neuro Flashcards
1
Q
pathophysiology parkinsons disease
A
progressive reduction of dopamine in the basal ganglia of the brain
2
Q
sx parkinsons disease
A
Classic triad: Resting tremor (pill rolling, unilateral), Rigidity (cogwheel), Bradykinesia
other: Depression, Sleep disturbance and insomnia, Loss of the sense of smell (anosmia), Postural instability, Cognitive impairment and memory problems
3
Q
mx parkinsons diease
A
Levodopa: less effective over time, SE=dyskinesias
COMT Inhibitors e.g. entacapone
Dopamine Agonists e.g. bromocryptine. SE=pulmonary fibrosis
4
Q
parkinsons plus syndromes
A
Progressive supranuclear palsy:
Multiple system atrophy
Cortico-basal degeneration:
Lewy body dementia:
5
Q
progressive supranuclear palsy
A
Parkinisonism and vertical gaze palsy.
6
Q
multiple system atrophy
A
Parkinisonism and early autonomic clinical features such as: postural hypotension, incontinence, and impotence
7
Q
cortico basal degeneration
A
parkinisonism and involves spontaneous activity by an affected limb, or akinetic rigidity of that limb.
8
Q
lewy body dementia
A
Parkinisonism and fluctuations in cognitive impairment and visual hallucinations, often before Parkinsonian features occur.
9
Q
types of motor neuron disease
A
Amyotrophic lateral sclerosis
Primary lateral sclerosis:
Progressive muscular atrophy
Progressive bulbar palsy
10
Q
amyotrophic lateral sclerosis
A
50% of patients): typically LMN signs in arms and UMN signs in legs
11
Q
primary lateral sclerosis
A
UMN only
12
Q
progressive muscular atrophy
A
LMN signs only, affects distal muscles before proximal
13
Q
progressive bulbar palsy
A
palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei, carries worst prognosis
14
Q
pathophysiology motor neuron disease
A
progressive degeneration of both upper and lower motor neurones. The sensory neurones are spared
15
Q
sx motor neuron disease
A
lower motor neurone disease (Muscle wasting, Reduced tone, Fasciculations, Reduced reflexes) and upper motor neurone disease (Increased tone or spasticity, Brisk reflexes, Upgoing plantar responses)
Often present as: late middle aged (e.g. 60) man, possibly with an affected relative. There is an insidious, progressive weakness of the muscles throughout the body affecting the limbs, trunk, face and speech. There may be increased fatigue when exercising. They may complain of clumsiness, dropping things more often or tripping over. They can develop slurred speech (dysarthria).
16
Q
UMN signs
A
Increased tone or spasticity, Brisk reflexes, Upgoing plantar responses
17
Q
LMN signs
A
Muscle wasting, Reduced tone, Fasciculations, Reduced reflexes
18
Q
mx motor neuron disease
A
no effective treatments for halting or reversing the progression of the disease.
Riluzole can slow the progression of the disease and extend survival by a few months in ALS.
Patients usually die due to resp failure or pneumonia
19
Q
pathophysiology MS
A
demyelination of the myelinated neurones in the central nervous system. This is caused by an inflammatory process involving the activation of immune cells against the myelin.
20
Q
causes MS
A
Combination of: Multiple genes, Epstein–Barr virus (EBV), Low vitamin D, Smoking, Obesity
21
Q
presentation MS
A
Optic neuritis, Eye movement abnormalities, Focal weakness (Bells palsy, Horners syndrome, Limb paralysis, Incontinence), Focal sensory symptoms (Trigeminal neuralgia, Numbness, Paraesthesia, Lhermitte’s sign), ataxia
22
Q
disease courses MS
A
Clinically Isolated Syndrome: the first episode
Relapsing-remitting: episodes of disease and neurological symptoms followed by recovery
Secondary progressive: was relapsing-remitting disease at first, but now there is a progressive worsening of symptoms with incomplete remissions
Primary progressive: worsening of disease and neurological symptoms from the point of diagnosis without initial relapses and remissions
23
Q
diagnosis MS
A
MRI scanscan demonstrate typical lesions – disseminated in time and space
Lumbar puncturecan detect “oligoclonal bands” in the cerebrospinal fluid (CSF)
24
Q
mxMS
A
Relapses: methylprednisolone
Disease modification: disease-modifying drugs and biologic therapy
Symptoms: Exercise to maintain activity and strength, Neuropathic pain can be managed with medication such as amitriptyline or gabapentin, Depression can be managed with antidepressants such as SSRIs, Urge incontinence can be managed with anticholinergic medications such as tolterodine or oxybutynin (although be aware these can cause or worsen cognitive impairment), Spasticity can be managed with baclofen, gabapentin and physiotherapy
25
presentation migraine
Headaches last between 4 and 72 hours. Moderate to severe intensity
Pounding or throbbing in nature
Usually unilateral, photophobia, phonophobia), With or without aura (visual changes - visual changes Sparks in vision, Blurring vision, Lines across vision, Loss of different visual fields),Nausea and vomiting
26
mx migraine
Acute: Paracetamol, Triptans, NSAIDs, Antiemetics
Prophylaxis: Propranolol, Topiramate, Amitriptyline
27
causes/RF migraine
Stress, Bright lights, Strong smells,Certain foods (e.g. chocolate, cheese and caffeine), Dehydration, Menstruation,Abnormal sleep patterns, Trauma
28
presentation tension headaches
mild ache across the forehead and in a band-like pattern around the head.
