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Neurological Disorders Flashcards

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1
Q

Features tension type headache

A

Symmetrical
Gradual Onset
Band or pressure tightness
No associated symptoms

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2
Q

Features migraine

A 
Episodes lasting 1-72 hours
Commonly bilateral
Pulsatile
Over temporal or frontal area
GI disturbance
Photophobia
Phonophobia
Aggravated by activity
Relived by sleep
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3
Q

Features migraine aura

A

Visual, sensory, or motor
Most commonly visual
Negative phenomena .e.g. hemianopia or scotoma
Positive phenomena .e.g. fortification spectra

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4
Q

Migraine triggers

A

Disturbance of biorhythm .e.g. late nights
Stress
Certain foods .e.g. cheese, chocolate, caffeine
Menstruation and OCP

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5
Q

Uncommon forms of migraine

A

Familial hemiplegic migraine (calcium channel defect)
Sporadic hemiplegic migraine
Basilar type migraine- vomiting with nystagmus
Periodic syndromes .e.g. cyclical vomiting, benign paroxysmal vertigo

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6
Q

Features space occupying lesion

A 
Visual field defects .e.g. craniopharyngioma
Cranial nerve abnormality
Abnormal gait
Torticollis (tilting of the head)
Growth failure
Papillodema
Cranial bruit
Early/late puberty
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7
Q

Features Raised intracranial pressure

A

Worse lying down
Morning vomiting
Night-time waking
Change in mood, personality, and educational performance

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8
Q

Mx Migraine- rescue treatments

A 
Analgesia:  paracetamol and NSAIDs
Antiemetics .e.g. prochlorperazine, cyclazine
Triptans .e.g. sumatriptan (nasal prep)
Warm pads
Cold compresses
Topical forhead balms
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9
Q

Mx Migraine- prophylactic

A

Indicated with frequent and intrusive headaches
Sodium channel blockers .e.g. topiramate, valproate
Beta-blockers .e.g. propranolol (not in asthma)
Tricyclics .e.g. pizotifen (causes weight gain and sleepiness), amitriptyline
Acupuncture

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10
Q

Features febrile seizure

A

Epileptic seizure in the presence of fever and absence of intracranial infection
Typically children aged 6m to 6y
Occurs with viral infection and rapid rise of temp
Brief generalised tonic-clonic

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11
Q

Features complex febrile seizures

A

Focal
Prolonged (>15-30m)
Repeated within same illness

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12
Q

Mx febrile convulsion

A

Admission for first seizure of complex seizure
Reassurance of parent/career
Antipyretics have no effect on seizure
In prolonged seizures: rescue therapy by buccal midazolam

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13
Q

Causes of paroxysmal disorders

A 
Ble breath holding spells
Reflex asystolic syncope (trauma, cold, fright, fever)
Syncope
Migraine
Benign paroxysmal vertigo
Cardiac arrhythmia
Tics, daydreaming, night terrors
Self-gratification
Non-epileptic seizures
Pseudoseizures
Fabricated
Induced illness
Paroxsymal movement disorders
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14
Q

Generalised epileptic seizures types

A 
Absence seizures (may be precipitated by hyperventilation)
Myoclonic seizures
Tonic seizures
Tonic-clonic seizures
Atonic seizures
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15
Q

Generalised epileptic seizures features

A 
Onset in both hemispheres
Loss of consciousness
No warning
Symmetrical seizure
Bilateral synchronous discharge on EEG
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16
Q

Features focal epileptic seizures

A

Originate in small group of dysfunctional neurones
Heralded by an aura
Can evolve to tonic-clonic

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17
Q

Focal seizures brain areas

A

Frontal- motor phenomena .e.g. Jacksonian march
Temporal lobe- auditory or sensory, lip-smacking, plucking at clothing, non-purposeful walking (spread to pre-motor)
Occipital- visual
Parietal- contralateral altered sensation

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18
Q

Ix Epileptic seizures

A

ECG, esp convulsive seizures
EEG: ictal, inter-ictal, sleep-deprived, 24hr or 5day video-telemetry
Brain imaging

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19
Q

Features Infantile spasms (West syndrome)

A 
3-12m
Violent spasms of head, trunk and limbs
Extension of arms
Lasts 1-2s
Mulitple bursts of 20-30
On waking or multiple times a day
EEG- hypsarrythmia (chaotic background of slow wave activity)
Similar to colic
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20
Q

