Neuro Exam Notes Flashcards
Tx for ADHD
Stimulants:
Methylphenidate (Ritalin and Concerta)
Amphetamine (Adderall and Dexedrine)
Nonstimulants:
Atomoxetine
Clonidine
Side effects of anti epileptic drugs (5/6)
Sedation
Agitation
Headache
Rashes
Hair loss
Tremors
Swollen gums
Features of Trisomy 21
epicanthic folds on widely spaced eyes, poor tone, single transverse
(“simian”) palmar crease, sandal gap between 1st and 2nd toes, 3rd fontanelle,
associated cardiac murmur from ventricular septal defect.
List the manifestations of neurofibromatosis NF1 (presentation) (9)
Cafe-au-lait spots
Axillary freckling and in groin area
Neurofibromas
Lisch nodules (small nodules in iris of eye)
Optic Glioma (tumour in optic nerve)
Bone deformities (scoliosis or bowed lower leg)
Learning difficulties
Larger than average head
Short statue
Differentiate between NF1 and NF2
NF1 tumours develop anywhere in the nervous system including brain, spinal cord and nerves, dx early in childhood by age 10
NF2 is less common, tumours develop in nerves that carry sound and balance from inner ear to brain, in both ears (acoustic neuromas) resulting in hearing loss also called vestibular Schwannoma.
Manifestations of NF2 (4)
Gradual hearing loss
Ringing in both ears
Poor balance
Headaches
What is Schwannomatosis
Rare type of Neurofibromatosis
Affects people after 20. (25-35)
Tumours develop in cranial, spinal and peripheral nerves. Rarely affects nerve carrying sound and balance, don’t affect both ears like NF2.
Symptoms of Schwannomatosis
Chronic pain
Numbness or weakness in various parts of the body
Loss of muscle
What can cause delay in closure of fontanelle
Normal variation
Hydrocele
Hypothyroidism
Bone disorders
AVM
Causes of meningism
Meningitis
Encephalitis
Acute OM
Severe tonsillitis
Pneumonia
Cervical lymphadenitis
How would palsies in CN 3 (oculomotor), 4 (Trochlear), and 6 (Abducens) be apparent
III- ptosis and mydriasis down and out
IV- inability to adduct with downwards gaze there may be a head tilt
VI- inability to abduct eye
What conditions cause Developmental regression in paeds
HIV encephalopathy
Subacute Sclerosing Panencephalitis
Inherited metabolic disease involving brain
Infections and tumours
Types of acute recurrent headache
- Migraine
-Throbbing
-Unilateral
-Aura present
-Relieved by sleep
-Ass/ nausea and vomiting
-Family hx
Common in childhood, characterised by episodic, periodic paroxysmal attacks of vasoconstriction and vasodilatation of cerebral vessels following waves of cortical depolarisation
Causes of chronic progressive headaches
Raised ICP
Traction headaches
Brain tumours
Space occupying lesions
Benign raised intracranial pressure
Causes of chronic non progressive headaches
Tension headache
Psychogenic headache
Medication overuse
Triggers or precipitating factors for migraines
Anxiety
Stress
Fatigue-lack of sleep
Head trauma
Exercise
Menstruation
Diet (tyrannies containing food-release serotonin, ice cream, red wine, cheese)
Medication- contraceptive
Dose of paracetamol in paeds
15-20mg/kg/dose
Prophylactic meds for headaches in paeds
- Sodium channel blockers (sodium valproate)
- Beta-blockers -Propanolon (don’t use in asthmatics)
Use in severe cases, most children won’t need this
What areas of the brain maintain consciousness (awareness of self and environment)
- Ascending Reticular Activating System (ARAS) in brain stem (maintains aroused state)
- Cerebral hemispheres (controls contents of consciousness)