29
mx tension headaches
Reassurance, Basic analgesia, Relaxation techniques, Hot towels to local area
30
causes/RF tension headaches
Stress, Depression, Alcohol, Skipping meals, Dehydration
31
presentation cluster headaches
Severe pain, occur in clusters, unilateral usually around the eye, Red, swollen and watering eye, miosis, ptosis, Nasal discharge, Facial sweating
32
mx cluster headaches
Acute Triptans, High flow 100% oxygen for 15-20 minutes
Prophylaxis: Verapamil, Lithium, Prednisolone (a short course for 2-3 weeks to break the cycle during clusters)
33
causes/RF cluster headaches
smoker. Attacks can be triggered by things like alcohol, strong smells and exercise.
34
presnetation medication overuse headaches
similar non-specific features to a tension headache.
35
mx medication over use headaches
Withdraw analgesia - slowly
36
causes/RF medication overuse headaches
long term analgesia use – opioids, triptans
37
presentation trigeminal neualgia
Intense electricity like facial pain that comes on spontaneously and last anywhere between a few seconds to hours. Triggers include cold weather, spicy food, caffeine and citrus fruits, touching the area
38
mx trigeminal neuralgia
Carbamazepine, surgery to decompress or intentionally damage the trigeminal nerve is an option
39
causes/RF trigeminal neuralgia
compression of the nerve (Ophthalmic (V1), Maxillary (V2), Mandibular (V3))
40
presentation GCA
Severe unilateral headache typically around temple and forehead, Scalp tenderness, Jaw claudication, Blurred or double vision, Irreversible painless complete sight loss can occur rapidly
41
mx GCA
Prednisolone,
42
causes/RF GCA
strong link with polymyalgia rheumatica. The patients at higher risk are white females over 50.
43
headache red flags
Fever, photophobia or neck stiffness (meningitis or encephalitis)
New neurological symptoms (haemorrhage, malignancy or stroke)
Dizziness (stroke)
Visual disturbance (temporal arteritis or glaucoma)
Sudden onset occipital headache (subarachnoid haemorrhage)
Worse on coughing or straining (raised intracranial pressure)
Postural, worse on standing, lying or bending over (raised intracranial pressure)
Severe enough to wake the patient from sleep
Vomiting (raised intracranial pressure or carbon monoxide poisoning)
History of trauma (intracranial haemorrhage)
Pregnancy (pre-eclampsia)
44
SAH
bleeding in to the subarachnoid space
45
causes/RF SAH
ruptured cerebral aneurysm.
Hypertension, Smoking, Excessive alcohol consumption, Cocaine use, Family history
46
features SAH
sudden onset occipital headache AKA “thunderclap headache”. Neck stiffness, Photophobia, Vision changes, speech changes, weakness, seizures and loss of consciousness
47
mx SAH
Surgery-clipping or coiling, nimodipine to prevent vasospasm
48
subdural haemorrhage
rupture of the bridging veins
49
causes/RF SDH
elderly or alcoholic patients
50
presnetation SDH
several week to month progressive history of either confusion, reduced consciousness or neurological deficit
51
mx SDH
Surgical decompression with burr holes
52
extraudral haemorrhage cause
rupture of the middle meningeal artery
53
cause EDH
trauma
54
presentation EDH
Often associated with temporal bone fracture due to trauma. traumatic head injury and ongoing headache. Period of improved neurological symptoms and consciousness followed by a rapid decline over hours
55
mx EDH
craniotomy and evacuation of the haematoma
56
seizures
Seizures are transient episodes of abnormal electrical activit
57
ix seizures
electroencephalogram (EEG) can show typical patterns in different forms of epilepsy and support the diagnosis.
An MRI brain
58
status epilepticus
Seizures lasting more than 5 minutes or more than 3 seizures in one hour.
