Nervous system Flashcards
(33 cards)
- ascending weakness and hyporeflexia. preceeding viral illness.
next step in management?
spirometry!!!
patient likely has GBS
6 months
cherry red macula and developmental milestone regression, arreflexia and hepatosplenomegaly
mostl ikely diagnosis?
sphingomyelinase deficiency!!! = Neimann pickk disease
not B hexosaminidase A deficiency/Tay sachs disease as it would cause similar symtoms but not hepatosplenomegaly or areflexia
galactocerebrosidase deficiency = krabbe disease = developmental regression, hypotonia and areflexia only
glucocerebrosidase deficiency = gauchers disease = anemia, thrombocytopenia and hepatosplenomegaly
lysosomal hydrolase deficiency/hurlers syndrome = coarse facial features, inguinal and umbilical hernias, corneal clouding, hepatosplenomegaly
bilateral retinal hemangioblastomas and FH of adrenal tumour.
most likely diagnosis?
Von hipple lindau disease!!! = retinal and cns hemangioblastomas. renal cell cancer, phaoes.
NOT neurofibromatosis 1 as it causes HYPERpigmented macules. the isolated hypopigmented patch on the patient was normal
associated with optic gliomas and iris harmartomas/lisch nodules instead.
14 yo. Chronic lower back / leg/ buttock pain + neurological deficits eg hyporeflexia in lower limbs, weak ankle dorsiflexion, gait abnormalities thin weak lower extremities muscle atrophy + lumbosacral anomaly eg lipoma + scoliosis
Most likely diagnosis?
Tethered spinal cord!! - other signs include foot deformities! and bladder dysfunction!,. can be part of spinal dysraphism/spina bifida
Contrast spastic diplegia which causes hypertonia and hyper flexia and less acute onset
NOT backers muscular dystrophy as chronic back pain and lipoma not expected
Developmental delay, microcephaly, small palpebral fissures, in distinct nasal philtrum . Uncle with Down syndrome. Most likely diagnosis?
Alcohol use in pregnancy
Learn how in utero phentoyin exposure presents
Baby born at less than 28 weeks old with episodes of apnea, what is this called? Management?
Apnea of prematurity
Reassurance
Headache plus siezures plus limb weakness in child. Single word answers/aphasia despite recent normal development. CT normal, eeg ordered, next step in management?
MRI head to rule out stroke
Obstructive hydrocephalus in newborn, gaze impairment. Most likely cause?
Vitamin K deficiency!!
Causing intracerebral bleeds
Infants growth chart shows head circumference below the 3rd percentile so microcephaly!! Plus developmental delay and hypotonia. First step in management?
MRI brain
Excessive daytime sleepiness, intellectual impairment or behavioural difficulties.difficulty opening hands after squeezing examiners fingers. Most likely diagnosis?
Myotonic dystrophy!
Not cerebral palsy as this occurs wtith hypertonia and hyper reflexia not myotonia
Redness, tearing of left eye. Photophobia and enlarged globe.
Facial port wine stain so likely underlying sturge Webber syndrome
First step in management?
Tonometry! = glaucoma in child!
Not conjunctivitis as doesn’t cause globe enlargement and causes purulent drainage
Neuroblastoma causes abdominal cancer tumour not brain primary
Mechanism behind a concussion?
Neuronal functional disturbance!!
NOT disruption of white matter tracts as this is seen in diffuse Axonal injury = severe trauma such as shaken baby syndrome and head impact during car collision - patients are severely obtunded or comatose
Chorea/twisting movements of limb and torso in infant, dystonia, hearing loss, gaze abnormalities such as cross eyed. Cephalohematoma at birth. Most likely cause?
Bilirubin induced neurotoxicity!! Kernicterus
Not autoimmune injury to basal ganglia as this happens in rheumatic fever
Post operative migraine can develop due to inadequate hydration. Stress and fasting are other triggers
Acute causes of hemiplegia in children?
Stroke, ICH, siezures, Hemiplegic migraine
Muscular dystrophy symptoms?
Weakness of facial and hand muscles, grip dystonia, cardiac conduction abnormalities, gi disturbances eg dysphagia, testicular atrophy
Autosomal dominant
NF type ONE is characterised by the dark spots, skin neurofibromas, inguinak and axillary freckling, scoliosis and what change to the eyes?
Optic pathway gliomas!!,
And lisch nodules!!
Increased siezure risk !!,!
Not acoustic neuromas - seen in NF2
Baby had siezure.mother said rolled off bed last night when sister was changing him. But also has large head and retinal hemorrhages and papilloedema
Subdural venous shearing!,
Shaken baby syndrome!!! - subdural hemorrhage causes enlarging head and siezures and papilloedema
Soreness in legs, difficulty standing today, tingling sensation in toes and fingers, lower back pain. Decreased strength and diminished reflexes I lower extremities. Bradycardia. What is most associated with underlying illness?
Recent diarrhoeal illness!
Parenthesias early are common, as well as neuropathic pain (back pain with no spinal tenderness) and autonomic dysfunction (arrhythmias bradycardia, lieu’s) in young people !
Respiratory compromise
Tongue lacerations is a common complication of siezures!
How long to rest after a concussion?
2 days of rest, followed by gradual return to activity
Neighbours baby passed away whilst sleeping;
Smoking exposure!!- most important - prenatal and post natal
But also avoid prone and side sleep positions
achondroplasia includes gross motor delay. not seen in nutritional rickets.
costochondral joint hypertrophy/prominences felt in rickets but not achondroplasia
