Neurology Flashcards
Cerebral palsy; definition, epi, aetio.
CP is a disorder of posture and movement due to non-progressive damage to the developing brain.
Presents by the age of 3yrs, 2per 1000 live births.
Aetio
-80% are antenatal in origin (due to genetic defects)
-10% due to HIE during delivery (preterms are esp vulnerable to brain damage from periventricular leucomalacia)
-10% are postnatal (meningitis, TBI, hydrocephalus, hyperbilirubinemia…)
Differentiating CP with other neurological conditions.
In CP, the child will not lose any milestones they’ve already gained even if motor disorder will evolve w/o Rx. (The milestones may be delayed)
In other neuro disorders, there is progressive loss of already gained milestones.
Classification of cerebral palsy.
Spastic 90%
Dyskinetic 6%
Ataxic 4%
Hemiplegic, diplegic, quadriplegic, monoplegic & triplegic
Categorisation of severity in CP.
Gross Motor Function Classification System (GMFCS)
I= walks w/o limitations
II= walks w/ limitations
III= walks w/ handheld mobility devices (frames)
IV= self mobility w/ limitations (may use powered ones)
V= transported in a manual wheelchair
Spastic CP features
Damage to the UMN in the pyramidal/corticospinal tract with spasticity and exaggerated reflexes. May have initial hypotonia.
Has types; hemiplegia, diplegia, quadriplegia
Dyskinetic CP features
Basal ganglia are affected with affection of the upper limb being more severe.
Most commonly due to HIE and kernicterus
CF are floppiness, poor trunk control and delayed motor development in infancy.
Ataxic CP features
Parkinsonism traits in gait etc and usually affects the same side and are mostly genetically determined.
Mx of CP
MDT consisting of parents, paediatrician, physiotherapists, occupational therapists, ENT surgeons, eye surgeons, orthopaedic surgeons, social workers, community care nurse and teachers.
Drugs used in CP
Baclofen to reduce muscle tone
Bensexole (Artaine) to reduce dystonia
Botulinum toxin
Surgery for contractures
Bacterial causes of meningitis in different age groups
Neonate - 3 months = GBS, E.coli, Listeria
1m- 6yrs = Neisseria meningitidis, S. pneumoniae, H. influenza
>6 yrs = Neisseria, Strept
When to consider neuroimaging in meningitis?
When fever doesn’t settle with ABx
Signs of brain abscess
Signs of increased ICP
In focal (one-sided) seizures
Contraindications for LP(8)
Cardiorespiratory instability
Focal neurological signs
Signs of increased ICP
Coagulopathy
Thrombocytopenia
Location infection at site of LP
Shock
No consent
CSF findings in partially treated meningitis
Lymphocytes will be high instead of neutrophils, proteins remain high, sugar remains low and culture is -ve.
Complications of meningitis
Hearing loss
Local vasculitis leading to cranial nerve palsies
Local cerebral infarctions
Subdural effusions
Hydrocephalus
Cerebral Abscess (vanco and drainage)
Causes of un-resolving fever in meningities
Wrong ABx and/or dose, viral/fungal aetio, TB, formation of a brain abscess
Common causes of encephalitis
HSV (ass. w/ temporal lobe epilepsy) Rx acyclovir IV
JE
Dengue
Clinical features of Febrile Seizures
Generalised
<15 minutes
One episode/day
No brain damage
Mx of febrile seizures
Advise parents to not out anything in the mouth during the seizure, and if it lasts more than 5 min; to hospitalise the child
Antiepileptics (eg: clobazam) is given only during fever days
Complication of Guillain-Barre Syndrome
Miller- Fischer Syndrome; ataxia, areflexia and external ophthalmoplegia
Duchenne’s muscular dystrophy; aetio, epi
X-linked recessive disease that affects the gene coding for dystrophin leading to necrosis of muscle cells and their replacement with fat
Usually present by 3-5yrs
Duchenne’s; CF
Problems in gross motor development milestones
Gower’s sign (stand using arms)
Pseudohypertrophy of calf muscles
Waddling gait
Language delay
Duchenne’s; Prognosis and complications
Poor prognosis w/o Rx; death by late 20s due to respiratory failure or ass. cardiomyopathies (dilated)
If good RX; 50-60s w/ loss of independent ambulation.
