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 (ISBN-13: 9780511276217)

The Pseudotumor Cerebri Syndrome
Cambridge University Press
978-0-521-86919-5 - The Pseudotumor Cerebri Syndrome: Pseudotumor Cerebri, Idiopathic Intracranial Hypertension, Benign Intracranial Hypertension and Related Conditions - by Ian Johnston, Brian Owler and John Pickard
Index

Index



Aarskog's syndrome 100

acetazolamide (Diamox) 4, 27, 196–8, 229

historical background in PTCS 17

inhibition of CSF secretion 270–1

Addison's disease 23, 110

adrenal disease 110

aetiology of PTCS 23, 25, 82–5, 277–8

individual factors

cranial venous outflow tract compromise 99–104

endocrine disorders 108–12

familial factors 98–9

female-specific factors 93–8

haematological disorders 104–7

head injuries 113–14

infections 112–13

nutritional disorders 119–20

other diseases and conditions 115–18

vitamins, drugs and chemicals 120–4

overview 85

case control and related studies 89–91

Glasgow and Sydney series 85–8

large series (1954–1988) 88–9

recent developments 91–2

all-trans-retinoic acid (ATRA) 92, 106, 122

amenorrhoea, link with PTCS 9

amiloride 272

amiodarone 124

anaemia 104, 105

angiography 32–4, 166–7

digital retrograde venous angiography (DRVA) 175

MR studies (MRA) 173

anti-coagulant therapy, systemic 223–4

antiphospholipid antibodies 185

apnoea, during sleep 139

arachnoid villi 2

Arnold–Chiari malformation 218, 221, 222

arsenic trioxide 106

average combined venous conduit score (ACCS) 173

balloon angioplasty 225

Behçet's disease 102, 115–16, 223, 228

benign intracranial hypertension (BIH) 2, 20, 24, 62, 68

betamethasone 201, 267

blood dyscrasias 102

bone marrow transplantation (BMT) 106

brain imaging studies 31–2

brain parenchyma 48–50

brucella meningitis 66, 113

bumetanide 272

carbonic anhydrase 272

cardiac diseases that may lead to PTCS 116–17

cardiac failure 19

cardiac glycosides, inhibition of CSF secretion 271–2

cases histories of PTCS

group I 78

group II 78

group III 79

group IV 80

ceftriaxone 113

cerebral blood flow (CBF) 34–5

effect of increased intracranial pressure on 253–4

investigations for PTCS 182–3

cerebral blood volume (CBV) 34–5, 50–2, 57

effect of increased intracranial pressure on 253–4

investigations for PTCS 182–3

cerebral metabolism 253–4

cerebral oedema 56

effects of steroids 270

cerebral pseudotumor 15

cerebrospinal fluid (CSF)