59
mx status epilepticus in community
Buccal midazolam, Rectal diazepam
60
mx status eplilepticus in hospital
Secure the airway, Give high-concentration oxygen, Assess cardiac and respiratory function, Check blood glucose levels, Gain intravenous access (insert a cannula)
IV lorazepam 4mg, repeated after 10 minutes if the seizure continues
If seizures persist: IV phenobarbital or phenytoin
61
non epileptic attacks
Psychogenic, patients who present with epileptic-like seizures but do not have characteristic electrical discharges
patients may have a history of mental health problems or a personality disorder
62
features non epileptic attacks
pelvic thrusting, family member with epilepsy, much more common in females, crying after seizure, don't occur when alone, gradual onset
63
features generalised tonic clonic seizure
There is loss of consciousness and tonic (muscle tensing) and clonic (muscle jerking) episodes. Typically the tonic phase comes before the clonic phase. There may be associated tongue biting, incontinence, groaning and irregular breathing. After the seizure there is a prolonged post-ictal period where the person is confused, drowsy and feels irritable or depressed
64
mx generalised tonic clonic seizure
First line: sodium valproate
Second line: lamotrigine or carbamazepine
65
features focal seizure
start in temporal lobes. They affect hearing, speech, memory and emotions. There are various ways that focal seizures can present: Hallucinations, Memory flashbacks, Déjà vu, Doing strange things on autopilot
66
mx focal seizure
lamotrigine or levetiracetam
67
features absence seizure
children. The patient becomes blank, stares into space and then abruptly returns to normal. During the episode they are unaware of their surroundings and won’t respond. These typically only lasts 10-20 seconds
68
mx absence seizure
ethosuximide
69
features atonic seizures
drop attacks”. They are characterised by brief lapses in muscle tone. These don’t usually last more than 3 minutes. They typically begin in childhood. They may be indicative of Lennox-Gastaut syndrom
70
mx atonic seizures
Sodium valproate, lamotrigine
71
features myoclonic seizures
sudden brief muscle contractions, like a sudden “jump”. The patient usually remains awake during the episode. They occur in various forms of epilepsy but typically happen in children as part of juvenile myoclonic epilepsy
72
mx myoclonic seizures
Sodium valproate, levetiracetam
73
features infantile spasms
West syndrome. It is a rare (1 in 4000) disorder starting in infancy at around 6 months of age. It is characterised by clusters of full body spasms. There is a poor prognosis: 1/3 die by age 25, however 1/3 are seizure free
74
mx infantile spasms
Prednisolone
Vigabatrin
75
features febrile convulsions
usually children, due to viral infection, brief and generalised tonic/tonic-clonic in nature
76
mx febrile convulsions
reduced fever-paracetamol
77
inheritance of muscular dystrophies
X-linked recessive
due to mutation in the gene encoding dystrophin,
in Duchenne muscular dystrophy there is a frameshift mutation resulting in one or both of the binding sites are lost leading to a severe form
in Becker muscular dystrophy there is a non-frameshift insertion in the dystrophin gene resulting in both binding sites being preserved leading to a milder form
78
features duchene muscular dystrophy
progressive proximal muscle weakness from 5 years
calf pseudohypertrophy
Gower's sign: child uses arms to stand up from a squatted position
30% of patients have intellectual impairment
79
features becker muscular dystrophy
develops after the age of 10 years
intellectual impairment much less common
80
mx muscular dystrophy
MDT, splints, physio
81
myasthenia gravis
autoimmune condition that causes muscle weakness that gets progressively worse with activity and improves with rest
82
pathophysiology myasthenia gravis
acetylcholine receptor antibodies are produced by the immune system. These bind to the postsynaptic neuromuscular junction receptors. This blocks the receptor and prevents the acetylcholine from being able to stimulate the receptor and trigger muscle contraction. Also antibodies against muscle-specific kinase (MuSK) and antibodies against low-density lipoprotein receptor-related protein 4 (LRP4). MuSK and LRP4
83
what disease is myasthenia gravis linked to
thymoma (tumours of the thymus gland)
84
presentation myasthenia gravis
weakness that gets worse with muscle use and improves with rest. Symptoms are typically minimal in the morning and worst at the end of the day. The symptoms most affect the proximal muscles and small muscles of the head and neck. It leads to: diplopia, ptosis, Weakness in facial movements, Difficulty with swallowing, Fatigue in the jaw when chewing, Slurred speech, Progressive weakness with repetitive movements
85
ix myasthenia gravis
Diagnosis can be made testing directly for the relevant antibodies:: Acetylcholine receptor (ACh-R) antibodies (85% of patients), Muscle-specific kinase (MuSK) antibodies (10% of patients), LRP4 (low-density lipoprotein receptor-related protein 4) antibodies (less than 5%)
A CT or MRI of the thymus gland is used to look for a thymoma. The edrophonium test :
86
mx myashtenia gravis
Reversible acetylcholinesterase inhibitors (usually pyridostigmine or neostigmine), Immunosuppression (e.g. prednisolone or azathioprine) suppresses the production of antibodies, Thymectomy can improve symptoms even in patients without a thymoma, Monoclonal antibodies (Rituximab Eculizumab)
87
what is myashtneic crisis
is a severe complication of myasthenia gravis.
It causes an acute worsening of symptoms,
88
trigger myasthenic crisis
often triggered by another illness such as a respiratory tract infection
89
mx myasthenic crisis
Patients may require non-invasive ventilation with BiPAP or full intubation and ventilation.
Medical treatment of myasthenic crisis is with immunomodulatory therapies such as IV immunoglobulins and plasma exchange.