causes of acute non-recurrent headaches
Infection and fever
Toxins
Post convulsions
Anaemia
Electrolyte imbalance
HPT
Post LP
Hunger
Trauma
What causes acute and recurrent headaches
Migraines
Management of comatose patients
8 Principles
1.Maintain oxygenation
2. Maintain circulation
3. Control glucose
4. Lower ICP
5. Stop seizures
6. Treat infections
7. Restore acid base balance and electrolytes
8. Adjust body temperature.
(jUst think A,B,C then Hs and Ts applicable here)
What are the features of an organic brain lesion
Severe headache of recent onsert
chronic or progressive
localised pain
Wakes the child up at night
Exacerbated by straining or change in position
Associated with neurological symptoms and signs
Change in headache pattern or severity
dIfferentiate between a communicating and a non-communicating hydrocele
Communicating is due to failure to reabsorb CSF
-Subarachnoid haemorrhage
-Meningitis (e.g. TB, Pneumococcal)
Non-communicating is due to obstruction in the ventricular system
-Congenital malformation
1. Aqueduct stenosis
2. Atresia of the outflow of the foramina of the 4th ventricle
3. Chiari malformation
- Posterior fossa neoplasm or vascular malformation
-Intraventricular haemorrhage in a preterm infant
How do you manage raised ICP
EVD- External Ventricular Drainage or Shunt
Define epilepsy
2 or more unprovoked epileptic seizures more than 24 hours
what is status epilepticus
Epileptic seizures that last more than 5min or more than 2 discrete seizures with incomplete recovery of conciousness in between
Outline the aetiology of epilepsy
Structural
Infections
Genetics
Metabolic
Immune
Unknown
Causes of neonatal seizures
HIE
Intracranial haemorrhage
Cerebral infarct
Metabolic causes (hypoglycemia)
Infections
Drug withdrawal eg heroin
cortical dysplasia and malformations
RIsk factors for epilepsy following febrile seizures
Complex febrile seizures
Afebrile seizures in a 1st degree relative
Abnormal neurological signs prior to the febrile seizures
Management of febrile seizures
Antiepileptics (decrease core temp)
Antiseizure not indicated for febrile seizures
FIrst aid advise
Recue medicine for seizure lasting more than 5min
EEG nor indicated
LP to exclude meningitis in less than 18months
Neuroimaging in complex febrile seizures
What is West’s syndrome and what is the classical triad of West’s syndrome
infantile spasms syndrome was also called West syndrome. Infantile spasms syndrome is considered an epileptic encephalopathy, conditions in which children have both seizures and cognitive and developmental impairments
- Epileptic spasms (extensors or flexors
- Hypsarrhythmia on interictal EEG
- Cognitive and developmental regression
Treatment of West’s syndrome
Hormonal
-0ACTH
-Prednisone
Vigabatrin
Tx for childhood absence seizures
Firts line tx is Ethosuximide, Valproate, lamatrogine (less effective but side effects)
What is the prefered drug of choice in childbearing age women for epilepsy
Lamatrigine
WHat are the causes of hypotonia (floppy child)
Lower Motor Neuron lesions
-Anterior horn cells (spinal cord)
1. Spinal muscular dystrophy (Hereditary)
2. Poliomyelitis (Acquired)
-Nerve Roots
3. Gullian Barre Syndrome
-Peripheral nerves
4. Demyelinating e.g Infectious polyneuritis, Peroneal muscular atrophy, Leucodystrophies
5. Diabetes, Porphyria (Axonal)
6. Hereditary motor sensory neuropathy
-Myoneural junction
7. Myasthenia gravis
Muscle
8. Congenital myopathy
9. Muscular dystrophy
10. Metabolic myopathoies
11. Myotonic syndromes
Central or Generalised conditions
1. Cerebella disorders
2. Birth trauma
3. Hypotonic CP
-Chromosomal
4. Down syndrome
5. Prada Willi syndrome
6. MArfan syndrome
7. Ehlers danlos syndrome
-Metabolic disorders
8. Hypercalcemia
9. Rickets
10. Hypothyroidism
11. Renal Tubular Acidosis
12. Coeliac disease
