Paediatrics Flashcards
1
Q
What factors should be included in the birth history for a child?
A
Mothers age Number of pregnancies / deliveries Prenatal care Delivery method Pregnancy illnesses / rashes Maternal ETOH / drug use Meconium staining Gestational age / corrected age
2
Q
What does a family history of neonatal deaths suggest?
A
Metabolic disorders
3
Q
Which tissues are of neuroectodermal origin?
A
CNS Hair Skin Teeth Nails
4
Q
What is a normal head circ. at birth?
A
35 cm. Grows at 2 cm/month for the first 3 months, then 1 cm/monh from 3-6 months
5
Q
What conditions may lead to an enlarged anterior fontanelle?
A
Hypothyroidism, trisomy syndromes, in utero malnutrition, hypophospatemia, rickets, osteogenesis imperfecta and hydrocephalus.
6
Q
How do you test CN2 in a neonate?
A
After 30 weeks gestation they have a blink response to bright light and have a reliable pupillary reflex. Fixing and following of gaze suggests normal visual acuity. Check the RAPD.
7
Q
How do you test CN3/4/6 in a neonate?
A
Eye movements by fixing and following on mother’s face. Dolls eye reflex.
8
Q
How do you test CN5 in a neonate?
A
Watching the neonate suck / swallow on a dummy / bottle feeding.
9
Q
How do you test CN7 in a neonate?
A
Facial symmetry at rest and during suckling
10
Q
How do you test CN8 in a neonate?
A
Spinning the infant to see if the eyes remain static or fixed on an object. Caloric testing can be performed for formal assessment. Hearing can be assessed by blink reflex to loud sounds
11
Q
How do you test CN10 in a neonate?
A
Infant cry sound
Gag reflex
12
Q
How do you test CN11 in a neonate?
A
Spontaneous shoulder function / movement
13
Q
How do you test CN12 in a neonate?
A
Observation of atrophy and / or tongue deviation
14
Q
How does tone change in neonates?
A
28-40 weeks there is increased flexor tone legs>arms; After 40 weeks the extensor tone becomes balanced.
15
Q
What does extension of the lower limbs in the premature infant indicate?
A
Hypotonia
16
Q
How is cervical tone tested?
A
Pulling the infant up to a sitting position. In a term infant there is some head lag. Tone is tested through vertical and horizontal suspension
17
Q
What is the vertical suspension test?
A
Holding the infant upright under the axilla. If hypotonic will slip through the hands.
18
Q
What is the horizontal suspension test?
A
Hold the infant in the prone position. The infant should be able to hold their head up
19
Q
When can reflexes be tested in neonates?
A
After 33 weeks gestation. Note extensor plantars and sustained clonus up to 8 beats is normal.
20
Q
What is the moro reflex?
A
Dropping the infants head in relation to the body results in extension and abduction of the arms. This is mediated by the vestibulospinal pathway and disappears by 6 months of age.
21
Q
When should the palmar grasp reflex be lost?
A
4-6 months
22
Q
How do you test CN3/4/6 in an infant?
A
Visually follow a toy
23
Q
Where do lesions causing upgaze paralysis localise to?
A
Periaqueductal gray matter (pineal region / tectal tumours).
Vertical gaze center is the rostral interstitial nucleus of the MLF whilst the horizontal gaze center is the PPRF.
24
Q
What is the difference between phorias and tropias?
A
Phorias are squints that can be overcome by fixating on an object whilst tropias are squints at rest that cannot be overcome by fixating on an object
25
How can phorias be identified?
The cover test - when fixating on an object, both eyes align. When the eye is covered the squint returns.
26
How are CN9/10/11 tested in infants?
Gag reflex Shoulder droop / head tilt
27
How are spasticity and rigidity differentiated?
Spasticity is clasp knife (velocity dependent) whilst rigidity has resistance throughout the full range of movement
28
At what age do infants crawl?
9-12 months
29
At what age do infants walk?
12-15 months
30
What does limb atrophy suggest?
LMN lesion
31
How can fine motor coordination be tested?
