neurology Flashcards
define cerebral palsy?
a permanent disorder of movement and posture due to a non-progressive lesion in the developing brain
what are the 2 biggest risk factors for cerebral palsy ?
prematurity
low birth weight
causes of cerebral palsy can fall of 3 large categories what are they? which one accounts for most cases of cerebral palsy
antenatal - 80% of cases
perinatal
neonatal
antenatal causes of cerebral palsy?
Cerebral vascular accident
structural malformations
perinatal cause of cerebral palsy?
hypoxic ischemic encephalopathy
neonatal causes of cerebral palsy?
hyperbilirubinemia
hypoglycaemia
infections: Torch, meningitis, encephalitis
head trauma
hydrocephalus
what are the 3 classifications of cerebral palsy ?
spastic
dyskinetic
ataxic
mixed
how can spastic cerebral palsy be further classified ?
hemiplegic
diplegic
quadriplegic
characteristic of hemiplegic cerebral palsy?
associated with MCA stroke
arms more affected then legs
diplegic
associated with prematurity and periventricular leukomalacia
lower limbs more affect than upper limbs
characteristics of quadriplegic cerebral palsy?
associated with small for gestational age and neonatal infections
dyskinetic cerebral is caused by damage to what area?
basal ganglia and hippocampus (MRI shows basal ganglia damage)
characteristics of dyskinetic cerebral palsy?
dystonic movements
ataxic cerebral palsy often has an identified …. cause
genetic
what is the GMFCS?
gross motor function classification system
characteristics of GMFCS 1
children perform gross motor skills such as running and jumping but speed coordination and balance are limited
characteristics of GMFCS 2?
Children walk in most settings and climb stairs holding onto a railing. They may experience difficulty walking long distances and balancing on uneven terrain, inclines, in crowded areas or confined spaces.
Children may walk with physical assistance, a handheld mobility device or used wheeled mobility over long distances. Children have only minimal ability to perform gross motor skills such as running and jumping.
characteristics of GMFCs 3?
Children walk using a hand-held mobility device in most indoor settings. They may climb stairs holding onto a railing with supervision or assistance. Children use wheeled mobility when travelling long distances and may self-propel for shorter distances.
characteristics of GMFCS 4?
Children use methods of mobility that require physical assistance or powered mobility in most settings. They may walk for short distances at home with physical assistance or use powered mobility or a body support walker when positioned. At school, outdoors and in the community children are transported in a manual wheelchair or use powered mobility.
characteristics of GMFCS 5?
GMFCS Level V
Children are transported in a manual wheelchair in all settings. Children are limited in their ability to maintain antigravity head and trunk postures and control leg and arm movements.
management of cerebral palsy?
- MDT: physio, psycho, dietician, speech and language input
- for spastic movements; baclofen oral/botulinism injections. if all fail consider baclofen pump or selective dorsal rhizotomy surgery
define hydrocephalus?
a disease characterized by the accumulation of CSF in the brain?
what are the 2 broad ways of categorizing hydrocephalus?
- obstructive
- communication
causes of obstructive hydrocephalus ?
congenital: aqueduct stenosis, type 2 chiari malformation, intraventricular hemorrhage in preterm infants, neoplasms in the posterior fossa
causes of non-obstructive (communicating) hydrocephalus
subarachnoid hemorrhage
meningitis
clinical features of hydrocephalus in neonate?
macrocephaly
bulging fontanelle
separation of the cranial sutures
sunsetting eyes
clinical features of hydrocephalus in older children?
early morning nausea and vomitting
headache
confusion
seizures
diagnosis of hydrocephalus ?
- antenatal scan revealing congenital anomalies
- occipital frontal circumference (OFC)
- ultrasound
- CT/MRI
management of hydrocephalus?
depends on the cause
1. VP shunts
2. endoscopic ventriculostomy
3. short term diuretics (furosemide and acetalzolamide) for patients unfit for surgical interventions
complications of VP shunts?
blockage
infection
over-drain (low ICP)
peritoneal perforation
define neural tube defects ?
a group of defects characterized by the failure of closure of the neural plate at day 26-28 of development
what are the manifestations of neural tube defects ?
- anencephaly: absence of the forebrain
- encephalocele: sac like protrusion of the brain through the skull
- spina bidifa
what are the 3 types of spina bifida?
oculta: appears only as a tuft of hair or a birth mark
meningocele: protrusion of the meninges
myelomeningocele: protrusion of the meninges and the spinal cord, most severe if above the level of L3
prevention of neural tube defects ?
folic acid supplements. 0.4 mg/d, starting 3 months prior to conception and throughout the first trimester. 5 mg/d If at high risk (e.g previous baby with neural tube defect)
what are maternal factors that high risk for fetus developing neural tube defect?
diabetes
smoking
teratogens (valproate, carbamazepine)
diagnosis of neural tube defects ?
- antenatal screening: raised maternal serum alpha-fetoprotein (ms-AFP), ultrasound showing spinal/cerebral defect
- those identified should be offered genetic karyotyping to rule out genetic disorders
complications of neural tube defects
hydrocephalus
scoliosis
neuropathic bladder/bowel
Lower limb sensory loss (tethered cord
management of neural tube defects
- surgical closure of the spinal opening
- VP shunt for hydrocephalus
- neuropathic bladder: intermittent urinary catheterization, prophylactic antibiotics, anticholinergics
- neuropathic bowel; prevent constipation, ACE procedure for incontience
- physiotherapy for lower limbs
what type of disorder is neurofibromatosis type 1 ?
neurocutaneous syndrome
genetics of neurofibromatosis type 1 ?
autosomal dominant
diagnostic criteria for neurofibromatosis type 1?
need 2 or more of the following (7);
1) 6 or more cafe au lait macules (>5 mm if prepubertal or >15 mm if post pubertal)
2) 2 or more neurofibroma
3) 2 or more lesch nodules in iris (no visual effects
4) optic pathway glioma (leads to blindness)
5) axillary freckling (crows sign)
6) sphenoid dysplasia
7) a first degree relative with NF1
how does NF2 mostly present?
hearing loss due to bilateral acoustic neuromas compressing the vestibulocochlear nerve (CN8)
genetics of tuberous sclerosis complex?
autosomal dominant, TSC1 or 2
manifestations of tuberous sclerosis complex ?
- ash leaf spots: hypo pigmented
- shagreen patches: rough skin
- angiofibromas: butterfly distribution on face
- brain lesion: subependymal nodules, subependymal giant cell astrocytoma
what are common presentations of tuberous sclerosis complex ?
- seizures
- 50% have autism (autism spectrum disorder)
management of tuberous sclerosis ?
- seizure control
- surgery for tumours
- MDT for cognitive difficulties
what is sturge weber syndrome ?
a sporadic disorder characterized by port wine stain (facial hemangioma) in the trigmeinal distribution. ipsilateral leptomeningeal angioma and calcifications (tram-track appearance on MRI)
complications of sturge weber syndrome?
- hemiplegia on the contralateral side to the port wine stain
- seizures
management of sturge weber syndrome?
MDT
seizure control.
laster therapy for the port wine stain
