Miscellaneous conditions Flashcards
? is an increase in the volume of CSF, causing the distension of cerebral ventricles (ventriculomegaly) due to insufficient passage of the CSF from its point of production in the ventricles into the systemic circulation 7
Hydrocephalus
What are the two types of hydrocephalus and explain each.
1) communicating (i.e. CSF can exit the ventricular system)
- with obstruction to CSF absorption
- without obstruction to CSF absorption including:
normal pressure hydrocephalus (NPH)
choroid plexus papillomas (part of the associated hydrocephalus is thought to be due to overproduction of CSF
2) non-communicating (i.e. CSF cannot exit the ventricular system, and thus there is by definition obstruction to CSF absorption)
numerous causes including :
foramen of Monro: colloid cyst
aqueduct of Sylvius: aqueduct stenosis, tectal glioma
fourth ventricle: posterior fossa tumour, or cerebellar infarct or cerebellar haemorrhage
? is narrowing of the cerebral aqueduct. This is the most common cause of congenital obstructive hydrocephalus, but can also be seen in adults as an acquired abnormality.
Aqueductal stenosis
the infant with enlarging head size, bulging fontanelles and gaping cranial sutures are seen. Setting sun phenomenon may also be present. In X-linked form (Bickers-Adams-Edwards syndrome), which is associated with profound intellectual disability, clinical assessment would reveal bilateral adducted thumbs.
classically characterised by the triad of gait apraxia/ataxia, urinary incontinence, and cognitive impairment, although not all patients with the condition have all three
Normal pressure hydrocephalus
What are the complications of CSF shunt?
infection
- ventriculitis
- meningitis
shunt malfunction
- disconnections/breaks (most
- common in the neck)
- migration
- leakage
shunt overdrainage
overshunting-associated myelopathy
slit ventricle syndrome
intracranial peri-shunt fluid collection with oedema 3
trapped ventricle: after lateral ventricular shunting
distal complications
peritoneal CSF pseudocyst
pleural effusion
rarely, the distal end can encircle the bowel and cause strangulation 4
WHat is the most common cause of Macrocephaly
Association?
Benign enlargement of the subarachnoid spaces in infancy (BESS or BESSI) also known as benign external hydrocephalus (BEH) It usually involves the frontal lobe subarachnoid spaces, and it is characterised clinically by macrocephaly or frontal bossing.
scaphocephaly
is a syndrome with signs and symptoms of increased intracranial pressure but where a causative mass or hydrocephalus is not identified.
most commonly affected demographic ?
Idiopathic intracranial hypertension (IIH), also known as pseudotumour cerebri.
middle-aged obese females
WHat conditions are associated with intracranial hypertension?
endocrine
adrenal insufficiency
Cushing disease
hypoparathyroidism
hypothyroidism
excessive thyroxine replacement in children
drugs
tetracyclines, such as doxycycline 2
growth hormone
hypervitaminosis A from dietary intake or other retinoids, such as all-trans retinoic acid, isotretinoin, or retinol
chronic renal failure
systemic lupus erythematosus (SLE)
MOST common association with intractable temporal lobe epilepsy
How is it treated?
Mesial temporal sclerosis, also commonly referred to as hippocampal sclerosis
(Findings include:
reduced hippocampal volume: hippocampal atrophy
increased T2 signal)
Temporal lobe epilepsy is initially managed medically with anti-epileptic agents. In patients who are refractory to medical management temporal lobectomy or selective amygdalohippocampectomy may be performed. Anterior temporal lobectomy is successful in 75-90% of patients with mesial temporal sclerosis.
defined as cerebrospinal fluid (CSF) pressure <6 cm H2O in patients with clinical presentation of postural headache, nausea, vomiting, neck pain, visual and hearing disturbances, and vertig. It most commonly results from a CSF leak somewhere along the neuraxis.
MOST common cause ?
Intracranial HYPOTENSION
it most commonly results from a CSF leak somewhere along the neuraxis.
Intracranial hypotension can broadly be divided into:
primary: usually referred to as spontaneous intracranial hypotension
secondary: iatrogenic (lumbar puncture or surgery), over-shunting due to diversion devices, or traumatic
How does intracranial hypotension present as ?
often presents as a postural headache which is relieved by lying in a recumbent position, usually within 15-30 minutes .
Features of intracranial hypotension? SEEPS
S: subdural fluid collections
E: enhancement of the pachymeninges
E: engorgement of the venous sinuses
P: pituitary hyperaemia
S: sagging brain
a condition where there is localised or diffuse inflammatory thickening of the dura. On imaging, it presents as a localised, multiple, or diffuse enhancing dural thickening commonly forming mass-like lesions.
Hypertrophic pachymeningitis
Causes of Hypertrophic pachymeningitis
- idiopathic
- infective
neurosyphilis
CNS tuberculosis: tuberculous pachymeningitis
CNS cryptococcosis
bacterial meningitis - inflammatory
IgG4-related hypertrophic pachymeningitis
granulomatosis with polyangiitis
neurosarcoidosis
polyarteritis nodosa
rheumatoid arthritis
relapsing polychondritis
Behçet disease - other
Rosai-Dorfman disease
haemodialysis
mucopolysaccharidoses