Have the child reach for objects and look for tremor or dysmetria
32
What does the absence of the parachute response predict?
A predictor of whether a child will develop the ability to walk. It should be present by 8 months
33
What is a conduction aphasia?
Results in lesion of the arcuate fasciculus. They retain the ability to name objects but not to repeat them.
34
Where does calculation function reside?
Dominant angular gyrus - damage to this area results in acalculia which is part of Gerstmann's syndrome
35
What levels do the spinal accessory nerve roots arise from?
C3/4
36
What is the GMFCS?
Gross motor function classification system: 1 = Normal 2 = Difficulty walking long distances 3 = walks with hand held mobility aid 4 = needs a wheelchair and help transferring 5 = powered wheel chair, limited head and trunk control
37
What is the pathophysiology of Cerebral palsy?
Perinatal infarct involving the peri ventricular WM which compromises descending inhibition from reticular nuclei to the spinal cord. This causes abberant spinal reflex arcs.
38
What is the management of cerebral palsy?
1. OT and physio for orthotics and mobility aids
2. Pharmacological intervention with baclofen / Botox injections
3. Orthopaedic surgery to release contractures and bone deformity.
4. Intrathecal baclofen pump or SDR
39
What is the selection criteria for SDR?
Based on Peacocks criteria:
1. Age \>3 years
2. \>1 year after ortho surgery
3. Spastic diplegia - no ataxia and good trunk control
4. GMFCS 2/3
5. PVL on mri that spares basal ganglia and cerebellum
6. No femoral head subluxation
40
What are the EMG responses to SDR?
Based on Fasano’s SDR protocol:
0 = single discharge
1 = sustained discharge in single segment
2 = sustained discharge adjacent segment
3 = discharge in all Ipsilateral segments
4 = contralateral muscle response
41
What proportion of the dorsal roots are sectioned in SDRs?
50-75% of the total
42
What are the indications for botox in focal spasticity?
1. Improve motor function
2. Improve care / hygiene
3. Reduce Pain / sleep disturbance
4. Improve tolerance of orthoses
43
What are the contraindications to Botox treatment for focal spasticity
1. Severe muscle weakness
2. Allergy to botoc
3. On aminoglycoside treatment
44
What is microcephaly?
A head circ \>2SD below the mean Primary microcephaly is present at birth Secondary microcephaly occurs post-natally
45
What are the causes of microcephaly?
Isolated Syndromic - Downs, Trisomy 13, Trisomy 18, fetal alcohol syndrome Development - NTDs, holoprosencephaly, lissencephaly etc Metabolic - aminoacidurias / storage disorders Environmental - Perinatal ischaemia / infection, toxin, malnutrition
46
What are the causes of macrocephaly?
Familial Hydrocephalus BESS Neurophakomatosis Achondroplasia Haemorrhage AVM Cyst Tumour
47
What is the dose of a fluid bolus in a child?
20 ml/Kg (dose of RBCs in 10 ml/kg!)
48
What is the normal HR and BP for a child?
\<12 months = \<160 & \>60 mmHg
12-24 months = \<150 & \>70 mmHg
2-5 years = \<140 & \>75 mmHg
6-12 years = \<120 & \>80 mmHg
\>13 years = \<100 & \>90 mmHg
49
What is the normal RR for a child?
\<12 months = \<60 12-24 months = \<40 2-5 years = \<35 6-12 years = \<30 \>13 years = \<30
50
What volume of blood loss results in bradycardia in a child?
\>45%
51
What are Brushfield spots?
Speckled iris seen in Down syndrome
52
What cardiac defects are seen in Down syndrome?
ASD / VSD / PDA / Tetralogy of fallot
53
What is clindodactyly\>
Curved little finger seen in Downs
54
After what age are the brainstem criteria in children the same as adults?
\>2 months
Between 37 weeks and 2 months diagnosis of brainstem death is very difficult.
Two different consultants should perform the first and second examinations \>12 hours apar if \>30 days old and \>24 hours apart if \<30 days old
Two apnea tests required with final pCO2 \>60
Ancillary tests not needed unless clinical examination and apnea cannot be completed
55
What is a Galant reflex?
