I-R Flashcards
(41 cards)
What is an Intraventricular haemorrage (IVH)?
IVH arising in the germinal matrix and periventricular parenchyma.
· Grade I: germinal matrix
· Grade II: blood in lateral ventricle, no dilation
· Grade III: blood in lateral ventricle, dilation
· Grade IV: GIII with periventricular parenchymal haemorragic infarct
What is the aetiology of Intraventricular haemorrage (IVH)?
Prematurity (major factor), High variability in BP (hypovolaemia, hypotension, pulmonary haemorrage, mechanical ventilation, shiftsi n pO2 and pCO2, PDA)
Other rare causes include acidosis, hypothermia, HIE, RDS, PTX, coagulopathies.
What is the epidemiology of Intraventricular haemorrage (IVH)?
Common in very low BW: 60% of <1kg. Maternal smoking.
What would you find in the history or exam of Intraventricular haemorrage (IVH)?
Highest in first 72h of life. 60% within 24h.
May be asymptomatic: Grade I/II) or seizures, poor tone, aopnea, lethargy, shock, anaemia.
Signs of raised ICP: bulging fontanelle, Cushing (high BP low HR). Head circumference.
What investigations would you do for Intraventricular haemorrage (IVH)?
Bloods: FBC, clotting, glucose, blood gas for acidosis and distress
USS: in dx and classification of IVH
What management would you use for Intraventricular haemorrage (IVH)?
Routine screening: USS in infants <32wk in first week of life, rpt in 2 week.
Prevention: maintain stable glucose, O2, BP etc
Supportive care: ventilation, transfusion if large.
Treat ICP: hypertonic saline and mannitol.
External ventriculostomy or permanent VP shunt may be required.
What are the complications and prognosis of Intraventricular haemorrage (IVH)?
Hydrocephalus (10-15%) and neurological impairment.
Grade I /II: Rarely have neurological consequences, as they originate in ventricular matrix which disappears. Grade III: 30-45% incidence of neurological impairment. Grade IV: 60-80% incidence of neurological impairment.
What is an Meningitis?
Infection of the subarachnoid space associated with inflammatory response of the meninges.
What is the aetiology of Meningitis?
Bacterial
· <3 month: GBS, E.Coli, Listeria
· 1m-6y: Meningococcal, Step Pena, HIB
· >6y: Meningococcal, Strep Pena, mumps
· TB: can cause meningitis at all ages. 6m-6y most commonly.
Viral
· Enter viruses (80%), CMV, arbovirus. HSV (but more commonly encephalitis)
What is the epidemiology of Meningitis?
Viral more common, 3000 py UK. Bacterial 2000/yr. UK.
Related to immunosuppression (young, HIV, defects of complement -> meningococcal susceptibility, splenii due to sickle cell). High crowding and contact RF.
What would you find in the history or exam of Meningitis?
Prodromal infection: otitis, tonsillitis, rasp, GI symptoms.
· Infants: fever, hypothermia, irritable, lethargic, seizure, poor feeding.
· Children: fever, headache, neck, photophobia, Kernigs, rash (non blanching)
Neck stiff, Kerning, non blanching rash (purpuric of petechial), high ICP (papillodema, low consciousness, focal neurology, cushing, decerebrate posturing).
What would you find in the investigations of Meningitis?
Bloods (+culture), MCS, CT scan to judge ICP.
LP if allowed by ICP -> BACTERIAL has low Neut, low Gluc, high Prot,. VIRAL has high Lymph, n Gluc, n Prot. MCS, AFB test, and viral PCR should be preformed.
What management would you use for Meningitis?
Resuscitation: ABC. Start empirical high dose antibiotic as indicated by trust.
· Bacterial: 3 gen cephalosporin. <3month, ampicillin.
· TB: 6-9 months of RIPE + streptomycin.
?corticosteroids if HIB, supportive care, notification of CDC, contact prophylaxis with rifampicin if meningococcal infection.
What are the complications and prognosis of Meningitis?