90
cause of lamberton eaten myasthnic syndrome
small-cell lung cancer. It is a result of antibodies produced by the immune system against voltage-gated calcium channels in small cell lung cancer (SCLC) cells
91
presntation lambert eaton myasthenic syndrome
The symptoms tend to develop slowly. The proximal muscles are most notably affected, causing proximal muscle weakness. It most notably presents with proximal leg muscle weakness. It can also affect the intraocular muscles causing double vision (diplopia), the levator muscles in the eyelid causing eyelid drooping (ptosis) and the oropharyngeal muscles causing slurred speech and swallowing problems (dysphagia). Patients may also experience dry mouth, blurred vision, impotence and dizziness due to autonomic dysfunction.
Patients with Lambert-Eaton have reduced tendon reflexes. A notable finding is that these reflexes become temporarily normal for a short period following a period of strong muscle contraction. For example, the patient can maximally contract the quadriceps muscle for a period, then have their reflexes tested immediately afterwards, and display an improvement in the response. This is called post-tetanic potentiation.
92
mx labert eaton myasthenic syndrome
Amifampridine
Immunosuppressants (e.g. prednisolone or azathioprine)
IV immunoglobulins
Plasmapheresis
93
inheritance huntingtons
autosomal dominant genetic condition that causes a progressive deterioration in the nervous system.
Huntington’s chorea is a “trinucleotide repeat disorder” of CAG that involves a genetic mutation in the HTT gene on chromosome 4.
94
presentation huntingtons
Asx until 30-50
cognitive, psychiatric or mood problems. These are followed by the development of movement disorders: Chorea (involuntary, abnormal movements), Eye movement disorders, dysarthria, dysphagia
Anticipation
95
mx huntingtons
Nothing slows the disease down
Medications that can suppress the disordered movement: Antipsychotics (e.g. olanzapine), Benzodiazepines (e.g. diazepam), Dopamine-depleting agents (e.g. tetrabenazine)
Death usually due to resp failure or suicide
96
cause meningitis
Viral: herpes simplex virus (HSV), enterovirus and varicella zoster virus (VZV)
Bacterial: children and adults = Neisseria meningitidis (meningococcus) and Streptococcus pneumoniae (pneumococcus), neonates = group B strep (GBS)
97
presentation meningitis
Viral meningitis tends to be milder than bacterial
fever, neck stiffness, vomiting, headache, photophobia, altered consciousness and seizures. Where there is meningococcal septicaemia children can present with a non-blanching rash.
98
ix meningitis
Kernig’s test: on their back, flexing one hip and knee to 90 degrees and then slowly straightening the knee whilst keeping the hip flexed at 90 degrees. Where there is meningitis it will produce spinal pain or resistance to movement.
Brudzinski’s test: patient flat on their back and gently using your hands to lift their head and neck off the bed and flex their chin to their chest. In a positive test this causes the patient to involuntarily flex their hips and knees.
Lumbar puncture
Blood cultures
99
mx meningitis
Bacterial: in community= IM benpen, hospital= <3 months – cefotaxime plus amoxicillin, > 3 months – ceftriaxone, Dexamethasone is given 4 times daily for 4 days to children over 3 months. Ciprofloxacin for contacts
Viral: acyclovir for HSV or VZV
100
complications for meningitis
Hearing loss, Seizures and epilepsy, Cognitive impairment and learning disability, Memory loss, Cerebral palsy, with focal neurological deficits such as limb weakness or spasticity
101
CSF bacterial meningitis
cloudy
high protein
low glucose
high WCC - neutrophils
102
CSF viral meningitis
clear
protein normal
normal glucose
high WCC - lymphocytes
103
features encephalitis
fever, headache, psychiatric symptoms, seizures, vomiting
focal features e.g. aphasia
peripheral lesions (e.g. cold sores) have no relation to the presence of HSV encephalitis
104
cause encephalitis
HSV-1 is responsible for 95% of cases in adults
typically affects temporal and inferior frontal lobes
105
ix encephalitis
CSF: lymphocytosis, elevated protein, PCR for HSV, VZV and enteroviruses
Neuroimaging: medial temporal and inferior frontal changes (e.g. petechial haemorrhages)
EEG: lateralised periodic discharges at 2 Hz
106
mx encephalitis
IV aciclovir
107
cause brain abscess
extension of sepsis from middle ear or sinuses, trauma or surgery to the scalp, penetrating head injuries and embolic events from endocarditis
108
presentation brain abscess
Headache: often dull, persistent
Fever: may be absent and usually not the swinging pyrexia seen with abscesses at other sites
focal neurology e.g. oculomotor nerve palsy or abducens nerve palsy secondary to raised intracranial pressure
other features consistent with raised intracranial pressure: nausea, papilloedema, seizures
109
ix brain abscess
CT
110
mx brain abscess
Surgery: a craniotomy is performed and the abscess cavity debrided
IV antibiotics: IV 3rd-generation cephalosporin + metronidazole
intracranial pressure management: e.g. dexamethasone
111
guillian barre syndrome
acute paralytic polyneuropathy that affects the peripheral nervous system. It causes acute, symmetrical, ascending weakness and can also cause sensory symptoms.
