Neurology + Neurodevelopment - TAS Flashcards
What are the danger signs of global developmental delay?
Parental concern
No social smile at 2 months
Not achieved good eye contact at 3 months
Not reaching for objects at 6 months
Failed distraction testing at 8 months
Not sitting with support at 9 months
Not walking unaided at 18 months
Not saying single words with meaning 18 months
Regression of acquired skills
Discordance in developmental areas
Name a common complication of right to left shunting cardiac conditions e.g. tetralogy of fallout?
Brain Abscess
Regression of language milestones with associated abnormal EEG in the form of continuous slow waves during wake and sleep =
Landau-Kleffner Syndrome
3Hz spike and wave pattern on EEG
Absence Seizures
Common complications of VP shunts?
Ventriculitis
Shunt failure/ blockage causing hydrocephalus
Raised ICP
Fracture or migration of shunt tubing
What causes hypsarrhythmia on an EEG? (chaotic high voltage slow waves and multifocal spikes) e.g. infantile spasms?
Tuberous sclerosis
NF1
Birth asphyxia
Congenital Infections
Encephalitis/ Meningitis
Brain trauma
Metabolic Conditions
Idiopathic (approx. 30%)
Treatment for infantile spasms?
ACTH, Prednisolone and Vigibatran
Question suggests deterioration following a partially treated meningitis, think ??
Cerebral Abscess
What are the EEG findings in Herpes Encephalitis?
Frontal-temporal slowing with periodic sharp wave complexes
Erbs palsy spinal roots?
C5-C6
Hip flexion spinal roots?
L1-L3
Treatment of complex seizures with treatment-refactory seizures?
Clobazam
Treatment of absence seizures?
Ethosuxamide
Which factors are associated with increased risk of recurrence of seizures upon discontinuation of treatment?
Older age
Use of multiple anticonvulsants
History of myoclonic or tonic clonic seizures
Previous abnormal EEG or imaging
Extradural bleed on CT scan = what shape?
Bi-convex (moon shaped)
Subdural bleed on CT scan = what shape?
Banana shape/ concave
Periventricular Calcifications are seen in which congenital infection?
CMV
Intracranial calcifications are seen in which congenital infection?
Toxoplasmosis
Chorioretinitis
IUGR
Intracranial diffuse calcifications
Toxoplasmosis
Jaundice
Petechiae
Hepatosplenomegaly
Periventricular Calcifications
CMV infection
Erbs Palsy management
No investigation required and usually self resolve spontaneously
Treatment for generalised tonic clonic seizures?
Lamotrigine (especially used in girls of child bearing age)
Sodium Valproate (used in boys)
Multicoloured circles aura occurring after a headache is suggestive of …?
Occipital lobe epilepsy
Complications of NF1?
Eyes: astrocytic haemartomas, congenital glaucoma
Skeletal: macrocephaly, scoliosis, short stature
Predisposition to tumours
Neurologically: spinal cord and nerve compression, learning difficulties
Renal artery stenosis leading to HTN
Epilepsy affecting adolescents which typically leads to myoclonic jerks in the morning?
Juvenile Myoclonic Epilepsy
Focal seizures, twitching of the mouth in the morning, incomprehensive noises whilst falling asleep, resolves spontaneously, occurs at 9-10 years of age
Benign Rolandic Epilepsy
Generally consanguineous parents, presentation is that of a floppy infant with proximal muscle weakness and hypotonia, areflexia, tongue fasciculation’s are PATHOGNOMONIC
Spinal muscular atrophy
Facial weakness + ptosis + fluctuations =
Congenital Myasthenia Gravis
Often require ventilation at birth, associated with cataracts, milk intellectual impairment, cardiomyopathy and glucose intolerance and low serum IgG
Myotonic Dystrophy
Features of Ataxia Telangectasia?
Telangectasia on face and conjunctiva
Cerebellar Dysfunction - nystagmus, dysarthria, ataxia
Reduced immunoglobulin subsets (IgA, IgG2, IgG4 + IgE) - therefore increased number of infections
Raised alpha-fetoprotein and CEA?
Ataxia Telangectasia
Which conditions show chromosomal fragility?
Blooms Syndrome
Ataxia Telangectasia
Fanconi Anaemia
Progressive, ascending lower limb weakness and paraesthesia?
Guillan-Barrer Syndrome
Management of GBS?
IVIg or Plasma exchange
Rare condition occurring 7-10 years after acquiring the measles virus?
Subacute Sclerosing Panencephalitis
Management of juvenile myoclonic epilepsy in all ages?
Sodium Valproate
Hand wringing and regression of previously acquired language and social skills?
Rett’s Syndrome
Abnormal high signal in the temporal lobe?
Herpes Simplex Encephalitis
What maternal factors increase the risk of neural tube defects?