During: seizure, odema, shock, DIC, sepsis. After: hear/sight loss, hydrocephalis, abscess, disability
Bacterial mortality 5-10%. Complicaitons 10-20%. Viral 5 have sequelae.
What is
Autosomal dominant multisystem disorder, characterized by progressive muscle wasting, weakness, and myotonia (abnormal sustained contraction)
What is the aetiology of
Genetic defect: caused by CTG expansion at the 3/UTR of the MD gene on chr 19 (DMPK)
Genetic anticipation: children of affected will have more rpt -> earlier disease.
What is the epidemiology of Myotonic dystrophy?
2/10k live births UK. Severe type very rare.
What would you find in the history and exam of What is the epidemiology of Myotonic dystrophy?
Depends on CTG repeat number
· Unaffected 2-27 rpt
· Mild MD: 50-100 rpt, cataracts, muscle weakness in adulthood
· Classic MD: 100-1000 rpt, myotonia, muscle waste, frontal bald, hypogonadism, arrhythmias, DM, resp impairment, cardiomyop, adverse reaction to anaesthesia.
· Congenital MD: 1000-4000 rpt
o @birth: hypotonia, resp and feeding difficulties, marked facial weakness. Myotonia not feature yet.
o If survives birth, gradual improvement of muscle strength, slow development, persistent facial weakness, severe LD, classic features of MD by 10.
Peripheral weakness: hands (unable to release grip), forearm and feet. (differentiated by other dystrophies by the fact that weakness proximal in others)
Myopathy facies: facial muscle weakness and wasting, bilateral ptosis, wasting of frontalis and temporalis, sternomastoids
Which investigations would you do for What is the epidemiology of Myotonic dystrophy?
Bloods: high CPK. Muscle biopsy diagnostic. EMG: characteristic diver bomber discharge by age of 3.
DNA mutation analysis for size of repeat array.
What is the management of What is the epidemiology of Myotonic dystrophy?
MDT approach. Physio, SALT, OT, genetic, psychological counseling.
Medical: myotonia may be treated with procainamide and quinine. Support resp/GI problems. Surgical cataracts operation. Orthopaedc intervention for ankle/foot drop.
What is the complications and prognosis of What is the epidemiology of Myotonic dystrophy?
Joint contractures, foot deformities, early onset dementia.
Depends on number of CTG repeats, extent of LD, MDt involvement. Most do not survive past 50.
What is Neurocutaneous syndromes?
Disorders of ectodermal tissue, leading to defective neural and skin tissue.
What is the aetiology of Neurocutaneous syndromes?
Transmitted 50%, de novo mutations 50%.
· NF1 (Von reickenghausen): mutation in chr17 on TSG neurofibromin
· NF2 (acoustic neuromas): mutation on chr22
· TS1: Mutation on c9 coding for TSG harmatin
· TS2: Mutaiton on chr16 for TSG tuberin
What would you find in the history and exam of Neurocutaneous syndromes?
NF:
· Café au lait spots (>6 with 5mm diameter, >to 15mm in puberty)
· Freckling
NF1:
· Skin neurofibromata over peripheral and cranial nerves, may compress à well defined tumors of elongated spindle cells and pleiomorphic fibroblast cells.
· Lisch nodules in eyes, iris harmatomas,
· Bony lesions (sphenoid dysplasia leading to fracture of long bones, unhealing)
NF2:
· Deafness/tintinnus, headaches, possible facial weakness, cerebellar ataxia
· Bilateral tumors of the CN8 ,can cause damage to neighboring nerves too.
TS:
· Skin: ash leaf lesions that fluoresce under UV (Wood’s light)
· Acne like rash, adenoma sebaceoum in butterfly distribution over nose
· Shagreen patch
· Developmental delay with learning disability
· Infantile spasms and epilepsy
· Renal angiolipomatas, flank pain, haematuria, HTN
· Cardiac rhabdomyoata, renal harmatomas
· -> multisystem involvent of small, benign tuber like growths of CT in the brain, kidney, heart, eyes, lungs etc.