Mx Infantile spasms

A 
Vigabatrin
Corticosteroids
60-70% response 
Relapse common
Most lose skills and develop learning disability
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21
Q

Features Lennox-Gastaut syndrome

A 
1-3 y
Mostly atypical, atonic absences
Tonic seizures in sleep
Neurodevelopmental arrest or regression
EEG- slow generalised spike and wave (1-3Hz)
Poor prognosis
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22
Q

Features Childhood Absence Epilepsy

A

4-12y
Momentary unresponsive stare with motor arrest
Sudden onset
Lasts a few seconds
Poor recall
Normal development, may interfere with schooling
Common in females
Induced by hyperventilation
EEG- generalised spike and wave discharges (3-4Hz)

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23
Q

Features Benign Rolandic Epilepsy

A

Tonic-clonic seizures in sleep or simple focal seizures
Abnormal feeling in tongue and distortion of face
EEG- sharp focal waves from rolandic area
Benign
Remits in adolescence

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24
Q

Features Panayiotopoulus Syndrome

A

1-5y
Autonomic features .e.g. vomiting, unresponsive staring, eye deviation
EEG- posterior focal sharp waves and occipital charges
Remits in childhood

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25
Features Juvenile Absence epilepsy
``` 10-20y Absences and generalised tonic-clonic seizures Photosensitivity EEG- 4-6Hz atypical spike Remission unlikely ```
26
Feature Juvenile Myoclonic Epilepsy
``` 10-20y Myoclonic seizures, generalised tonic-clonic, abscences Typical Hx throwing drinks or cereal Occurs in morning Remission unlikely ```
27
Anti-epileptic drugs
Tonic-clonic: valproate Abscence: valproate Myoclonic: valproate Focal: carbamezapine
28
S/E Anti-epileptic drugs
Valproate- weight gain, hair loss, teratogenic, idiosyncratic liver failure Carbamazepine- rash, hyponatraemia, ataxia, live enzyme induction Lamotrigine- rash, insomnia, ataxia Ethosuximide- nausea and vomiting Levetiracetam- irritability Gabapentin- insomnia Topiramate- weight loss, depression, paraesthesia Vigabatrin- irritability, restriction of visual fields
29
Non-medication Mx Epilepsy
Ketogenic diet (low carb, fat based) Vagal nerve stimulation Epilepsy surgery (concordance between ictal EEG, MRI and functional imaging) -temporal lobectomy in medial temporal sclerosis -hemispherectomy (disconnection of the hemisphere)
30
Safety netting Epilepsy
``` Avoiding deep baths No swimming unsupervised Driving (1y seizure free) Avoidance of heights No pregnancy with some medications ```
31
Definition status epilepticus
Epileptic seizure lasting 30 minutes or repeated epileptic seizures for 30m without recovery
32
Corticospinal tract disorders
``` Cerebral dysgenesis Global hypoxia-ichaemia Arterial ischaemic stroke Cerebral tumour Acute disseminated encephalomyeltitis Postictal paralysis Hemiplegic Migraine ```
33
Basal Ganglia Disorders
``` Acquired brain injury -Acute and profound hypoxia-ischaemia -Carboin monoxide posioning -Post-cardiopulmonary bypass chorea Post-strep chorea (Rheumatic fever) Mitochondrial cytopathies Wilson Disease Vit E deficiency Pontocerebellar hypoplasia ```
34
Cerebellar Disorders
Acute- medication and drugs Post viral esp. varicella Posterior fossa lesion/tumour Genetic and degenerative .e.g. Friedreich
35
Features Cerebellar disorders
Past pointing Poor alternating movements Wide based ataxic gait Nystagmus
36
Features Basal Ganglia disorder
Difficulty initiating movement Jerky movements Fluctuating increased tone
37
Features Corticospinal tract disorders
``` Weakness in pattern: -adduction at shoulder -flexion at elbow -adduction and internal rotation at hip -flexion at hip and knee -plantar flexion at ankle Brink hyperreflexia Extensor plantar reflexes Loss of fine finger movement ```
38
Features Neuromuscular disorders
Hypotonia Delayed motor milestones Muscle weakness- may be progressive or static Unsteady/abnormal gait Fatiguability Muscle cramps (suggest metabolic myopathy)