Maternal alcohol
Rubella
Valproate use in pregnancy
Maternal diabetes
Severe inactivation of the MECP2 gene?
Rett syndrome
Patients can develop this triad of symptoms 1-2 months following rheumatic fever - triad of choreiform activity, hypotonia and emotional lability?
Sydenham’s Chorea
Fine motor developmental milestones - numbers?
1 year - scribble
2 years - parallel lines
3 years - circle
4 years - cross
5 years - triangle
6 years - six sided objects
Centrotemporal spikes on EEG suggests …
Benign Rolandic Epilepsy
Infantile spasms and developmental delay?
West Syndrome
Tramline intracranial calcifications?
Sturge-Weber Syndrome
What condition is associated with epilepsy, learning difficulties and hemiparesis (on the contralateral side to the port wine stain)?
Sturge-Weber Syndrome
Paroxysms of high voltage slow waves with otherwise normal EEG activity?
Sub-acute sclerosing Panencephalitis
EEG findings of high amplitude spikes in the Centro-temporal region?
Benign Rolandic Epilepsy
Mixed upper and lower motor neurone signs with associated cardiomyopathy, scoliosis and pes cavus?
Freidrich’s Ataxia
CSF sample: Protein <1, WCC <1000, normal glucose, mostly lymphocytes?
Viral Meningitis
CSF sample: Protein >1, glucose <2.2, WCC >500 (mostly neutrophils)
Bacterial Meningitis
CSF flow pathway?!
Come - choroid plexus
Lets - lateral ventricle
Migrate - foramen of munro
To - third ventricle
States - Sylvian Aquaduct
For - fourth ventricle
Money - foramen of magendie
Love - foramen of luschka
Sex - subarachnoid space
Ahh - arachnoid villi
Presents with reduced deep tendon reflexes, NORMAL facial expressions and generalised weakness?
Spinal Muscular Atrophy (Werdnig-Hoffman Disease)
Presents with reduced movements in utero, ptosis, fasciculations, generalised weakness including facial weakness?
Congenital myasthenia gratis
Presents with generalised weakness, dysmorphic features (triangular facies), weakness of facial expressions, reduced deep tendon reflexes, cataracts, poor swallow, ?history of polyhydramnios?
Congenital Myotonic Dystrophy
Congenital facial nerve palsy and abducent nerve palsy?
Moebius Syndrome
What is the treatment of choice for absence seizures?
Ethosuxamide
Sodium Valproate (not at child bearing age)
Lamotrigine
Treatment options for benign rolandic epilepsy?
No treatment required if not impacting on lifestyle
What features does occlusion of the anterior cerebral artery cause?
Hemiparesis of the contralateral foot and leg
Sensory loss of the contralateral foot and leg
Motor dysphasia
What are the features of occlusion of the middle cerebral artery?
Hemiparesis of the contralateral face and limbs
Sensory loss of the contralateral face and limbs
Dysphasia
Contralateral neglect
Homonymous hemianopia
Dorsilateral prefrontal dysfunction
What are the features of occlusion of the posterior cerebral artery?
Contralateral loss of pain and temperature sensation
Contralateral hemianopia
Prosopagnosia
Ipsilateral cranial nerve defects
Horner’s syndrome
What are the features of Aicardi syndrome?
Partial or complete absence of the corpus collosum (the part of the brain which connects the left and right cerebral hemispheres and enables interhemispheric communication)
Chorioretinal lacunae
Severe seizures
Severe learning difficulties
Poor prognosis
What is the rate of nerve repair?
Approx. 1mm per day
Children on vigabatrin should have what monitored?
Visual fields (can develop a condition called visual field constriction attributable to vigabatrin)
What is West syndrome?
Triad of infantile spasms + hypsarrhythmia on EEG + reduced IQ
Bells palsy characteristics?
Involves BOTH upper and lower part of face - causes unilateral facial weakness involving the forehead and unilateral failure of eye closure
What are the 5 branches of the facial nerve?
Temporal branch
Zygomatic branch
Buccal branch
Marginal mandibular branch
Cervical branch
Which anti-epileptic medication is contraindicated in juvenile myoclonic epilepsy?
Carbamazepine
What does the vestibulocerebellum control?
Balance and spatial orientation
What does the spinocerebellum control?
fine-tuned body movements
What does the cerebrocerebellum control?
Involved in planned movement and the conscious assessment of movement
Controls sleep wake cycle?
Reticular formation
Plays an important role in reward, addiction and movement?
Substantia nigra
Management options for benign intracranial hypertension?
Weight reduction, serial LPs, diuretics
Acute cases - steroids
Chronic cases - acetazolamide
Surgical - optic nerve sheath fenestration
Rhyme for sensory/ motor function of cranial nerves?
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