112
trigger GBS
Infection and is particularly associated with to campylobacter jejuni, cytomegalovirus and Epstein-Barr virus (GI stuff).
113
presentation GBS
Symmetrical ascending weakness (starting at the feet and moving up the body), Reduced reflexes, There may be peripheral loss of sensation or neuropathic pain, It may progress to the cranial nerves and cause facial nerve weakness
114
ix GBS
The Brighton criteria can be used for diagnos, Nerve conduction studies (reduced signal through the nerves), Lumbar puncture for CSF (raised protein with a normal cell count and glucose)
115
mx GBS
IV immunoglobulins, Plasma exchange (alternative to IV IG), Supportive care, VTE prophylaxis, In severe cases with respiratory failure patients may need intubation, ventilation and admission to the intensive care unit.
Prognosis: 80% will fully recover, 15% will be left with some neurological disability, 5% will die
116
presentation brain cancer
focal neurological symptoms Brain tumours often present with symptoms and signs of raised intracranial pressure. As a tumour grows within the skull it takes up space. This leaves less space for the other contents of the skull (such as the CSF) to squeeze in to and leads to a rise in the pressure within the intracranial space.
Raised Intracranial Pressure : Concerning features of a headache that should prompt further examination and investigation include: Constant, Nocturnal, Worse on waking, Worse on coughing, straining or bending forward, Vomiting. Other presenting features of raised intracranial pressure may be: Altered mental state, Visual field defect, Seizures (particularly focal), Unilateral ptosis, Third and sixth nerve palsies,
Papilloedema (on fundoscopy)
Fundoscopic Changes: Blurring of the optic disc margin, Elevated optic disc (look for the way the retinal vessels flow across the disc to see the elevation), Loss of venous pulsation, Engorged retinal veins, Haemorrhages around optic disc, Paton’s lines which are creases in the retina around the optic disc
117
what metastasises to brain
: Lung, Breast, Renal cell carcinoma, Melanoma
118
gliomas
Astrocytoma (glioblastoma multiforme is the most common), Oligodendroglioma, Ependymoma. Gliomas are graded from 1-4. Grade 1 are most benign (possibly curable with surgery). Grade 4 are the most malignant (glioblastomas).
119
meningioma
Meningiomas are tumours growing from the cells of the meninges in the brain and spinal cord. They are usually benign, however they take up space and this mass effect can lead to raised intracranial pressure and neurological symptoms.
120
pituitary tumour
Pituitary tumours tend to be benign. If they grow large enough they can press on the optic chiasm causing a specific visual field defect called a bitemporal hemianopia. This causes loss of the outer half of the visual fields in both eyes. They have the potential to cause hormone deficiencies (hypopituitarism) or to release excessive hormones leading to: Acromegaly, Hyperprolactinaemia, Cushing’s disease, Thyrotoxicosis
121
mx brain tumour
Palliative care, Chemotherapy, Radiotherapy, Surgery
122
mx pituitary tumour
Trans-sphenoidal surgery, Radiotherapy, Bromocriptine to block prolactin-secreting tumours, Somatostatin analogues (e.g. ocreotide) to block growth hormone-secreting tumours
123
bulbar palsy
'lower motor neurone' lesion affecting cranial nerves 9, 10 and 12. This causes impairments in speech and swallowing
124
features bulbar palsy
absent or normal jaw jerk reflex, an absent gag reflex, a flaccid fasciculating tongue, nasal quiet speech, and signs suggestive of the cause e.g. limb fasciculations of motor neurone disease.
125
causes bulbar palsy
motor neurone disease (in particular the progressive bulbar palsy variant), myasthenia gravis, Guillain-Barré syndrome, brainstem stroke (the lateral medullary syndrome), and syringobulbia.
126
pseudobulbar palsy
bilateral lesion affecting the corticobulbar tracts (running from the motor cortex to the motor nuclei of cranial nerves 9, 10, and 12 in the medulla). Can be thought of as an 'upper motor neurone' lesion of speech and swallow.
127
features pseudobulbar palsy
spastic tongue, a slow thick ("hot-potato") speech, a brisk jaw jerk reflex, and emotional lability. Other upper motor neurone features in the limbs may be present e.g. spastic hypertonia, pyramidal weakness, and hyper-reflexia.
128
causes pseudobulbar palsy
vascular causes (such as a bilateral internal capsule stroke), degenerative causes (such as motor neurone disease and progressive supranuclear palsy), neoplastic causes (such as upper brainstem tumours), autoimmune causes (such as multiple sclerosis), and traumatic causes.
129
sx cerebella disease
D - Dysdiadochokinesia, Dysmetria (past-pointing), patients may appear 'Drunk'
A - Ataxia (limb, truncal)
N - Nystamus (horizontal = ipsilateral hemisphere)
I - Intention tremour
S - Slurred staccato speech, Scanning dysarthria
H – Hypotonia
130
causes cerebella disease
Friedreich's ataxia, ataxic telangiectasia
neoplastic: cerebellar haemangioma
stroke
alcohol
multiple sclerosis
hypothyroidism
drugs: phenytoin, lead poisoning
paraneoplastic e.g. secondary to lung cancer
131
causes cerebral palsy
Antenatal=: Maternal infections, Trauma during pregnancy.