absorption 248

experimental studies 267

composition

abnormalities 43–4, 65

investigations for PTCS 158–9

distribution

MR studies 170–2

effect of steroids 267

laboratory study 267–9

failure of absorption 18

formation 247

increased volume

nomenclature and terminology 61–2

infusion studies 37–8, 159–61

involvement in PTCS 52–3

associated conditions 53

clinical measurements 52

direct observations 52

theoretical considerations 53

therapeutic considerations 53

pressure 4, 248, 249

continuous monitoring for PTCS 151–8

effect of pressure gradients on cranial and spinal compartments 254

effect on CBF, CBV and cerebral metabolism 253–4

effect on cranial venous outflow pressure 250–3

investigations for PTCS 149–50

persistent elevation 240–1

Pouisouille equation 249

rhinorrhoea 139

secretion inhibitors 270

acetazolamide 270–1

cardiac glycosides 271–2

frusemide 272

shunting 4, 28, 214, 217, 230

complications 218–19

Glasgow series 214

removal 220–2

revisions 217–18

shunted cases 214–16

Sydney series 214

types of shunt 216–17

Chiari malformation 222

children, clinical features of PTCS 145–16

chlordexone 240

chlorothiazides 27

chlorthalidone 199

chlorthiazide 199

cholesteatoma 102

chronic intracranial hypertension 44

chronic meningitis 44

chronic respiratory disease 19

ciprofloxacin 124

cisternal CSF shunts 216

classification of PTCS

1 – primary pseudotumor cerebri syndrome (Primary PTCS) 70

no recognised cause 71

recognised precipitating cause 71

2 – secondary pseudotumor cerebri syndrome (Secondary PTCS) 70

abnormal CSF composition 65

extracranial venous outflow impairment 73–4

intracranial venous outflow impairment 72–3

3 – atypical pseudotumor cerebri syndrome (Atypical PTCS) 70

infantile PTCS 75–6

normal CSF pressure 70

symptoms/signs/both absent 74

4 – pseudo-pseudotumor cerebri syndrome (Pseudo PTCS) 70

normal volume hydrocephalus 71–2

occult mass lesion 76–7

claudins 270

clinical features of PTCS 1, 26, 127, 278–9

aspects of diagnosis 146–7

atypical presentations 137–9

asymptomatic PTCS 137

CSF rhinorrhoea 139

headache without eye signs 138

other presentations 139

sleep apnoea 139

children 145–6

duration of symptoms and signs 232–4

incidence, age and sex distribution 127–31

males 145

presenting clinical signs 139–45

extraocular movement abnormalites 142

optic atrophy 142

other signs 144

papilloedema 141

reduced visual acuity 142

restriction of visual fields 143

presenting symptoms 131–9

diplopia 133

headache 132

nausea and vomiting 134

obesity and menstrual irregularity 135

other symptoms 134

tinnitus 134

visual disturbances 133

clinical investigations seeinvestigations

communicating hydrocephalus (CH) 45

computed tomography (CT) scanning 168–9

concussion 12

confocal scanning laser ophthalmoscopy (CSLO) 163

corticosteroids 23

cranial venous outflow

effect of CSF pressure 250–3

impairment 5

investigations for PTCS 175–80

obstruction 40–1

experimental studies 254–5

treatment 222–3, 230

tract compromise 99–104

cryptococcal meningitis 66, 113

Cushing's disease 111

cyclosporin A 106

cytarabine hydrochloride 106

danazol 90, 124, 240

Dandy criteria 26, 60, 63–4, 113

criterion 1 – raised ICP 64–5

criterion 2 – absence of focal neurological signs 65

criterion 3 – increased CSF pressure 65

criterion 4 – normal CSF composition 65–6

criterion 5 – normal imaging studies 66–7

criterion 6 – no identifiable cause 67

