Neurology - TLOC/Ataxia/Brian Tumours/Devlopmental Regression/Other Flashcards
What are the potential causes of funny turns?
- breath holding attacks - toddler holds breath when crying but rapidly recovers
- reflex anoxic seizures - occurs when infant are in pain and they become hypoxic which may stimulate tonic clinic seizure
- syncope
- migraine
- benign paroxysmal vertigo
- cardiac causes - cardiomyopathy or prolonged QT
- pseudoseizure
- NAI
- paroxysmal movement disorder
What is ataxia?
incoordination of movement, speech and posture due to either cerebellar or posterior pathway problems
What are the common causes of ataxia?
Causes of cerebellar ataxia
- acute causes - meds, drugs, alcohol, solvents, trauma
- post viral - seen with varicella infection
- posterior fossa lesions - CPA syndrome
- genetic and degenerative disorders
Genetic and degenerative disorders:
- ataxic CP
- friedreich’s ataxia
- ataxia telangiectasia
What are the investigations for ataxia?
genetic testing
LP
brain scans to check for tumours and damage
What are the presenting features of brain tumours?
HEADSMART
- persistent/recurrent vomiting
- persistent/recurrent headache
- balance/co-ordination/walking problems
- abnormal eye movements or suspected loss of vision
- double vision
- behaviour change, particularly lethargy
- fits/seizures
- abnormal head position
- increasing head circumference
- delayed or arrested puberty
What is the usual history of breath holding?
brief periods where the young child stops breathing
cause child to pass out
usually occurs when child is angry, in pain or afraid
occurs in children most commonly between 1-3 years
should not cause serious damage
eventually resolve
What are the common causes of developmental regression?
battens disease retts syndrome leukodystrophies wilsons disease SSPE (subacute sclerosis panencephalitis)
What is obstructive hydrocephalus?
obstruction may be within ventricular system or aqueduct
What is communicating hydrocephalus?
the obstruction may be at the arachnoid villi, the site of absorption for CSF (can also be due to CSF overproduction or venous drainage insufficiency)
What is external hydrocephalus?
this is a benign condition with a self limiting absorption deficiency of infancy and early childhood that leads to increased ICP.
What is macrocephaly?
head circumference above the 98th centile
What are the common causes of macrocephaly?
tall stature familial macrocephaly raised ICP hydrocephalus chronic subdural haematoma cerebral tumour neurofibromatosis cerebral gigantism (sotos syndrome) CNS storage disorders (hurler syndrome)
What are the common causes of microcephaly (<2nd centile)?
- familial - when present at birth and development normal
- autosomal recessive condition - when associated with developmental delay
- caused by congenital infection
- acquired after an insult to the developing brain e.g. perinatal hypoxia, hypoglycaemia
How are the different types of headache divided?
primary - migraines, tension-type headache, cluster headache
secondary - symptomatic of underlying pathology
trigeminal and other cranial neuralgias - including nerve root pain
What are the headache red flag symptoms for a SOL?
headache worse on lying down/coughing wakes up child confusion morning/persistent N&V personality, behavioural or educational change
What are the headache red flag signs for a SOL?
growth failure visual field defects - crainopharyngioma squint cranial nerve abnormality torticollis abnormal coordination - cerebellar gait change papilledema in fundus bradycardia cranial bruits - AVM
What are the treatment options for raised ICP?
Tumour – removal surgically or radio/chemotherapy
Subdural haematoma – surgical drainage Hydrocephalus – ventriculoperitoneal shunt
Idiopathic - specific diuretics can be prescribed by the neurologist.
Hypoventilation of the patient can temporarily
decreased ICP by decreased CO2 and causing vasoconstriction
Craniotomy may be performed if other procedures have not worked
What are the causes of subdural haematoma?
characteristic of NAI caused by shaking or direct trauma to infants or toddlers
occasionally seen after a fall from height
What are the symptoms of subdural haematoma?
altered mental state seizures apnoea breathing difficulties headaches lethargy sudden cardiac arrest
What is craniosynostosis?
premature fusion of one or more sutures
What are the features of myotonic dystrophy?
poor feeding
failure to meet milestones and hypotonia
progressive distal muscular weakness, ptosis, weakness and thinning of the face and SCM along with a carp mouth
What are the features of Duchenne Muscular Dystrophy (DMD)?
waddling gait language delay slower than peers Gowers sign serum creatinine kinase is elevated life expectancy is late 20s - respiratory failure, cardiomyopathy
How is DMD treated?
Physio to prevent contractures Overnight CPAP Corticosteroids to preserve mobility and prevent scoliosis Night splints Passive stretching
What is a congenital muscular dystrophy?
heterogeneous group of disorders recessive inheritance present with weakness, hypotonia or contractures present at birth longer life expectancy than DMD
What are the features spinal muscular atrophy?
progressive weakness denervaion areflexia fasciculations wasting severe form - death from resp failure in 12 months
What is spinal muscular atrophy?
recessive degeneration of anterior horn cells
SMN gene
What is the most severe type of spinal muscular atophy?
spinal muscular atrophy type 1 (Werdnig-Hoffman disease)
How does Gullain-Barre syndrome present?
acute post-infectious polyneuropathy
presentation is typically 2-3 weeks after an URTI or gastroenteritis
ascending symmetrical weakness
areflexia
difficulty swallowing
How is Gullain-Barre managed?
full recovery in 95% of cases
management is supportive esp with respiration
What is Bell’s Palsy?
LMN - facial weakness
must rule out compressive lesion in cerebellopontine angle and sarcoidosis if bilateral weakness
How is Bell’s Palsy managed?
corticosteroids to reduce oedema
associated with HSV infection
usually full recovery
What is Charcot Marie Tooth disease?
distal muscle wasting and sensor loss with proximal progression over time
usually autosomal dominant but can occur without family history
What are the features of Charcot Marie tooth disease?
muscle weakness and wasting starting distally sensory loss is similar generalised tendon areflexia may be foot drop and difficulty walking spinal deformities in 50%
How is Charcot Marie tooth disease managed?
supportive management
no effective treatment
What is CIDP (chronic inflammatory demyelinating polyneuropathy)?
Acquired, immune mediated inflammatory disorder of the PNS
Thought to be a chronic version of Charcot Marie Tooth disease
What are the features of CIDP?
Relapsing symptoms Proximal and distal limbs are affected Weakness Tingling and numbness Deep tendon reflexes reduced and gait is abnormal
How to tension headaches present?
symmetrical
bilateral
gradual evening onset
How do migraines present?
unilateral relieved by sleep worse on exertion photophobia 90% in children without an aura 10% aura - most commonly visual disturbance
What is Becker Muscular Dystrophy?
onset 11 years
similar to duchenne but slower progression
life expectancy late 40s - normal