Clinical features of spinal muscular atrophy (SMA)
Proximal muscle weakness and wasting
Facial weakness in 50%
Tongue fasciculations
Absent or reduced tendon reflexes
Sweating in soles and palms
(just think LMN lesion presentation)
Differentiate between the different types of Spinal Muscular Atrophies (SMA)
Type 1:
-Presents in first few weeks of life
-Generalised Hypotonia
-Marked weakness and wasting of trunk, neck and limbs, legs more than arms
-Bulbar weakness with difficulty sucking, swallowing and cough and cry weak
-Intercoastal muscle weakness, with difficulty breathing and bell-shaped chest. Infants prone to Pneumonias
-Tendon reflexes absent
-Contractures
-Internal rotation of arms and hands with hands facing outwards
Type 2
-First few months of life
-Slower and less progressive, clin features not as severe as in Type 1
-Involvement of Resp system
-Tendon reflexes reduced rather than absent
-Tongue fasciculations
Type 3
-Usually have normal milestones in the first year but may not be able to walk
which gene is associated with Spinal muscular atrophy (SMA)
It’s an Autosomal Recessive inherited condition
Gene for SMA is located on Chromosome 5
Which organisms are associated with Guillain Barre syndrome
Viruses: Coxsackie, Echo virus, HSV
Campylo Jejuni
Epstein barr virus
Causes of peripheral neuropathy in children
Drugs (Isoniazide, Vincristine)
Toxins (lead, mercury, gold, benzene, glue)
Hereditary motor and sensory neuropathy
Chronic inflammatory demyelinating polyradiculopathy
Metabolic neuropathies
Diabetes
Vitamin deficiencies
Krabbe disease
Tx of Myesthenia gravis
Neostigmine or Pyridostigmine
Avoid aminoglycosides antibiotics, worsen it
Treatment of hypersalivation
Atropine
(anticholinergics)
What is the most common muscular dystrophy in children
Duchenne’s muscular dystrophy (DMD)
-Progressive weakness of proximal muscles due to deficiency of the subsarcolemmal membrane protein DYSTROPHIN
-X-linked
Features of Duchenne’s dystrophy
Frequent falls
Abnormal gait
Difficulty climbing stairs
Delayed motor milestones
Toe walk
What is Gowers sign
-Seen in Muscular dystrophies: Duchenne’s muscular dystrophy
-Child goes from supine to prone position, then a knee elbow position, then extending knees and elbows and slowly climb with legs supporting
Tx of DMD (Duchenne’s Muscular Dystrophy
- Physiotherapy
- Glucocorticoids- steroid tx improve power, muscle mass, rate of muscle breakdown
WHat are the 4 Types of hyperkinetic movement disorders seen in children
Chorea
Myoclonus
Tics
Tremors
What is chorea, what are its causes and clinical features
Type of movement disorder
Clinical Presentations
-Figgety, twist or jerking in arms, legs or muscles of the face
-Grip changes (milkmaid’s grip)
-Tingue movements
-Headaches
-Seizures
-Slurred speech
(worsens when stressed, anxious, another medical condition or medication side effects)
CAuses:
1. Overactivity of Dopamine that affects basal ganglia (movement)
1. Sydenham Chorea
2. CP
3. Huntington’s
4. Endocrine: hyperthyroidism
5. SLE
6.Brain tumor in basal ganglia
7. Drug induced: Phenytoin, Lithium, Alcohol
Tx of chorea
What are the 3 commonly seen Hypokinetic disorders in children
Akinetic rigid syndrome
Juvenile Parkinsonism
Progressive pallidal degenerations
List some of the most common causes of strokes in children
- Congenital heart disease
- Acquired heart diseases (RHD, Kawasaki, cardiomyopathy, Myocarditis, prostatic valves)
- HPT
- Atherosclerosis
- DM
- Vasculitis: Meningitis, SLE
- Vasculopathy: Moyamoya syndrome, HIV vasculopathy
- aneurysms
- Trauma: carotid dissection, penetrating trauma
Which conditions are commonly linked to Moyamoya disease
Neurofibromatosis
TB
Sickle cell
HIV
Down Syndrome
Cranial irradiation
What is CP
Group of disorders that affect movement and posture.