criterion 7 – benign clinical course 67

dexamethasone 201, 202, 267

diabetes mellitus 112

diagnosis of PTCS 146–7

see also Dandy criteria

diagnostic error 242–4

evidence from literature 243

Glasgow series 244

Sydney series 244

elimination of other conditions 62

Diamox see acetazolamide

digital retrograde venous angiography (DRVA) 175

digoxin 199, 228

diplopia 133

diuretics 24

as treatment for PTCS 198–9

dizziness 134

doxycycline 123

dural AVMs 102

electroencephalography (EEG) 164–5

emphysema 19, 116

encephalography 167–8

encephalopathy addisonienne 24

endocrine disorders predisposing to PTCS 108–12

endocrine disturbances 41–2

endovascular techniques for PTCS 225–8

enzyme deficiencies that may lead to PTCS 117–18

epidemiology 3

epidemiology of PTCS 25

incidence, age and sex distribution 127–31

états hypertensifs 14

états méningés hypertensifs 61

ethinyl oestradiol 90

evidence from pathology 38–9

experimental studies 246

conclusions 273–4

cranial venous outflow obstruction 254–5

extracranial obstruction 255–9

intracranial obstruction 259–63

inhibitors of CSF secretion 270

steroids 267–70

theoretical considerations 247–54

CSF absorption 248

CSF formation 247

CSF pressure 248–53

vitamin A 263–6

calves as subjects 265–6

rabbits and chicks as subjects 264–5

extracranial venous outflow impairment 73–4

extraocular movement abnormalites 142

eyes and vision, signs and symptoms involving

see also papilloedema

diplopia 133

extraocular movement abnormalites 142

ophthalmological investigations 161

optic atrophy 142

optic nerve disorders 10

optic neuritis 9, 10

orbits

MR studies of contents 172–3

papilloedema 161–3

reduced visual acuity 142

restriction of visual fields 143

visual disturbances 133

visual field assessment 163–4

factor V Leiden 185

familial cases 44

familial factors predisposing to PTCS 98–9

female-specific factors

menarche 94

menstrual irregularity 94

oestrogens, exogenous 95–6, 102

polycystic ovary syndrome (PCOS) 96–8

predisposing to PTCS 93–4

pregnancy 94–5

fluorescein angiography 162, 163

Glasgow series 162

frusemide 198, 199, 272

furosemide 27

Glasgow series 93

age range of PTCS sufferers 129

angiography 166

CSF composition 158

CSF pressure, continuous monitoring 151

CSF shunting 214

diagnostic error 244

duration of symptoms and signs of PTCS 232

fluorescein angiography 162

incidence of PTCS 128

lumbar puncture 150

outcomes 242

recurrence of PTCS 238

subtemporal decompression 205

ventriculography/encephalography 167

glucocorticoids 24

glycerol 198, 199

growth hormone (rhGH) 92

replacement therapy 109, 240

Guillain–Barré syndrome 19, 44, 57, 65, 66, 113

haematological and related abnormalities 43

haematological investigations for PTCS 183–5

head injuries leading to PTCS 113–14

headache 132, 138

historial background of PTCS 6–9, 275

Period 1 – first descriptions (1866–1896) 9–11

Period 2 – definition of a syndrome (1897–1904) 11–13

Period 3 – pre-neuroradiological (1904–1936) 13–17

Period 4 – radiology and new treatments (1937–1970) 17–24

Period 5 – modern period (1971–2005) 24–9

hydrocephalus, communicating see communicating hydrocephalus

hydrocephalus, ‘external’ or ‘toxic’ 18

hydrocephalus, normal volume see normal volume hydrocephalus

hydrocephalus, otitic see otitic hydrocephalus

hydrocephalus, rhachitical 10

hydroflumethazide 199

hydropisie méningée 14

hyper- and hypo-vitaminosis A see undervitamin A (retinol)