Scratching the skin on the back lateral to the spinous process resulting in curvation of the trunk (concave to the stimulated side)
56
What is a crossed extensor reflex?
Passive flexion of one lower limb results in extension of the other lower limb with adduction and internal rotation
57
What is the Rossolimo response?
Tapping of the toes causes flexion
58
What is the suprapubic extensor response?
Pressing the skin over the pubic bone causes extension, adduction and internal rotation of the lower limbs
59
At what age does a child sit with and without support?
6 and 9 months
60
When should a child walk independently?
15 months
61
When can a child write their own name?
6 years (also identifies written letters and numbers)
62
What are the features of cloverleaf deformity aka kleeblattschadel?
Frontal towering
Bitemporal expansion
Bilateral supraorbital recession with proptosis
Hypertelorism
Midface hypoplasia
Caused by premature fusion fo the sagittal, coronal and lambdoid sutures
63
What synostosis syndrome is associated with a broad big toe or thumb?
Pfeiffer syndrome
64
What synostosis syndrome is associated with syndactyly?
Aperts syndrome
65
What head shape does metopic synostosis cause?
Trigonocephaly. Associated with Chiari 1 in 30% due to reduced intracranial volume, callosal dysgenesis and holoprosencephaly. Metopic suture closes normally between 3-9 months.
66
What causes scaphocephaly?
Sagittal suture synostosis
67
What causes a parallelogram-shaped head?
Positional plagiocephaly. Note the ear and forehead go forward, unlike with unilateral lambdoid synostosis where the forehead and ear go backwards.
68
What is the incidence of craniosynostosis?
1 in 2000-2500 live births
69
What is the most common type of synostosis?
Sagittal \> Metopic \> Unicoronal \> Lambdoid
70
What causes a Harlequin sign?
Unicoronal suture synostosis causes restriction of the orbit to advance
71
What are the causes of copper beating?
Chronic hydrocephalus
Craniosynostosis
Hypophosphatasia
72
What are the features of Saethre-Chotzen syndrome?
Caused by a genetic mutation in the TWIST1 gene.
Causes unicoronal or bicoronal (brachycephaly) synostosis.
Associated with ptosis, low hairline, wide-spaced eyes and broad nose and hand/feet abnormalities.
Second and third fingers may be webbed and the big toe may have a duplicate bone.
73
What bony abnormalities are seen with NF1?
Lytic defects in the lambdoid suture
Absent orbital roof or floor causing pulsatile exopthalmos
Elevated lesser sphenoid wing
Enlarged middle cranial fossa
Enlarged CN foramina
J-shaped sella turcica
74
What do skull epidermoids look like?
Sharply demarcated skull lesions with well defined sclerotic borders. They usually disappear within a few years of discovery.
75
What causes multiple destructive foci in the calvaria?
Think TB, myeloma and LCH
76
What is the cephalic index?
CI = Biparietal distance / orbitofrontal distance.
Normal is 74-83.
77
What is dolichocephaly?
When the head is longer than expected. The cephalic index is \<74. Sagittal synostosis is a form of dolichocephaly.
78
Which craniosynostosis is associated with low-mid frequency sensorineural hearing loss?
Muenke's sydrome (61%)
Seen more in males
Uni or bicoronal synostosis
\*Note the rest of the craniosynostosis syndromes have conductive hearing loss
79
What are the features klippel-feil?
Short neck
Low posterior hairline
Limited neck mobility
80
What gene mutation causes achondroplasia?
FGFR3 gene
Autosomal dominant but 80% of cases are sporadic
81
What causes hydrocephalus in achondroplasia?
Flattened skull base causes foraminal stenosis and venous hypertension or cervicomedullary compression. This results in macrocephaly. Most stabilise so do not shunt.
82
What are probst bundles?
Non-crossing callosal axons in cases of callosal agenesis resulting in widely spaced lateral ventricles and racing car sign
83
Where are intracranial lipomas?
Most commonly in the interhemispheric fissure (50%) occupying the subarachnoid space so blood vessels and cranial nerves course through them.
