Neurological Disorders Flashcards

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
Q

Features Juvenile Absence epilepsy

A
10-20y
Absences and generalised tonic-clonic seizures
Photosensitivity
EEG- 4-6Hz atypical spike
Remission unlikely
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26
Q

Feature Juvenile Myoclonic Epilepsy

A
10-20y
Myoclonic seizures, generalised tonic-clonic, abscences
Typical Hx throwing drinks or cereal
Occurs in morning
Remission unlikely
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27
Q

Anti-epileptic drugs

A

Tonic-clonic: valproate
Abscence: valproate
Myoclonic: valproate
Focal: carbamezapine

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28
Q

S/E Anti-epileptic drugs

A

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

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29
Q

Non-medication Mx Epilepsy

A

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)

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30
Q

Safety netting Epilepsy

A
Avoiding deep baths
No swimming unsupervised
Driving (1y seizure free)
Avoidance of heights
No pregnancy with some medications
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31
Q

Definition status epilepticus

A

Epileptic seizure lasting 30 minutes or repeated epileptic seizures for 30m without recovery

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32
Q

Corticospinal tract disorders

A
Cerebral dysgenesis 
Global hypoxia-ichaemia
Arterial ischaemic stroke
Cerebral tumour
Acute disseminated encephalomyeltitis
Postictal paralysis
Hemiplegic Migraine
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33
Q

Basal Ganglia Disorders

A
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
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34
Q

Cerebellar Disorders

A

Acute- medication and drugs
Post viral esp. varicella
Posterior fossa lesion/tumour
Genetic and degenerative .e.g. Friedreich

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35
Q

Features Cerebellar disorders

A

Past pointing
Poor alternating movements
Wide based ataxic gait
Nystagmus

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36
Q

Features Basal Ganglia disorder

A

Difficulty initiating movement
Jerky movements
Fluctuating increased tone

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37
Q

Features Corticospinal tract disorders

A
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
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38
Q

Features Neuromuscular disorders

A

Hypotonia
Delayed motor milestones
Muscle weakness- may be progressive or static
Unsteady/abnormal gait
Fatiguability
Muscle cramps (suggest metabolic myopathy)

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39
Q

Define Gower’s Sign

A

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

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40
Q

Ix Myopathy

A

Plasma creatinine kinase
Muscle biopsy
DNA testing
US and MRI of muscles

41
Q

Ix Neuropathy

A

Nerve conduction studies
DNA testing
Nerve biopsy (sural nerve in leg)
EMG

42
Q

Features spinal muscular atrophy (type 1)

A
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
Q

Features Charcot-Marie-Tooth

A
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
Q

Features Guillian Barre Syndrome

A

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
Q

Ix Guillian Barre

A

CSF: WCC normal, protein raised
MRI spine: to exclude spinal lesion
Nerve conduction studies: reduced velocities

46
Q

Features Bell Palsy

A

Isolated lower motor neurone paralysis
Facial weakness
Associated with herpes or Lyme disease

47
Q

Mx Bell Palsy

A

Aciclovir if Herpes suspected
Corticosteroids
Eye protection and ointment

48
Q

Features Juvenile Myasthenia gravis

A
>10y
Ophthalmoplegia and ptosis
Loss of facial expression
Difficulty chewing
Generalised proximal weakness
Muscle fatiguability
Improved with IV edrophonium/oral pyridostigmine
49
Q

Mx Juvenile Myasthenia

A

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
Q

Epidemiology Duchenne Muscular Dystrophy

A

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
Q

Features Duchenne Muscular Dystrophy

A
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
Q

Mx Duchenne

A

Physiotherapy o prevent contractures
Tendoachilles lengthening and scoliosis surgery
Overnight CPAP to prevent nocturnal hypoxia
Corticosteroids to preserve mobility and prevent scoliosis

53
Q

Features Beckers Muscular Dystrophy

A
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
Q

Features Limb Girdle Muscular Dystrophy

A

Proximal upper and lower limb weakness
Cardiomyopathy
Difficulty breathing
Raised plasma CK

55
Q

Features congenital muscular dystrophies

A
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
Q

Metabolic myopathies presentation

A

Floppy infant

muscle weakness or cramps on exercises

57
Q

Causes of metabolic myopathy

A

Glycogen storage disorder
Disorder of lipid metabolism
Mitochondrial cytopathies

58
Q

Causes of floppy infant

A
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
Q

Features Benign acute myositis

A

Postviral phenomenon, follows URTI
Self-limiting course
Pain and weakness
Raised plasma CK