hyperparathyroidism 19

hypertension, benign intracranial see benign intracranial hypertension

hypertension, idiopathic intracranial see idiopathic intracranial hypertension

hypertension, intracranial see intracranial hypertension

hypertension, intracranial 57

hypertensive meningeal hydrops 2, 18, 22, 61

hypoparathyroidism 111

hypothyroidism 109

idiopathic intracranial hypertension (IIH) 2, 3, 26, 61, 62, 68

idiopathic macrocephaly 55

indomethacin 228

infections that may lead to PTCS 112–13

intracranial hypertension 1

intracranial hypertension, benign see benign intracranial hypertension

intracranial hypertension, idiopathic see idiopathic intracranial hypertension

intracranial tumours 12

intracranial hypertension 57

of unknown cause 22

intracranial venous outflow impairment 72–3

intracranial/CSF pressure

effects of steroids 269

monitoring 36–7

investigations for PTCS 27, 148–9, 187–8, 279

angiography 166–7

cerebral blood flow and volume studies 182–3

cranial venous outflow tract studies 175–80

CSF composition 158–9

CSF infusion studies 159–61

CSF pressure 149–50

continuous monitoring 151–8

CT scanning 168–9

electroencephalography (EEG) 164–5

haematological investigations 183–5

lumbar puncture 150–1

metabolic and endocrine studies 185–7

MR techniques 170

angiography (MRA) 173

brain water and CSF distribution 170–2

orbital contents 172–3

standard static MR 170

venography (MRV) 173–5

ophthalmological investigations 161

papilloedema 161–3

radionuclide studies 180–2

skull X-rays 166

ventriculography/encephalography 167–8

visual field assessment 163–4

leukaemia 105

leuprorelin 124

lithium carbonate 90, 124

lumbar percutaneous CSF shunts 216

lumbar puncture 4

historical background 7

investigation for PTCS 150–1

treatment for PTCS 192–5, 229

lumbar valved CSF shunts 216

Lyme disease 113

magnetic resonance (MR) techniques 170

angiography (MRA) 173

brain water and CSF distribution 170–2

orbital contents 172–3

standard static MR 170

venography (MRV) 173–5

males, clinical features of PTCS 145

Marchiafava–Micheli syndrome 107

mastoiditis 101, 112

mechanisms leading to PTCS 1, 2, 25, 30–1, 57–9, 275–6

clinical evidence

angiography and venography 32–4

brain imaging studies 31–2

cerebral blood flow and volume studies 34–5

CSF infusion studies 37–8

evidence from pathology 38–9

intracranial/CSF pressure monitoring 36–7

radionuclide studies 35–6

intracranial compartments 47–8

brain parenchyma 48–50

cerebral blood volume 50–2

CSF 52–3

possible related conditions

communicating hydrocephalus 45

infantile macrocephaly 46–7

normal volume hydrocephalus 45–6

putative causal factors

cranial venous outflow obstruction 40–1

CSF composition abnormalities 43–4

endocrine disturbances 41–2

familial cases 44

haematological and related abnormalities 43

obesity 42–3

steroid administration and withdrawal 40

vitamin A excess and deficiency 39–40

theoretical considerations 54–7

men, clinical features of PTCS 145

menarche 94

meningioma 102

méningites vrais 14

meningitis, brucella 66

meningitis, cryptococcal 66

meningitis, syphilitic 66

meningitis serosa 61

menstrual irregularity 94, 135

mesalazine 124, 240

methylprednisolone 201

middle ear disease 101, 112

historical background of PTCS 13, 15, 16

minocycline 90, 123

myeloma 106

nalidixic acid 90, 124, 240

nausea 134

neck stiffness 134

nitrofurantoin 124

nitroglycerin 124

nomenclature of PTCS and related conditions 1, 26, 61, 276–7

alternative terms 67–9

diagnostic elimination 62

eponymous terms 63

increased CSF volume 61–2

specific terms 62–3

Nonne's disease 13

nosology of PTCS 60–1

nutritional disorders that may lead to PTCS 119–20

obesity 42–3, 135

weight reduction 199–201

occludin 270

ocreotide 228

oestrogens, exogenous 95–6, 102

‘old theories’ of PTCS 24

‘old therapies’ for PTCS 24

omeprazole 272

ophthalmological investigations 161

papilloedema 161–3

visual field assessment 163–4

ophthalmoscope 6, 11

optic atrophy 142

optic nerve disorders 10

optic nerve sheath decompression (ONSD) 4, 28, 208–14, 229

historical background 10

optic neuritis 9, 10

orbitotomy 209

otitic hydrocephalus 2, 13, 16, 20, 21, 61

otitis media 12, 15–17

ouabain 271

outcomes 4, 232, 244–5, 281–2

CSF shunting 217

diagnostic error 242–4

duration of symptoms and signs 232–4

Glasgow series 242

persistent elevation of CSF pressure 240–1

psychological and psychiatric sequelae 241–2

recurrence 238–40

Sydney series 243

visual function 235–8

overall summary 235–6

papilloedema 236–8

PaCO2 50

papilloedema 19, 141, 236–8

historical background in PTCS 9, 10, 15, 16, 18

investigations for PTCS 161–3

penicillin 124

perhexilene maleate 124

phenytoin 240

phospholipase A-2 270

Pickwickian syndrome 116

pituitary tumours 108

plasma homocysteine 185

platelet disorders 104, 106

POEMS 106

poliomyelitis 44, 57, 113

polycystic ovary syndrome (PCOS) 95–8

polycythaemia vera 19, 104, 106

Pouisouille equation 249

prednisolone 201, 202, 267

prednisone 201, 267

pregnancy 94–5

propranolol 272

prothrombin index 72

pseudo-brain abscess 15, 62

pseudo-meningitis 9, 62

pseudotumor cerebri (PTC) 61, 62

pseudotumor cerebri syndrome (PTCS)