Show a chemical shift artefact.
84
What is a chemical shift artefact?
Where fat is mismapped onto the image
85
What is this condition?
Note the pituitary bright spot in the floor of the third ventricle as the posterior pituitary has not formed
86
How can you differentiate a persistent blake's pouch cyst from a dandy-walker malformation?
Blake's pouch cysts have choroid plexus displaced into the cyst. These are due to an imperforate foramen of magendie.
87
What are the features of a mega-cisterna magna?
Enlarged cisterna magna
No hydrocephalus
Intact vermis
Most likely due to cerebellar volume loss
Asymptomatic patients with an incidental finding,
\*\*IF there is hydrocephalus then think Persistent Blakes pouch cyst.
88
What are the types of lissencephaly?
Type 1 = classical with lissencephaly and band heterotopia. Cortex has 4 layers.
Type 2 = Lissencephaly with cobblestone cortex. No layers.
LIS1 gene mutation
89
What lines schizencephaly?
Abnormal gray matter (polymicrogyria)
Cortical development phases are proliferation, migration and organisation. Schizencephaly can be caused by disruption of any of these
90
What is the most common location for polymicrogyria?
Sylvian cortex.
MRI shows irregular appearance of the inner and outer cortex with diffuse cortical thickening.
91
What are the considerations in a patient with a spinal dysraphism?
Cutaneous features
Neurological status (lower limb power and sphincter function)
Bowel / bladder function (neurogenic bladder) \*\*Must do urodynamic testing for preoperative evaluation\*\*
Orthopaedic (scoliosis, kyphosis, lordosis, leg length discrepancy and foot deformity)
92
What are the presenting features of a tethered cord?
Back pain
Leg pain
Regression of sphincter control
Regression of mobility
Scoliosis
93
Why do an MRI in a patient with a myelomeningocele?
Assess associated pathologies such as split cord malformation, lipoma, dermoid, epidermoid and chiari 2.
94
What did the MOMS trial show the benefits of fetal myelomeningocele repair were?
Lower risk of death and need for VP shunt insertion during the 1st year of life (68% vs 98%) with fetal closure between 18-25 weeks. There was improved motor and mental development at 30 months.
95
What is the acute management of a myelomeningocele?
Keep prone
Assess lesion then cover with sterile gauze and cling film
Prophylactic antibiotics
Surgical closure within 72 hours
96
How do you treat a neurogenic bladder in a child with spinal dysraphism?
Clean intermittent catheterisation
Antibiotic prophylaxis
Anticholinergic medications (reduce the reflex parasympathetic contraction against a closed sphincter which causes vesicoureteric reflex).
97
What causes a new or rapid progression of a scoliosis in a patient with a myelomeningocele?
Shunt block
Syrinx
Tethering
Most children with an MMC above L2 will develop a scoliosis
98
What is the difference between a lipomyelocele or a lipomyelomeningocele?
In lipomyelocele the lipoma:placode interface is within the canal whilst in lipomyelomeningocele the interface is outside the canal.
99
What substance is T1 bright but dark on STIR?
FAT
100
When does the L5 lamina fuse posteriorly in normal cases?
5-6 years
101
What is a dermal sinus tract?
An epithelium lined cavity between the skin and the CNS. Patients have a midline dimple. Caused by a failure of gastrulation due to defective notochord development.
102
What are the types of split cord malformation?
Type 1 (25%) = 2 dural sacs with a midline fibrous septum
Type 2 (75%) = 1 dural sac and no midline septum
103
What is caudal regression syndrome?
Failure of secondary neurulation resulting in spinal anomalies, imperforate anus, cloacal/genital anomalies, pulmonary hypoplasia, limb dysplasias
104
What is a dorsal enteric cyst?
Where the is a continuation of bowel through the vertebral body, spinal canal and cord to the skin
105
How do you test mothers for fetal NTDs?
Serum maternal AFP at 15-20/40
106
What fetal USS findings are suggestive of a Chiari 2?
Lemon - narrow frontally due to low pressure
Banana - posterior convex cerebellum
Microcephaly
Obliteration / full foramen magnum
107
What stage of embryological development is affected in Currarino triad?