Perinatal =Birth asphyxia, Pre-term birth.
Postnatal= Meningitis, Severe neonatal jaundice, Head injury
132
types cerebral palsy
Spastic:
Dyskinetic:
Ataxic:
Mixed:
Spastic CP is also known as pyramidal CP. Dyskinetic CP is also known as athetoid CP and extrapyramidal CP.
133
spastic cerebral palsy
hypertonia (increased tone) and reduced function resulting from damage to upper motor neurons. Monoplegia: one limb affected. Hemiplegia: one side of the body affected. Diplegia: four limbs are affects, but mostly the legs. Quadriplegia: four limbs are affected more severely, often with seizures, speech disturbance and other impairments
134
dyskinetic cerebral palsy
problems controlling muscle tone, with hypertonia and hypotonia, causing athetoid movements and oro-motor problems. This is the result of damage to the basal ganglia.
135
ataxic cerebral palsy
problems with coordinated movement resulting from damage to the cerebellum
136
presentation cerebral palsy
Failure to meet milestones, Increased or decreased tone, generally or in specific limbs, Hand preference below 18 months is a key sign to remember for exams,, Problems with coordination, speech or walking, Feeding or swallowing problems, Learning difficulties, hemiplegic or diplegic gait, signs of an upper motor neurone lesion, with good muscle bulk, increased tone, brisk reflexes and slightly reduced power
137
complications/associated conditions with cerebral palsy
Learning disability, Epilepsy, Kyphoscoliosis, Muscle contractures, Hearing and visual impairment, Gastro-oesophageal reflux
138
mx cerebral palsy
Children at risk of developing cerebral palsy, such as those with hypoxic-ischaemic encephalopathy, need to be followed up to identify any signs and symptoms that develop.
OT, physio, SALT, dietician
Medication: Muscle relaxants (e.g. baclofen) for muscle spasticity and contractures, Anti-epileptic drugs for seizures, Glycopyrronium bromide for excessive drooling
Orthopaedic surgeons can perform procedures to release contractures or lengthen tendons (tenotomy).
139
causes peripheral neuropathy
A – Alcohol
B – B12 deficiency
C – Cancer and Chronic Kidney Disease
D – Diabetes and Drugs (e.g. isoniazid, amiodarone and cisplatin)
E – Every vasculitis
140
charcot marie tooth inheritance
autosomal dominant pattern
141
features charcot marie tooth
affects the peripheral motor and sensory nerves.
: High foot arches (pes cavus), Distal muscle wasting causing “inverted champagne bottle legs”, Weakness in the lower legs, particularly loss of ankle dorsiflexion, Weakness in the hands, Reduced tendon reflexes, Reduced muscle tone, Peripheral sensory loss
142
cause carpal tunnel syndrome
compression median nerve
143
sx carpal tunnel
Sensory sx (Numbness, Paraesthesia, Burning sensation, Pain) in palmar aspect of Thumb, Index and middle finger, The lateral half of ring finger.
Motor sx: Weakness of thumb movements, Weakness of grip strength, Difficulty with fine movements involving the thumb, Wasting of the thenar muscles. Tinnels and phalens
144
features of damage to ulnar nerve
Damage at wrist='claw hand' - hyperextension of the metacarpophalangeal joints and flexion at the distal and proximal interphalangeal joints of the 4th and 5th digits, wasting and paralysis of intrinsic hand muscles (except lateral two lumbricals), wasting and paralysis of hypothenar muscles, sensory loss to the medial 1 1/2 fingers (palmar and dorsal aspects). Damage at elbow = as above plus radial deviation of wrist
145
cause meralgia paraesthetica
compression of the lateral cutaneous nerve of the thigh underneath the inguinal ligament
146
sx meralgia paraesthetica
shooting pains along the outer aspect of the upper leg
147
ttpes of diabetic neuropathy
Distal Symmetrical Sensory Neuropathy
Small-fibre Predominant Neuropathy
Diabetic Amyotrophy
Mononeuritis Multiplex
Autonomic Neuropathy
148
distal symmetric sensory diabetic neuropathy
Most common, Caused by loss of large sensory fibres., Sensory loss in a glove and stocking distribution, Often affecting touch, vibration and proprioception.
149
Small-fibre Predominant deiabetic Neuropathy:
Caused by loss of small sensory fibres, Presents with deficits in pain and temperature sensation in a glove and stocking distribution along with episodes of burning pain.
150
diabetic amyotrophy
Caused by inflammation of lumbosacral plexus or cervical plexus, Severe pain around the thighs and hips, Proximal weakness.
151
mononeuritis multiplex
Invariably painful, Neuropathies involving =2 peripheral nerves.
152
autonomic neuropathy
Presents with postural hypotension, gastroparesis, constipation, urinary retention, arrhythmias and erectile dysfunction.