60
Q

Features dermatomyositis

A

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
Q

Ix dermatomyositis

A

Inflammatory markers raised

Inflammatory cell infiltrate and atrophy on muscle biopsy

62
Q

Features Dystrophia Myotonica

A

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
Q

Causes of Ataxia

A
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
Q

Features Friedreich ataxia

A
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
Q

Cx Fredreichs ataxia

A
Kyphoscoliosis
Diabetes
Cardiomyopathy
Cardiorespiratory compromise
Death aged 40-50
66
Q

Features Ataxia Telangiectasia

A
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
Q

Cx Ataxia Telangiectasia

A

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
Q

Extradural Haemorrhage Mechanism

A

Direct head trauma
Skull fracture
Tearing of middle meningeal passing through foramen spinosum of sphenoid bone
Bleeding into extradural space

69
Q

Extradural Haemorrhage Hx

A

Direct head trauma
Lucid interval
Deterioration of conscious level

70
Q

Mx Extradural haematoma

A

CT scan
Correct hypovolaemia
Urgent evacuation of the haematoma
Arrest of bleeding

71
Q

Features Extradural Haematoma

A
Focal neurology: 
-dilation of ipsilateral pupil
-paresis of contralateral limbs
-false localising Vith nerve paresis
Shock
Anaemia
72
Q

Subdural Haematoma Mechanism

A

Tearing of bridging veins crossing subdural space

Characteristic of NAI by shaking, or brain shrinkage

73
Q

Subarachnoid haemorrhage Hx

A

Rapid onset severe headache with vomiting, confusion or lowered GCS

74
Q

Mx Subarachnoid Haemorrhage

A

CT identifying blood in CSF (or LP)

Neurosurgical or interventional radiology

75
Q

Childhood Stroke Pathology

A

Commonly carotid, anterior cerebral arteries and middle cerebral arteries- hemiparesis and speech disturbance
Rarely posterior circulation- visual and cerebellar signs

76
Q

Causes childhood stroke

A

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
Q

Ix Childhood stroke

A

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
Q

Neural tube defect prevention

A

Forms at day 28
Folic acid supplementation pre-conceptually
Improved maternal nutrition
10x increase in subsequent births

79
Q

Anencephaly

A

Determined by US
Failure of cranium and brain development
Fatal (termination offered)

80
Q

Encephalocele

A

Extrusion of brain and meninges through midline skull defect
Surgical correction possible
Often associated cerebral malformations

81
Q

Spina bifida occulta

A

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
Q

Myelomeningocele Features

A
Variable paresis of lower limbs]
Hypotonia
Muscle imbalance
Sensory loss
Bladder denervation
Bowel denervation
Scoliosis
Hydrocephalus (Chiari type 2 malformation)
83
Q

Mx (myelo)meningocele

A
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
Q

Difference between meningocele and myelomeningocele

A

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
Q

Causes of hydrocephalus

A
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
Q

Features of hydrocephalus

A
Skull sutures not fused
Disproportionate head circumference or excessive growth rate
Bulging anterior fontanelle
Scalpveins prominent
Fixed downward gaze or sunsetting eyes
87
Q

Mx Hydrocephalus

A

Symptomatic relief of raised ICP
Monitoring of head circumference
Insertion of ventriculoperitoneal shunt

88
Q

Cx Ventriculoperitoneal shunt

A

Blockage
Infection, usually coagulase-negative staph
Overdrainage causing low-pressure headache

89
Q

Features Neurofibromatosis Type 1

A

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
Q

Epidemiology Neurofibromatosis

A
1/3000 live births
Autosomal dominant
High penetrance
Variable expression
50% de novo
91
Q

Neurofibromatosis type 2 features

A

Presenting in adolescents
Bilateral acoustic neuromata (deafness and cerebella-pontione angle syndrome)
5th nerve paresis
Cerebellar ataxia

92
Q

Cutaneous features tuberous sclerosis

A

Depigmented ash leave patches
Amelanotic naevi fluorescing under Wood’s UV light
Roughened patches(shagreen) over lumber spine
Angiofibroma (butterfly distribution)- unusual <3y

93
Q

Neurological features tuberous sclerosis

A
Infantile spasms
Developmental delay
Focal epilepsy
Intellectual disability
Autism
94
Q

Extra-neurocutaneous features tuberous sclerosis

A
Fibromata beneath nails
Dense white areas on retina (phakomata)
Rhabdomyomata of heart
Angiomyolipomas
Polycystic Kidneys
Cysts in lungs
95
Q

Sturge-Weber syndrome features

A

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
Q

Ix Sturge-Weber

A

Calcification of gyri showing rail-road track on Xray

97
Q

Neurodegenerative disorders (commonest)

A

Lysosomal storage disorders
Peroxisomal enzyme defects
Wilson disease

98
Q

Features Adrenoleukodystrophy

A
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