Gastrulation (notochordal abnormality)
108
What rate do neonated produce CSF?
25 ml/day. This increases to adult capacity as a toddler.
109
What is the most common intrauterine infection that causes hydrocephalus?
Toxoplasmosis \> Mumps \> CMV
110
What is the risk of a shunt tap causing an infection?
2 in 1000
111
What is the ETV success score?
Age: \<1 month = 0, 1-6 months = 10, 6-12 moths = 30, 1-10 years = 40 and \>10 years = 50
Etiology: Infection = 0, MMC/IVH/tumour = 20, Aqueductal stenosis = 30
Shunt previously: No = 10
Provides the 6 month ETV success rate.
112
What are Lundberg waves?
Describe mean ICP patterns in patients
A = mean wave ICP \>50 mmHg lasting between 5-20 minutes. Suggests ICP exceeding cerebral compliance
B = mean wave ICP 20-50 lasting \< 5 minutes. Seen in sleep.
C = mean wave ICP \< 20 occuring every 10 seconds. Due to oscillations in baroR and chemoR control.
113
What is thought to be the underlying etiology of BESS?
Immaturity of the arachnoid villi
114
What is the triad of shaken baby syndrome?
Subdural haemorrhages
Retinal haemorrhages
Encephalopathy
115
116
What organism causes intracerebral abscesses in neonates?
Citrobacter Koseri
117
What is the LCAM1 mutation associated with?
X-linked hydrocephalus
118
What is a birds nest appearance of distal shunt tubing associated with?
Migration of distal catheter out of the peritoneum into the subcutaneous tissue
119
What is the incidence of haemangioblastoma in children?
1% of posterior fossa tumours in children
120
What is the peak age for pilocytic astrocytoma?
5-13 years
121
What is the treatment of an OPG?
Resect nerve of the blind eye to save contralateral vision if threatening the chiasm
Chemotherapy is given if \< 9 years
Radiation is contraindicated in NF-1 due to risk of moya moya and other brain tumours
122
What are DIPGs histologically?
High-grade fibrillary astrocytomas
Median survival 12 months
Treatment is with radiotherapy (54Gy)
123
Which germ cell tumours have high AFP and HCG?
Embryonal carcinomas
(AFP rise alone = Yolk sac \> Teratoma and HCG rise alone = choriocarcinoma)
124
What does a pineal tumour with a rim of exploded calcium suggest?
Pineal parenchyma tumour (pineocytoma / pineoblastoma)
125
What is the treatment of a non-germinomatous germ cell tumour?
Neoadjuvant chemotherapy, followed by resection and post-op irradiation (craniospinal radiation may be need if evidence of cs seeding)
126
What is the difference on MR Spect between a choroid papilloma and carcinoma?
Papillomas have a myoinositol peak where as carcinomas have an elevated choline peak
127
What is the treatment for medulloblastoma?
Maximal safe resection
If Standard risk \> craniospinal irradiation with post fossa boost and concurrent chemotherapy.
If high risk \> craniospinal irradiation if \>3 years, myeloablative chemotherapy followed by stem cell transplantation.
128
What does an INI1 mutation in posterior fossa tumour signify?
ATRT.
Treat with max safe resection, chemotherapy and radiotherapy
129
What is aside effect of platinum-based chemotherapy?
High freq hearing loss
130
What are the most common paediatric intramedullary tumours?
Astrocytoma 46% \> Ganglioglioma 35% \> Ependymoma 12% \> Haemangioblastoma 2%
131
What is the paediatric GCS?
E (1- no response, 2- pain, 3- voice, 4- spontaneous)
V ( 1- no response, 2- inconsolable, 3- intermittently inconsolable, 4- consolable, 5- orientated/ smiles)
M (1- no movement, 2- extension to pain, 3- flexion to pain, 4- withdrawal to pain, 5- withdraws from touch, 6- spontaneous and purposeful movement)
132
What is Reye syndrome?
A reaction to aspirin seen in children that have a viral illness characterised by vomiting, encephalopathy and hepatic dysfunction
133
What is the PECARN algorithm?