39
Define Gower's Sign
Need to turn prone to rise to standing from a supine position Normal until 3y Very weak- climb legs with hands to gain standing position Sign of proximal muscle weakness
40
Ix Myopathy
Plasma creatinine kinase Muscle biopsy DNA testing US and MRI of muscles
41
Ix Neuropathy
Nerve conduction studies DNA testing Nerve biopsy (sural nerve in leg) EMG
42
Features spinal muscular atrophy (type 1)
``` Progressive weakness and wasting Alert expression Fasciculations of the tongue Symmetrical flaccid paralysis Absent deep tendon reflex Intercostal recession Weakness of bulbar muscles- weak cry, saliva pooling Neer sit unaided Death by respiratory failure by 1y ```
43
Features Charcot-Marie-Tooth
``` Progressive distal muscle wasting Present at pre-school Bilateral foot drop Loss of ankle reflexes Pes Cavus Lower limbs>> Upper Limbs Hypertrophic nerves (attempted remyelination) ```
44
Features Guillian Barre Syndrome
Typically 2-3w after URTI or gastroenteritis Ascending progressive symmetrical weakness Loss of tendon reflexes Autonomic involvement Sensory symptoms in trunk and distal limbs mild Bilateral facial weakness (often missed in young children) Difficulty chewing/swallowing Maximum muscle weakness 2-4w after onset of illness
45
Ix Guillian Barre
CSF: WCC normal, protein raised MRI spine: to exclude spinal lesion Nerve conduction studies: reduced velocities
46
Features Bell Palsy
Isolated lower motor neurone paralysis Facial weakness Associated with herpes or Lyme disease
47
Mx Bell Palsy
Aciclovir if Herpes suspected Corticosteroids Eye protection and ointment
48
Features Juvenile Myasthenia gravis
``` >10y Ophthalmoplegia and ptosis Loss of facial expression Difficulty chewing Generalised proximal weakness Muscle fatiguability Improved with IV edrophonium/oral pyridostigmine ```
49
Mx Juvenile Myasthenia
Identifying acetylcholine or anti-MuSK antibodies Choline esterase inhibitor .e.g. pyridostigmine, neostigmine Immunosuppressive therapy Immunomodulators .e.g. prednisolone, azothiaprine, mycophenolate mofetilis Monoclonal antibodies .e.g. rituximab Thymectomy is thymoma or with antibody +ve Plasma exchange for crisis
50
Epidemiology Duchenne Muscular Dystrophy
1/3000-6000 male infants X linked recessive 1/3 de novo mutations Dystrophine gene delection- Ca+ influx causing calcium calmodulin complex breakdown andmyofibre necrosis
51
Features Duchenne Muscular Dystrophy
``` Usually diagnosed aged 5 Waddling gait Language delay Stairs one by one Slow runners compared to peers Gowers +ve Slower and clumsier than peers Progressive muscle atrophy and weakness Non-ambulant by 10-14y Reduced life expectancy 1/3 learning difficulties Scoliosis common complication ```
52
Mx Duchenne
Physiotherapy o prevent contractures Tendoachilles lengthening and scoliosis surgery Overnight CPAP to prevent nocturnal hypoxia Corticosteroids to preserve mobility and prevent scoliosis
53
Features Beckers Muscular Dystrophy
``` Some functional dystrophin production Clinically milder disease Slower progression Average age of onset is 11y Ambulatory until 20s Life expectancy to middle/old age ```
54
Features Limb Girdle Muscular Dystrophy
Proximal upper and lower limb weakness Cardiomyopathy Difficulty breathing Raised plasma CK
55
Features congenital muscular dystrophies
``` Present at birth or early infancy Weakness Hypotonia Contractures Slowly progressive proximal weakness Tendency to contracture once ability to walk is lost Feeding and breathing difficulty ```
56
Metabolic myopathies presentation
Floppy infant | muscle weakness or cramps on exercises
57
Causes of metabolic myopathy
Glycogen storage disorder Disorder of lipid metabolism Mitochondrial cytopathies
58
Causes of floppy infant