Outline the causes of Cerebral Palsy
Prenatal
-Multiparous/Multigestation
-genetic actors
-Antenatal bleeding
-infections
-cerebral malformations
-strokes in mom ?
Perinatal
-Multiple pregnancy
-Premature
-IGR
-Small for GA
-Infections
-Birth trauma
-Perinatal strokes
-Cerebral hamerrhages
Postnatal
-Infections (meningitis)
-Head injury
-Metabolic
-hypoglycemia
-hypocalcemia
-hyponatremia
-vascular
-strokes
-thrombosis/ embolism
-toxins
Differences between paraplegia and diplegia
Paraplegia (paralysis of the legs) results form a spinal cord lesion and diplegia (symmetrical paralysis of either the arms or the legs) comes from a CNS nervous system lesion
What are the early warning signs of CP
Delayed milestones
Primitive reflexes or persistence of certain postures
Hypertonia or hypotonia
Clumsiness
Toe walking
Bulbar dysfunction, difficult speech, feeding and drooling
What are the commonest causes of spastic CP (Diplegia, Hemiplegia, Quadriplegia
IUGR
Low birth weight
Birth asphyxia
Differentiate between dystonia and rigidity
Dystonia is posture a rigid muscle is pulled into.
Rigidity is tone felt by examiner which is constant throughout range of movement
Associatetd problems with CP/ complications
Intellectual impairment
Language, vision and hearing abnormalities
Epilepsy
MAlnutrition
Behavioural problems
Common clinical features of meningitis
Fever
Rased ICP: vomitting, headache
Meningeal irritation: Neck stiffness, Kernigs and Brudzinski
encephalopathy and coma
In younger babies: poor feeding, irritable, bulging fontanele, vomitting and fever
CAuses of meningitis on
1. Neonates
2. Infants
3. Childhood and adolescents
Neonates
-Group B strep
-Ecoli
-Listeria
-Klebsiella
-Enterobacter
Infants
-Group B strep
-Neisseria meningitis
-strep pneumonia
-Haemophilus Influenza
-Salmonella species
Childhood
-Neisseria meningitis
-Strep pneumonia
-Haemophilus Influenza
Early sign of Neisseria meningitis
MEningoccoacal rash- Petechiae or purpuric rash taht can evolve very quickly into purpura fulminans
Complications of meningitis
Cerebral Oedema
Hydrochephalus
Brain abscess
Seizures
Cranial mnerver palsies
Hemiolegias
Vasculitis
Subdural effusions
SISADH
Management of Bacterial meningitis in infants less than 2 months
Initially:
Cefotaxime 50mg/kg 8-hourly (12 hourly if less than 1 week old)
Or
Amoicillin 50mg/kg 6-hourly (8hourly if less than 1 week old)
When organism is identified
Group B Strep: Cefotaxime or Ampicilin (alone or with aminoglycoside) for 14 days
Gram negative enteric bacilli: Cefotaxime plus aminoglycoside(1st 7 days) min 21 days
Listeria: Ampicillin alone for 14 days
Neisseria: ceftriaxone and ciprofloxacin IV? For 7days
Tx of bacterial meningitis in infants and children older than 2 months
Initial therapy
Ceftriaxone 100mg/kg then 80-100mg/kg once daily OR cefoxamine 75mg/kg 8-hourly
AND
Dexamethasone 0.6mg/kg/d 8hourly ideally 15-30min priopr to initiating antibiotics
Duration of therapy
H. Influenza 7-10 days
S. Pneumonia 10-14 days
N. Meningitidis- 7days
What prophylactic medication would you close contacts to Meningoccocal disease
Rifampicin
Ceftriaxone
What prophylactic meds would you give to haemophilus influenza type B contacts
Rifampicin 20mg/kg daily for 4 days in 5 years and younger
Complications of brain abscesses
Epilepsy
Focal neurological deficits
Hydrocephalus
Mental retardation
Organism responsible for Neurocysticosis
Taenia solium the pig tape worm
Tx with albendazole or Praziquziquintel
When should a child with spinal bifida be managed
refer for surgical correction within 48 hours of delivery!