153
radiculopathy
Radiculopathy describes a range of symptoms produced by the pinching of a nerve root in the spinal column. The pinched nerve can occur at different areas along the spine (cervical, thoracic or lumbar).
154
common sx radiculopathy
Sharp pain in the back, arms, legs or shoulders that may worsen with certain activities, even something as simple as coughing or sneezing, Weakness or loss of reflexes in the arms or legs, Numbness of the skin, “pins and needles,” or other abnormal sensations (paresthesia) in the arms or legs
Cervical: sx usually in hands and arms
Thoracic: least common place, symptoms often follow a dermatomal distribution
Lumbar: sciatica sx. L5 radiculopathy= foot drop and weakness of hip abduction
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causes radiculopathu
Foraminal stenosis, herniated discs, bony spurs
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causes foot drop
common peroneal nerve lesion - the most common cause
L5 radiculopathy
sciatic nerve lesion
superficial or deep peroneal nerve lesion
other possible includes central nerve lesions (e.g. stroke) but other features are usually present
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features common peroneal nerve lesion
foot drop and weakness of foot dorsiflexion and eversion. Reflexes will be normal
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L5 radiculopathy features
foot drop and weak hip abduction
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spinal cord compresion
results from processes that compress or displace arterial, venous, and cerebrospinal fluid spaces, as well as the cord itself.
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features spinal cord compression
Cord compression typically presents with acute (or less commonly subacute) upper motor neuron signs and sensory disturbance below the level of the lesion.
Deep and localised back pain is often also present, along with a stabbing radicular sensory disturbance at the level of the lesion. Bladder and bowel involvement is also commonly seen.
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causes spinal cord compression
Trauma
Neoplasia (seen in 5-10% of cancer patients, presenting complaint in 20% of these)
Infection (especially TB in at-risk patients)
Disc prolapse
Epidural haematoma
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mx spinal cord compression
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.
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.
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cauda equina syndrome
surgical emergency where the nerve roots of the cauda equina at the bottom of the spine are compressed.
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causes cauda equina syndrome
Herniated disc (the most common cause)
Tumours, particularly metastasis
Spondylolisthesis (anterior displacement of a vertebra out of line with the one below)
Abscess (infection)
Trauma
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red flags for cauda equina
Saddle anaesthesia (loss of sensation in the perineum – around the genitals and anus)
Loss of sensation in the bladder and rectum (not knowing when they are full)
Urinary retention or incontinence
Faecal incontinence
Bilateral sciatica
Bilateral or severe motor weakness in the legs
Reduced anal tone on PR examination
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mx cauda equina
Cauda equina is a neurosurgical emergency. It requires:
Immediate hospital admission
Emergency MRI scan to confirm or exclude cauda equina syndrome
Neurosurgical input to consider lumbar decompression surgery
Surgery should be performed as soon as possible to increase the chances of regaining function. Even with early surgery, patients can be left with bladder, bowel or sexual dysfunction. Leg weakness and sensory impairment can also persist.
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complete spinal cord injury
paraplegia, tetraplegia
complete loss of motor and sensory function below injury
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incomplete spinal cord injury
paraplegia, tetraplegia
partial or random preservtation of motor or sensory function below injury
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brown sequard syndrome
ipsilateral motor weakness or paralysis
contralateral loss sensation
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anterrio cord syndrome
motor paralysis and loss of pain and temp sensation
preserved: proprioception, touch and vibration sense
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posterior cord syndrome
loss of sensory funciton
motor function preserved
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central cord syndrome
due to cervical spine injuries
greater motor impairment in upper boday compaired to lower, variable sensory loss
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features narcolepsy
typical onset in teenage years
hypersomnolence
cataplexy (sudden loss of muscle tone often triggered by emotion)
sleep paralysis
vivid hallucinations on going to sleep or waking up
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ix narcolepsy
Epworth sleepiness scale, multiple sleep latency EEG
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mx narcolepsy
daytime stimulants (e.g. modafinil) and nighttime sodium oxybate
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cataplexy
the sudden and transient loss of muscular tone caused by strong emotion (e.g. laughter, being frightened). Around two-thirds of patients with narcolepsy have cataplexy
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features cataplexy
range from buckling knees to collapse.
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features chronic fatigue syndrome
Debilitating fatigue that is worsened by activity, is not caused by excessive cognitive, physical, emotional or social exertion, and is not significantly relieved by rest.
Post-exertional malaise after activity in which the worsening of symptoms: is often delayed in onset by hours or days, is disproportionate to the activity, has a prolonged recovery time that may last hours, days, weeks or longer.
Unrefreshing sleep or sleep disturbance (or both), which may include: feeling exhausted, feeling flu-like and stiff on waking, broken or shallow sleep, altered sleep pattern or hypersomnia.
Cognitive difficulties (sometimes described as 'brain fog'), which may include problems finding words or numbers, difficulty in speaking, slowed responsiveness, short-term memory problems, and difficulty concentrating or multitasking.
