps/ Flexed Posture)
6) Hypomimic Faces
7) Festination= Patient picks ups speed when they are travelling in a particular direction
8) Postural Instability= LATE FEATURE
What are the Non-Motor Features of Parkinson’s?
1) Autonomic Involvement= Constipation, Postural Hypotension, Erectile Dysfunction
- If Autonomic Dysfunction is PROMINENT- rule out Multiple System Atrophy
2) Olfactory Loss
3) REM Behavioural Disorder- patient performs violent reenactments of their dreams
4) Psychiatric Features- Depression, Anxiety, Hallucinations. If Cognitive Dysfunction is early= Dementia with Lewy Bodies
What is the treatment of Parkinson’s?
LEVODOPA IF FUNCTIONAL IMPAIRMENT
Levodopa/ Dopamine Agonists (Bromocriptine, Cabergoline, Ropinirole, Rotigotine, Apomorphine)/ Monoamine Oxidase Inhibitors (ENTACAPONE) if NO Functional Impairment
Adjunctive Therapy to help with Side Effects= Anticholinergics and the Antiinfluenza Drug (Amantidine)
Monoamine Oxidase Inhibitors (Entacapone) and COMT Inhibitors (Selegilline) reduce Dopamine Breakdown so can be used
What excludes Parkinson’s?
Failure to respond to 1-1.5g of Levodopa daily
What are the side effects of Levodopa and how can they be reduced?
Hypotension
Restlessness
GI Upset
Dopamine EXCESS= Psychosis
Drug-Induced Dyskinesia (Erratic Movements)
Peripheral DOPA Inhibitor- CARBIDOPA can be given
What drugs can make Parkinson’s Worse?
Metoclopramide
Promethazine
Prochlorperazine
When should drug-induced Parkinson’s be suspected?
If following a drug AND if BILATERAL (as Parkinsons is normally UNILATERAL to begin with)
What is the ON/OFF Phenomenon in Levodopa use?
It fluctuates in EFFICACY throughout the day
What should be considered with Parkinson’s Medications?
They are TIME-CRITICAL
- They NEED to be given at the specific time or the patient may come into harm
What are some examples of Dopamine Agonists?
Bromocriptine
Cabergoline
Ropinirole
Rotigotine
Apomorphine
What is the triad of Parkinsonism?
Resting Tremor
Hypertonia
Bradykinesia
What are the 4 Parkinson-plus Syndromes and their symptoms?
1) Progressive Supranuclear Palsy
- Parkinsonism and Vertical Gaze Palsy and Cognitive Impairment
2) Multiple System Atrophy
- Parkinsonism and Early Autonomic Clinical Features= Postural Hypotension, Incontinence and Impotence
3) Cortico-basal Degeneration
- Parkinsonism and involves Spontaneous Activity by an Affected Limb, or Akinetic Rigidity of that Limb
4) Lewy Body Dementia
- Parkinsonism and Fluctuations in Cognitive Impairment and Visual Hallucinations (often before Parkinsonism)
In addition to the Parkinson-plus Syndromes, what other conditions are differentials in Parkinson’s?
1) Small Vessel Diseases of the Basal Ganglia and MotorPlanning Areas (Tremor Negative and Localised to Lower Limbs)
2) Drugs (Antipsychotics and Metoclopramide))
3) Wilson’s Disease (Liver Disease and Kayser-Fleischer Rings)
What should be done if a patient presents with facial drooping WITHOUT forehead sparing?
So it is not a stroke as it is not forehead sparing
Otoscopy (look for Ramsay Hunt)
Prednisolone within 72 hours (assume it is Bell’s Palsy)
What are the Risk factors for a Spontaneous Subarachnoid Haemorrhage?
Causes of Berry Aneurysms-
- Hypertension
- Polycystic Kidney Disease
- Ehler’s Danlos
- Coarctation of the Aorta
Also
- Pituitary Apoplexy
- Arteriovenous Malformation