Outline the classification of seizures
Focal seizures:
-Aware or Unaware
-Aura
-Motor
-Autonomic
Generalised
-Tonic-clonic
-Absence
-Clonic
-Tonic
-Myoclonic
what is a colic
frequent, prolonged and intense crying or fussiness in an infant, can be caused by spasm, obstruction or twisting of a gollow organ such as intestine, ureter or gall bladder
Tx of infantile spasms (epileptic)
ACTH or prednisone
Which medications do you give for Generalised Epilepsy vs
Focal Epilepsy
Generalised - Sodium valproate
Focal- Carbamezapine (careful in childbearing give Lamatrigine)
Approach to Status Epilepticus
- ABCD (O2, sats, pulse / BP, glucose)
- Total 2 doses benzo….
- Pr diazepam (0.5mg/kg) out of hospital
- IV access IV lorazepam (0.1mg/kg) preferred
Access failure – IO line / intranasal midazolam
(200mcg/kg)
* IV phenobarbitone (20mg/kg)
* Repeat PB (10mg/kg)
* Repeat PB (10mg/kg)
* Refer PICU ……..
Tx for migranes
Analgesia: Ibuprofen, Panado
Antiemetic
Migrane specific: Sumatripan (serotonin 5HT1 receptor agonist, affects vasoconstriction, oral or nasal spray tx)
Prophylaxis for migraine: Propranolol (NB in asthma, DM, CCF), Valproate (ideal in epileptics), Amitriptyline(depression), Topiramate (ideal in epileptics), Flunarizine
Outline the 3 brainstem syndromes
Weber syndrome = 3rd N + opp. hemi
(midbrain)
* Millard-Gubler syndr. = 6th /7th + opp. hemi
(pons)
* Jackson syndrome = 10th, 12th + opp. Hemi
(medulla)
differentials for hemiplegia
stroke
todds paralysis
ADEM
Mass lesion (neoplasisms eg)
Trauma
HSV encephalitis
COmplicated migranes
Metabolic eg MELAS
Risk factors for stroke in children
Sickle cell disease
Vasculitis eg Meningitis, SLE, Takayasu
Vasculopathies e.g Post Varicella, Moyamoya
Congenital heart disease eg Complex CHD
Acquired Heart Disease eg RHD, IE
Congenital HD eg ASD ?
Genetic: NF1, Homocystinuria
What is moyamoy and what are some of its risk factors
Cerebrovascular disorder, involves occlussion or stenosis or occlusion of terminal internal carotis (circle of willies?)
Risk factors
* Down’s Syndrome
* Neurofibromatosis
* Sickle Cell Disease
* Homocystinuria
* Radiotherapy (brain)
* Infections (?HIV), etc.
What is the anatomical position of the lesion that causes cerebella motor syndrome
Anterior lobe of the cerebellum
What is the location of the lesion that causes non-motor symptomatology of the cerebellum (emotion, mood, cognition)
Posterior lobe of the cerebellum
WHat is the drug management and prophylaxis of sydenham chorea
Penicillin
Why is Moyamoya important in SA/Africa
Moya moya is the endstage of vascular occlusive disease which can occur in the vasculitis associated with HIV and TB, as well as sickle cell disease, trisomy 21, and neurofibromatosis