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ix chronic fatigue syndrome
must exclude other causes e.g. anaemia
May reveal: orthostatic intolerance and autonomic dysfunction, temperature hypersensitivity, neuromuscular symptoms, including twitching and myoclonic jerks , flu-like symptoms, heightened sensory sensitivities, pain, including pain on touch, myalgia, headaches, eye pain, abdominal pain or joint pain without acute redness, swelling or effusion
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mx chronic fatigue syndrome
MDT care
Energy management
Manage symptoms: sleep, pain
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neurofibromatosis
genetic condition that causes nerve tumours (neuromas) to develop throughout the nervous system.
Neurofibromatosis type 1 is more common than type 2.
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neurofibromatosis type 1 criteria
Café-au-lait spots, Relative with NF1, Axillary or inguinal freckles, Bony dysplasia such as Bowing of a long bone or sphenoid wing dysplasia, Iris hamartomas (Lisch nodules), Neurofibromas (2 or more) or 1 plexiform neurofibroma, Glioma of the optic nerve.
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complications neurofibromatosis type 1
Migraines, Epilepsy, Renal artery stenosis causing hypertension, Learning and behavioural problems (e.g. ADHD), Scoliosis of the spine, Vision loss (secondary to optic nerve gliomas), Malignant peripheral nerve sheath tumours, Gastrointestinal stromal tumour (a type of sarcoma), Brain tumours, Spinal cord tumours with associated neurology (e.g. paraplegia), Increased risk of cancer (e.g. breast cancer), Leukaemia
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features neurofibromatosis type 2`
bilateral acoustic neuroma
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tuberous sclerosis
genetic condition that causes features in multiple systems.
The characteristic feature is the development of hamartomas
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tuberous sclerosis features
Skin Signs: Ash leaf spots are depigmented areas of skin shaped like an ash leaf, Shagreen patches are thickened, dimpled, pigmented patches of skin, Angiofibromas are small skin coloured or pigmented papules that occur over the nose and cheeks, Subungual fibromata are fibromas growing from the nail bed. They are usually circular painless lumps that grow slowly and displace the nail, Cafe-au-lait spots are light brown “coffee and milk” coloured flat pigmented lesions on the skin, Poliosis is an isolated patch of white hair on the head, eyebrows, eyelashes or beard
Neurological Features: Epilepsy, Learning disability and developmental delay
Other Features: Rhabdomyomas in the heart, Gliomas (tumours of the brain and spinal cord), Polycystic kidneys, Lymphangioleiomyomatosis (abnormal growth in smooth muscle cells, often affecting the lungs), Retinal hamartomas
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motor GCS
6=obeys verbal commands to move
5=localises to pain
4=withdraws from pain
3=flexure posturing with stimulus
3=extensor posturing with stimulus
1=no response
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verbal GCS
5=fully orientated
4=not fully orientated
3=intelligable but not organised
2=unintelligible sounds
1=no vocalisation
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eye opening GCS
4=spontaneous
3=open to speech
2=open to pain
1= none
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features normal pressure hydrocephalus
classical triad of features is seen: urinary incontinence, dementia and bradyphrenia, gait abnormality (may be similar to Parkinson's disease. Symptoms typically develop over a few months.
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ix normal pressure hydrocephalus
hydrocephalus with ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement
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mx normal pressure hydrocephalus
ventriculoperitoneal shunting
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presentation menieres disease
recurrent attacks of vertigo, and symptoms of hearing loss, tinnitus and a feeling of fullness in the ear, Unexplained falls (“drop attacks”) without loss of consciousness, Imbalance, which can persist after episodes of vertigo resolve
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mx menierres disease
acute attacks= Prochlorperazine, Antihistamines (e.g., cyclizine, cinnarizine and promethazine). Prophylaxis = Betahistine
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acoustic neuroma
AKA Vestibular Schwannoma)
tumours of the Schwann cells. They occur around the “cerebellopontine angle”
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sx acoustic neuroma
Hearing loss, tinnitus, Balance problems. They can also be associated with a facial nerve palsy. usually unilateral. Bilateral acoustic neuromas are associated with neurofibromatosis type 2.
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inheritance benign essential tremot
autosomal dominant condition
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features benign essential tremor
postural tremor: worse if arms outstretched, improved by alcohol and rest
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mx benign essential tremot
propanolol
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features horners syndrome
by the triad of ptosis (drooping eyelid), anhidrosis (lack of sweating) and miosis (constricted pupil) on the ipsilateral side
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features bells palsy
acute, unilateral, idiopathic, facial nerve paralysis.
: lower motor neuron facial nerve palsy - forehead affected, patients may also notice post-auricular pain (may precede paralysis), altered taste, dry eyes, hyperacusis
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mx bells palsy
oral prednisolone within 72 hours of onset +/- antivirals, eye care is important to prevent exposure keratopathy-prescription of artificial tears and eye lubricants should be considered
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neuro causes collapse
haemorrhage
vasovagal
TIA
stroke
space occupying lesion