``` Hypoxic ischemic encephalopathy Intracraial haemorrhage Cerebral malformation Chromosomal disorder Congenital infection Peroxismal disorder Drug effects .e.g. benzos Brith trauma esp. breech Syringomyelia Spinal muscular atrophy Myasthenia gravis Infantile botulism Muscular dystrophy Congenital myopathies Heridotry neuropathy Inborn error of metabolism Carnitine deficiency ```
59
Features Benign acute myositis
Postviral phenomenon, follows URTI Self-limiting course Pain and weakness Raised plasma CK
60
Features dermatomyositis
systemic illness Onset 5-10y Insidious: fever, misery, symmetrical muscle weakness Pharyngeal muscle involvement affecting swallowing Violaceous pink purple rash on eyelids Rash affecting extensor surfaces of joints Subcutaneous calcifications (calcinosis)
61
Ix dermatomyositis
Inflammatory markers raised | Inflammatory cell infiltrate and atrophy on muscle biopsy
62
Features Dystrophia Myotonica
Relatively common Newborns- hypotonia, feeding and resp difficulty Thin ribs Talipes at birth Oligohydramnios and reduced fetal movements Myopathic facial appearance Myotonia Adults: baldness, cataracts, testicular atrophy, T2DM Slow release of handshake or tight fist Cx: cardia dysrhythmia
63
Causes of Ataxia
``` Freidrich ataxia Ataxia Telangiectasia Cerebrellar agenesis/dysgenesis Post-infctious cerebellilitis- varicella Posterior fossa tumours Hereditory Miller Fisher syndrome (Guillian Barre variant) Mitochondrial disease Drugs e.g. carbamezipine, lamotrigine, ethanol ```
64
Features Friedreich ataxia
``` Worsening ataxia Dysarthria Distal wasting in lower limbs Absent reflexes Pes Cavus Impairment of joint position and vibration sense Extensor plants Optic atrophy Cerebellar component more apparent with age ```
65
Cx Fredreichs ataxia
``` Kyphoscoliosis Diabetes Cardiomyopathy Cardiorespiratory compromise Death aged 40-50 ```
66
Features Ataxia Telangiectasia
``` Mild delay in motor development Oculomotor problems Inco-ordincatio Delay in ocular pursuits and saccades Difficulty with balance Complex eye movement disorders Wheelchair required by adolescents Telangiectasia develop in conjunctiva and shoulder (4y) ```
67
Cx Ataxia Telangiectasia
Increased susceptibility to infection Deficiency of IgA and IgE Malignancy .e.g. lymphoma and acte leukaemia Progressive pulmonary disease with bronchiectasis Raised serum alpha-feroprotein Sensitivity to ionising radiation
68
Extradural Haemorrhage Mechanism
Direct head trauma Skull fracture Tearing of middle meningeal passing through foramen spinosum of sphenoid bone Bleeding into extradural space
69
Extradural Haemorrhage Hx
Direct head trauma Lucid interval Deterioration of conscious level
70
Mx Extradural haematoma
CT scan Correct hypovolaemia Urgent evacuation of the haematoma Arrest of bleeding
71
Features Extradural Haematoma
``` Focal neurology: -dilation of ipsilateral pupil -paresis of contralateral limbs -false localising Vith nerve paresis Shock Anaemia ```
72
Subdural Haematoma Mechanism
Tearing of bridging veins crossing subdural space | Characteristic of NAI by shaking, or brain shrinkage
73
Subarachnoid haemorrhage Hx
Rapid onset severe headache with vomiting, confusion or lowered GCS
74
Mx Subarachnoid Haemorrhage
CT identifying blood in CSF (or LP) | Neurosurgical or interventional radiology
75
Childhood Stroke Pathology
Commonly carotid, anterior cerebral arteries and middle cerebral arteries- hemiparesis and speech disturbance Rarely posterior circulation- visual and cerebellar signs
76
Causes childhood stroke
Cardiac .e.g. cyanotic heart disease Haematological .e.g. sickle cell Postinfective Inflammatory .e.g. SLE Metabolic/Genetic -homocystinuria -MELAS (myoclonic epilepsy, lactic acidosis, stroke) -CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencepgalopathy) Vascular malformation .e.g. AV malformation, MoyaMoya Trauma
77
Ix Childhood stroke
cerebral and external carotid vascular MRI, with angiogram and venography Echo to detect embolism Thrombophilia and vasculitis screen Metabolic tests for homocysteine and mitochondrial
