Chapter
Period 5: The modern period (1971-2005). New techniques, old theories, and old therapies
Evidence from clinical investigative studies
Angiography and venography
Cerebral blood flow and volume studies
Intracranial and CSF pressure monitoring
Pathology: macroscopic and microscopic
Evidence from putative causal factors
Vitamin A excess and deficiency
Steroid administration and withdrawal
Cranial venous outflow obstruction
Haematological and related abnormalities
Abnormalities of CSF composition
Evidence from possibly related conditions
Communicating hydrocephalus
Normal volume hydrocephalus
Which intracranial compartment is involved?
Theoretical considerations
Therapeutic considerations
Theoretical considerations
4 Nosology, nomenclature, and classification
Criterion #1: Signs and symptoms of raised ICP
Criterion #2: Absence of focal neurological signs
Criterion #3: Measured increase in CSF pressure
Criterion #4: Normal CSF composition
Criterion #5: Normal imaging studies
Criterion #6: No identifiable cause
Criterion #7: Benign clinical course (other than ophthalmological)
Nomenclature: The alternatives
Definition of the pseudotumor cerebri syndrome (PTCS)
Group I: Primary PTCS, no recognized cause
Group I: Primary PTCS, recognized precipitating cause
Group II: Secondary PTCS, abnormal CSF composition
Group II: Secondary PTCS, intracranial venous outflow impairment
Group II: Secondary PTCS, extracranial venous outflow impairment
Group III: Atypical PTCS, symptoms or signs or both absent
Group III: Atypical PTCS, normal CSF pressure
Group III: Atypical PTCS, infantile PTCS
Group IV: Pseudo-PTCS, occult mass lesion
Group IV: Pseudo-PTCS, 'normal volume' hydrocephalus
An overview of aetiology in PTCS
The Glasgow and Sydney series (Tables 5.2 and 5.3)
Large series from 1954 to 1988
Case-control and related studies
Recent developments in the aetiology of PTCS
Individual factors in the aetiology of PTCS
Polycystic ovary syndrome (PCOS)
Cranial venous outflow tract compromise
Other diseases and conditions
Systemic lupus erythematosus
Cardiac and respiratory diseases
Vitamins, drugs, and chemicals
Vitamin A and related compounds
Tetracycline and related compounds
Incidence, age, and sex distribution
Obesity and menstrual irregularity
Headache without eye signs
Presenting clinical signs
Papilloedema and related findings
Extraocular movement abnormalities
Restriction of visual fields
7 Clinical investigations
Cerebrospinal fluid pressure
Continuous CSF pressure monitoring
Cerebrospinal fluid composition
Cerebrospinal fluid infusion studies
Ophthalmological investigations
Ventriculography and encephalography
Computed tomography scanning
Magnetic resonance techniques
Standard static magnetic resonance
Magnetic resonance studies of brain water and CSF distribution
Magnetic resonance of orbital contents
Magnetic resonance angiography
Magnetic resonance venography
Cranial venous outflow tract studies
Cerebral blood flow and volume studies
Haematological investigations
Metabolic and endocrine studies
Subtemporal decompression
Optic nerve sheath decompression
Cerebrospinal fluid shunting
Treatment of cranial venous outflow obstruction
Systemic anti-coagulant therapy
Direct surgical treatment
Venous by-pass techniques
Optic nerve sheath decompression
Subtemporal decompression
Cerebrospinal fluid shunting
Approaches to cranial venous outflow tract obstruction
Duration of symptoms and signs
Outcome for visual function
Persistent elevation of CSF pressure
Psychological and psychiatric sequelae
Other diseases and diagnostic error
Theoretical considerations
What determines CSF formation and how is it affected by alterations in CSF pressure?
What determines CSF absorption and how is it affected by increased CSF pressure?
What effect does raised CSF pressure per se have on cranial venous outflow tract pressure?
How are CBF, CBV and cerebral metabolism affected by increased ICP?
Does an increase in CSF pressure create pressure gradients within the cranial and spinal compartments and what effects might these have?
Cranial venous outflow obstruction
Extra-cranial obstruction
Intra-cranial obstruction
Steroids and CSF dynamics
Measurement of CSF absorption
Resistance to CSF absorption
Intracranial pressure and other parameters
Steroids and cerebral oedema
Inhibitors of CSF secretion
The Pseudotumor Cerebri Syndrome
：Pseudotumor Cerebri, Idiopathic Intracranial Hypertension, Benign Intracranial Hypertension and Related Conditions
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