The likelihood of developing a clinically important TBI in a child GCS 14/15 and hence the need for CT.
All children GCS14 or with palpable skull fracture need a CT.
134
What is the upper limited of normal ICP in a ventilated infant and older child?
\<5 mmHg in an infant and \<10 mmHg in a child.
\*\*In the context on an LP in the lateral decubitus position then 13 mmHg is the limit in a child\*\*
135
What is the management of a growing skull fracture?
Duroplasty and autologous cranioplasty
136
What is the CPP range for children with TBI?
40-50 mmHg to keep a brain oxygen tension \>10 mmHg
137
What is the max rate of rewarming after induced hypothermia in a child?
0.5 C/hour
138
What metabolic condition should be ruled out in patients with suspected NAI?
Glutaric aciduria type 1
139
How do you treat SCIWORA?
12 weeks in a brace
140
How do you confirm atlanto-axial rotatory subluxation?
Dynamic CT with 3 positions (neutral, left and right)
If does not resolve within 4 weeks attempt reduction/manipulation and then immobilize. Surgery is needed if irreducible.
141
What is odontoid epiphysiolysis?
Fracture through the C2 dentocentral synchondrosis. This fuses at 7 years and is vulnerable in young children.
Fractures through the epiphysis will heal with reduction and immobilisation using a halo for 10 weeks.
142
How many pins are used with a Halo?
\<2 years = 12
2-7 years = 8
\>8 years = 8
Torque in lb/inch is the same as the age.
143
What is the first-line management of a child with an unstable subaxial injury and no neurology?
HALO for 3 months
144
What is more likely to heal, a bony fracture or purely ligamentous injury?
Purely ligamentous has a lower rate of of healing
145
What is the difference between a cephalhematoma and a caput succedaneum?
Cephalhematomas are subperiosteal haematomas and no to cross sutures
Caput succedaneum is a soft tissue scalp swelling after delivery
146
What is pseudosubluxation?
Normal translation \< 4mm at C2/3 that occurs in children \< 8 years due to ligament laxity. Look at the spinolaminar line between C1-3 to differentiate from a true injury.
147
What causes a vein of galen malformation?
Persistence of a single median prosencephalic vein of markowski (which is the primitive internal cerebral vein). As a result normal beep veins are absent.
148
What is the fetal function of the median prosencephalic vein of Markowski?
It drains the choroid plexus between the 5-10th week of fetal life. With the development of the thalamus, the median prosencephalic vein regresses and the internal cerebral veins take over.
149
What is the difference in presentation between children and adults with Moya Moya?
Children present with TIAs and Strokes.
Adults present with bleeds.
150
What are some risk factors for Moya Moya?
Asian origin
Radiotherapy/Proton beam
NF-1
Down's syndrome
151
Where do moya moya related bleed tend to occur?
Basal ganglia
Thalamus
Mesial temporal lobe
Intraventricular
(as this where prominent collaterals form from the posterior circulation)
152
Why do you need to assess the collateral supply pre-operatively in Moya moya patients?
So that surgery does not disturb them!
153
What is the classification system for Vein of Galen malformations?
Lasjaunicas classsififcation:
Choroidal vs Mural
Choroidal type has feeders from the chorodal A, thalamo-perforators and pericallosal A that enter the anterior part of the median prosencephalic vein in the velum interpositum. These present in young children with cardiac failure.
Mural type has feeders from the collicular and posterior choroidal A that enter the inferior lateral part of the median prosencephalic vein and present with failure to thrive and macrocephaly due to hydrocephalus from impaired venous outflow.
154
What is the treatment for vein of galen malformations?
Transarterial embolization when the arterial feeders are large enough to hold a microcatheter.
Transvenous when the arteries are of small calibre and in very high flow shunts
155
What are the characteristic features of Wyburn-Mason?
Retinal angiomatosis, facial haemangioma and midbrain AVM
156
What are the common approaches to brainstem cavernomas?