78
Neural tube defect prevention
Forms at day 28 Folic acid supplementation pre-conceptually Improved maternal nutrition 10x increase in subsequent births
79
Anencephaly
Determined by US Failure of cranium and brain development Fatal (termination offered)
80
Encephalocele
Extrusion of brain and meninges through midline skull defect Surgical correction possible Often associated cerebral malformations
81
Spina bifida occulta
Failure of fusion of vertebral arch Overlying skin lesion .e.g. tuft of hair, lipoma, birth mark, dermal sinus Underlying tethering presents with neurological deficits
82
Myelomeningocele Features
``` Variable paresis of lower limbs] Hypotonia Muscle imbalance Sensory loss Bladder denervation Bowel denervation Scoliosis Hydrocephalus (Chiari type 2 malformation) ```
83
Mx (myelo)meningocele
``` Some functional dystrophin production Clinically milder disease Slower progression Average age of onset is 11y Ambulatory until 20s Life expectancy to middle/old age ```
84
Difference between meningocele and myelomeningocele
Meningocele has an outpouching of pia and arachnoid covered by skin and dura Myelomeningocele has an outpouching with a neural plaque at the surface
85
Causes of hydrocephalus
``` Aqueduct stenosis Atresia of outflow foramina of 4th ventricle Chiari maformation Posterior fossa neoplasm Vascular malformation Intraventricular haemorrhage in preterm infant Subarcahnoid haemorrhage Meningitis ```
86
Features of hydrocephalus
``` Skull sutures not fused Disproportionate head circumference or excessive growth rate Bulging anterior fontanelle Scalpveins prominent Fixed downward gaze or sunsetting eyes ```
87
Mx Hydrocephalus
Symptomatic relief of raised ICP Monitoring of head circumference Insertion of ventriculoperitoneal shunt
88
Cx Ventriculoperitoneal shunt
Blockage Infection, usually coagulase-negative staph Overdrainage causing low-pressure headache
89
Features Neurofibromatosis Type 1
Cafe au lait spots (>6 >5mm/15mm, pre/post puberty) Neurofibroma: firm nodular overgrowth of nerve Axillary freckling Optic glioma, possible visual impairment Lisch nodule: hamartoma of the iris (seen on slit lamp) Bony lesions from sphenoid dysplasia (eye protrusion)
90
Epidemiology Neurofibromatosis
``` 1/3000 live births Autosomal dominant High penetrance Variable expression 50% de novo ```
91
Neurofibromatosis type 2 features
Presenting in adolescents Bilateral acoustic neuromata (deafness and cerebella-pontione angle syndrome) 5th nerve paresis Cerebellar ataxia
92
Cutaneous features tuberous sclerosis
Depigmented ash leave patches Amelanotic naevi fluorescing under Wood's UV light Roughened patches(shagreen) over lumber spine Angiofibroma (butterfly distribution)- unusual <3y
93
Neurological features tuberous sclerosis
``` Infantile spasms Developmental delay Focal epilepsy Intellectual disability Autism ```
94
Extra-neurocutaneous features tuberous sclerosis
``` Fibromata beneath nails Dense white areas on retina (phakomata) Rhabdomyomata of heart Angiomyolipomas Polycystic Kidneys Cysts in lungs ```
95
Sturge-Weber syndrome features
Haemangiomatous facial lesion (port wine stain) Distribution of trigeminal nerve Similar intracranial lesion (ipsilateral leptomeningeal angioma) Epilepsy Intellectual diability Contralateral hemiplegia High risk of ipsilateral glaucoma
96
Ix Sturge-Weber
Calcification of gyri showing rail-road track on Xray
97
Neurodegenerative disorders (commonest)
Lysosomal storage disorders Peroxisomal enzyme defects Wilson disease
98
Features Adrenoleukodystrophy
``` Peroxisomal defects (fatty acid breakdown) Neonatal-hypotonia, feeding problems, seizures, facial features Xlinked childhood cerebral- boys 4-12y, school failure, behaviour changes, regression, ataxia Xlink Adrenomyeloneuropathy- males with slow progressive spastic paraparesis and dorsal column sensory disturbance ```

Paediatrics (22 decks)

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