definition 69–70

nomenclature 61, 68, 80–1

psychiatric disorders that may lead to PTCS 117

purpura 9, 16

radionuclide studies for PTCS 35–6, 180–2

recurrence of PTCS 238–40

Glasgow series 238

Sydney series 238

reduced CSF absorption syndrome 61

renal disease that may lead to PTCS 116

respiratory diseases that may lead to PTCS 116–17

retinoic acid, all-trans (ATRA) 92, 106, 122

retinoids 90

retinol see vitamin A

retinol-binding protein (RBP) 264

rhachitical hydrocephalus 10

rhinorrhoea 139

sequelae of PTCS 5

serous meningitis 2, 11, 13

shunt types

cisternal 216

lumbar percutaneous 216

lumbar valved 216

ventricular 217

signs and symptoms 1, 26

see also clinical features

aspects of diagnosis 146–7

atypical presentations 137–9

asymptomatic PTCS 137

CSF rhinorrhoea 139

headache without eye signs 138

other presentations 139

sleep apnoea 139

children 145–6

Dandy criteria 63

criterion 1 – raised ICP 64–5

criterion 2 – absence of focal neurological signs 65

criterion 3 – increased CSF pressure 65

criterion 4 – normal CSF composition 65–6

criterion 5 – normal imaging studies 66–7

criterion 6 – no identifiable cause 67

criterion 7 – benign clinical course 67

duration 232–4

males 145

presenting clinical signs 139–45

extraocular movement abnormalites 142

optic atrophy 142

other signs 144

papilloedema 141

reduced visual acuity 142

restriction of visual fields 143

presenting symptoms 131–9

diplopia 133

headache 132

nausea and vomiting 134

obesity and menstrual irregularity 135

other symptoms 134

tinnitus 134

visual disturbances 133

sinography 19

skull X-rays 166

sleep disorders that may lead to PTCS 117

sleep apnoea 139

slit-ventricle syndrome 46, 57

spinal cord tumour 44, 57

steroids 23, 27, 90, 122–3, 240

administration and withdrawal 40

experimental studies 267–70

cerebral oedema 270

CSF dynamics 267–9

effects on intracranial pressure 269

treatment for PTCS 201–5, 229

streptokinase 225

subtemporal decompression (STD) 4, 28, 205–8, 230

Glasgow series 205

Sydney series 206

superior sagittal sinus (SSS) pressure 54, 72

experimental occlusion 260

surgical treatments for PTCS 224

Sydney series 93

age range of PTCS sufferers 129

angiography 166

CSF composition 158

CSF pressure, continuous monitoring 155

CSF shunting 214

diagnostic error 244

duration of symptoms and signs of PTCS 232

lumbar puncture 150

outcomes 243

recurrence of PTCS 238

subtemporal decompression 206

ventriculography/encephalography 167

syphilitic meningitis 66, 113

syringomyelia 218, 221, 222

systemic lupus erythematosus (SLE) 102, 115

tetracycline 90, 123–4, 240

thyroid disease 109

thyroid replacement medications 90

tinnitus 134

topiramate 228

torcular epidermoids 102

toxic external hydrocephalus 61

treatments for PTCS 2, 4, 27, 189–90, 228–31, 279–81

acetazolamide 4, 27, 196–8, 229

historical background 17

cranial venous outflow obstruction 222–3, 230

CSF shunting 4, 28, 214, 217, 230

complications 218–19

shunt removal 220–2

shunt revisions 217–18

shunted cases 214–16

types of shunt 216–17

diuretics 198–9

effect of no treatment 191–2, 229

endovascular techniques 225–8

lumbar puncture 4

historical background 7

optic nerve sheath decompression (ONSD) 4, 28, 208–14, 229

historical background 10

other treatments 228

serial lumbar punctures 192–5, 229

steroids 27, 201–5, 229

subtemporal decompression (STD) 4, 28, 205–8, 230

surgery 224

systemic anti-coagulant therapy 223–4

venous by-pass techniques 224–5

weight reduction 199–201

Turner's syndrome 111

urea 199

urokinase 225

vein of Galen 100

venography 32–4

MR studies (MRV) 173–5

venous by-pass for PTCS 224–5

venous obstruction 51

ventricular CSF shunts 217

ventriculography 167–8

visual acuity, reduced 142

visual disturbances 133

visual field assessment 163–4

visual field, restriction of 143

vitamin A (retinol) 23, 90, 120–2, 240

excess and deficiency 39–40

experimental studies 263–6

calves as subjects 265–6

rabbits and chicks as subjects 264–5

hypervitaminosis 5, 120, 264

hypovitaminosis 5, 120, 122, 264

vitamin D (calciferol) 119

vomiting 134

weight reduction as treatment for PTCS 199–201

X-ray investigations of skull 166


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