OZ, pterional and subtemporal for ventrolateral and lateral
Transpetrous, retrosigmoid and combined approaches for dorsolateral
Suboccipital for dorsal
157
Why does crying and hyperventilation cause strokes in Moya Moya?
Lowers the pCO2 and the resulting vasoconstriction causes ischaemia
158
What are the common features of Marfan's syndrome?
Arachnodacyly, high arched palate, lens dislocation, pneumothorax, mitral valve prolapse and intracranial aneurysms
159
How is spasticity graded?
Ashworth scale
Note, score of 3 are candidates for intrathecal baclofen. If a test dose of 25-50 micrograms improves by 1 point then it is successful
160
What are the main risk factors with intrathecal baclofen pumps?
Headache
CSF leak (5-15%)
161
Where do you place the catheter tip of an intrathecal baclofen pump?
T6-10 for diplegia
T1-2 for quadriplegia
Midcervical region for dystonia
162
What are the different parts of a baclofen pump?
Central port for drug filling (yellow arrow)
The red circle is the pump
Catheter access port for troubleshooting (blue arrow)
Catheter connector (pink arrow)
163
What level rhizotomy reduces spasticity in the hip flexors?
L1!
164
What is the mechanism of action of phenytoin and carbamazepine?
Na channel blockers!
165
What is the DBS target for dystonia?
Bilateral GPi
166
What are the features of baclofen withdrawal?
Fevers, seizures and rebound spasticity
167
What alpha-2-blocker is used in spasticity management?
Tizanidine
168
What is a characteristic feature of dystonia?
The body parts affected get worse when they move other parts of the body (twisting / patterned movements)
169
What dystonia mutation results in an abnormal TorsinA protein?
DYT1
170
How does trihexyphenidyl work?
Antimuscarinic used in the treatment of dystonia
171
What is the risk of SAH during pregnancy?
Not increased during pregancy, labor or puerperium
172
How do you treat a pregnant patient with a brain tumour?
Same treatment as non-pregnant. Try to delay surgery until the second trimester.
Fetal exposure to chemotherapy and radiotherapy to the mother has not shown adverse effects and the fetus should be shielded!
173
How do you treat SAH in pregnancy?
If \<26 weeks then do what is best for the mother and then allow pregnancy to progress to test
If \>34 weeks then C-section and aneurysm treatment.
Between 26-34 weeks treat the aneurysm first and then allow pregnancy to progress to test
174
When ar AVM ruptures most likely to occur during pregnancy?
1st and 2nd trimester. Note that the risk of rupture is no higher during pregnancy compared to the rest of life. Elective C-section should be performed if untreated or partially treated AVMs
175
Which hormone causes ligamentous laxity in pregnancy?
Relaxin
176
How do you treat Cauda equina syndrome in pregnancy?
If \>34 weeks then C-section and discectomy
If \<34 weekness then discectomy can be prone using a 4-poster frame or in the lateral decubitus position.
177
What is the risk of childhood cancer in a fetus whose mother had a Ct head?
1 in a million
178
How do you treat eclamptic seizures?
Magnesium via a 6g loading dose IV followed by 2g/hour IV infusion - goal is to achieve a serum level of 4-8 mg/dl
179
What is the risk of coiling to a fetus?
Fetal radiation exposure is very small - no additional risk to the fetus
180
How should you manage delivery in a patient with a VP shunt?
Prophylactic antibiotics during labour and delivery.
If the patient has no symptoms of raised ICP then vaginal delivery is prefered. If raised ICP then GA and C-section. Note epidural anaesthesia is contraindicated with elevated intracranial pressure.
181
If a pregnant patient has a seizure during pregnancy what should you do?
Rule eclampsia (high BP and protein in urine)
Then tap the shunt measuring the opening pressure and MC&S
182
What genes are involved with VoGM?
RASA1 EPHB4 HHT
183
Why do VOGM form?
Do due presence (failure of regression) of the median prosencephalic vein of markowski
184
How do VoGMs present?
Early with high output cardiac failure (right to left shunt with reverse flow - Eisenmenger’s) causing systemic ischaemia and multi-organ failure Late with hydrocephalus from venous hypertension