Scoliosis is another common complication
Duchenne’s; Investigations
Serum creatinine phosphokinase is markedly elevated (x1000)
Muscle biopsy
DNA testing
Duchenne’s; Mx
Medical = corticosteroids 10d each month
MDT consisting of physiotherapy, paediatricians, speech therapist, occupational therapist, orthopaedics, social worker etc
Causes of Bell’s palsy in children
CP angle tumour
Parotid enlargement due to mumps
Forceps delivery
Herpes
Idiopathic
Juvenile myasthenia; patho, epi, CF
Progressive muscle weakness throughout the day due to declining levels of acetylcholine due to antibodies against ACh receptors.
Presentation is usually by 10y/o w/ ophthalmoplegia and ptosis, difficulty chewing and loss of facial expression (+generalised muscle weakness).
Juvenile myasthenia; Ix, Mx
Ix = IV edraphonium over minutes followed by pyridostigmine/neostigmine over days in the HDU/ICU
Mx = ACh inhibitors; pyridostigmine, neostigmine, immunosuppressive therapy, immuno-modulating drugs (MMF, prednisolone, azathioprine)
Thymectomy is thymoma present
Plasma exchange is used during a crisis.
Neurofibromatosis 1; genetics, criteria
De novo (50%) mutation of the neurofibromin-1 (NF-1) gene w/ autosomal dominant inheritance
Criteria; >2 more of the following;
->6 cafe-au-lait spots prepuberty, >15 after puberty
->1 neurofibroma
-axillary freckling
-optic glioma
-1 Lisch nodule (hamartoma on the iris seen on slit-lamp exam)
-bony lesions from sphenoid dysplasia
-1st degree relative w/ NF1
Neurofibromatosis 2; gene, CF
Less common, autosomal dominant inheritance of the NF-2 gene presenting w/ multiple inherited Schwannomas, meningiomas and ependymomas
Associated conditions w/ neurofibromatosis
MENS (endocrine disorders)
Phaeochromocytoma
Pulmonary HTN
Renal artery stenosis
Tuberous sclerosis; cutaneous features
Depigmented ash leaf patches or a melanotic naevi (light up under UV)
Shagreen patches; roughened patches of skin over the lumbar spine
Angiofibromata (adenoma sebaceum) over the bridge of the nose and cheek; may be present from birth/ present at 3yrs
Tuberous sclerosis; neurological features
Infantile spasms
Epilepsy (often focal)
Intellectual disability (often w/ autism)
Tuberous sclerosis; other features
Subungual fibromata
Phakomata (dense white areas on the retina from degeneration)
Rhabdomyomata of the heart in infancy that resolves
Angiomyolipomas
Polycystic kidneys
Cysts in lungs
Tuberous sclerosis; Mx
Seizures; vigabatrine
cardiology follow-up
USS KUB
Special education
Sturge-Weber syndrome
Sporadic disorder w/ a haemangiomatous facial lesion (port-wine stain) in the Cr5 distribution and ass. intracranial lesion (leptomeningeal angioma)
There is a high risk of ipsilateral glaucoma
DDx of meningocele vs. meningomyelocele
Meningocele; covered by skin and nerve tissue is normal
Meningomyelocele; not covered by skin therefore is at a higher risk of rupture, infection and meningitis. Nerve tissue lies outside
Myelomeningocele associations
Variable paralysis of the legs
Muscle imbalance
Sensory loss
Bladder denervation
Bowel denervation
Scoliosis
Hydrocephalus from a Chiari malformation
Types of hydrocephalus with their relative causes.
Communicating = obstruction at the arachnoid villi (the site of absorption of CSF).
- SAH, meningitis
Non-communicating = obstruction w/i the ventricular system or aqueduct.
- Congenital malformation (Chiari, aqueduct stenosis), posterior fossa neoplasm or vascular malformation, intraventricular haemorrhage in a preterm infant
10 features to look for in hydrocephalus
Sunsetting sign
Papilloedema
Cushing’s reflex; HTN w/ bradycardia
Examine shunt
Features of shunt infection
Association; meningomyelocele
Hepatosplenomegaly
Severity
Vision, hearing, bowel and bladder function
