Case Studies in Uncommon Headache Disorders Ambar Chakravarty
INDEX
Page numbers followed by b refer to box, f refer to figure, and t refer to table.
A
Acetazolamide 59
intravenous 59
oral 59
Achalasia 201
Acid-fast bacilli 183
Acquired immunodeficiency syndrome 164
Airplane headache, pathophysiology of 33
Alice in Wonderland’ syndrome 10, 81
Allodynia 47, 189
Amino acids 20
Amphetamine 149
Angle closure glaucoma 59, 213
Antiepileptic drug 69, 81, 84
Antiphospholipid syndrome 14
Antituberculous drug 187
Anxiety disorder
generalized 127
separation 127
social 127
Aphasia 73
Apoplexy, pituitary 146, 164
Areflexia 27
Arterial constrictions 202
Arterial disease, peripheral 181
Arterial dissection 146
transmural 148
Arteriovenous malformation 154
Arteritis, temporal 179, 181
Artery
anterior communicating 90
biopsy, temporal 177f, 180
lower limb 179
retinal 174
Arthralgia 67
Ataxias 9, 10, 13, 24, 28
episodic 28
Attacks
agitation during 14
duration of 106
fulfilling criteria 9
Aura 9, 15, 22, 3032, 53, 73t
acute-onset 32
attack 52
duration of 73
epileptic 72
gastrointestinal 73
persistent 15
prolonged 32
stage 4
symptoms 184
typical 31, 32, 81
Autonomic dysfunction 87, 90
Autonomic nervous system 2, 92, 114, 118, 132, 173
B
Back pain 67
Basal cistern arteries 199f
Basal ganglia 151
Basal lamina 38
Basal meningitis, tubercular 61
Basilar artery 201
Behçet's syndrome 164
Blindness 213
transient 24
Blood
biochemistry 61
brain barrier 67
pressure 130, 213
venereal disease research laboratory 193
Bomb-like explosion 196
Boyle's law 33
Brain
and neck, magnetic resonance angiography of 113
arteries, angiogram of 34
base of 198f
computed tomography scan of 155
flair sequence 37f
imaging 82
injury, traumatic 14
lesions, subclinical 56
magnetic resonance
angiography of 152
imaging of 86, 130, 152, 158
Brainstem 209
aura 13, 15, 32, 43
symptoms 41
Brisk jerks 35
Bulbar palsy 193
Bulbus dilatation 191
Burning tongue 208
Bursting headache following micturition 158
C
CADASIL coma 39
Calcitonin gene-related peptide 5, 6, 47, 65, 67, 94, 95, 98
Calcium channel blockers 150
Call-Fleming syndrome 149
Carbamazepine 204, 208
Cardiovascular homeostasis, maintenance of 67
Carlow's proposition 63
Carotid
artery
dissection 55
internal 61
disease 114
dissection 146
stroke population 56
plexus 115
thromboendarterectomy 114
Catecholamine-secreting tumor 149
Central nervous system 3, 14, 39, 66, 114, 139, 149, 150
benign angiopathy of 149
Central neuromodulation targets 95
Central trigeminovascular neurons, sensitization of 7
Cerebellar ataxia 27
permanent 24
Cerebellum 151, 209
Cerebral
arteriovenous malformation 148
artery
vasoconstriction 150
trunk of middle 199f
autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy 14
edema, brain causing 202
hyperexcitability 54f
infarction 164
malaria 164
vasoconstriction syndrome, reversible 39, 130, 146, 149, 158
vasospasm, transient segmental 158
venous thrombosis 90, 146, 164
vessels, magnetic resonance angiography of 130
Cerebrospinal fluid 12, 26, 61, 140, 148, 152, 155, 167, 169, 193
lymphocytosis 23, 24, 27, 175
pressure, low 172
Cerebrovascular accident 14
Cerebrovascular disease 181
atherothrombotic 181
hypertensive 181
Cervical
complex, trigeminal 5, 96
nerves stimulation, high 96
spine 35
spondylotic myelopathy 35
Chiari anomaly, genesis of 192
Chiari malformation 192
signs of 193
symptoms of 193
Child's speech development 26
Choreoathetosis 28
Chromosome 201
Ciliochoroidal effusion 59
Circle of Willis 201
Cisplatin 164
Clonazepam 208
Cluster headache 88, 92, 93, 102, 114, 124, 128, 138, 142, 171
attacks 139
chronic 98
diagnosis of 118
episodic 122
genesis of 115
hemiplegic 131
recurrence of 114
Cluster-tic syndrome 109
Cold-stimulus headache 145
Collagen vascular
disease 164
disorders 164
Computed tomography
angiogram 148
contrast-enhanced 113
scan, abdominal 135
Confusion, recurrent episodes of 12
Confusional state, acute 14
Conjunctival injection 88, 101103, 105, 106, 110, 126, 134
Connective tissue diseases 194
Consciousness 9, 73
Conversion disorder 14
Convulsion 159
benign infantile 20
Corneal haziness 213
Cortical spreading depression, characteristic of 23
Cough hemicrania 170
Cranial
autonomic
sign 108, 128
symptoms 87, 88, 103, 105107
nerve palsies 187, 194
neuralgias 171
neuropathy, diabetic 61
Craniotomy 194
C-reactive protein 178
Cushing's disease 14
Cyclic guanosine monophosphate 99
Cyclical brainstem activity, role of 3
Cyclophosphamide 149
Cyclosporin A 164
Cytarabine 164
Cytomegalovirus 186
Cytotoxic drugs 164
D
Deep brain stimulation 109
Dementia, history of 40
Depression
cortical spreading 4, 9, 18, 75
spreading cortical 10
Depressive disorder 127
Diabetes mellitus 53, 61
Digital subtraction angiography 176
Diplopia 9, 13, 24
Direct revascularization procedure 202
Divalproex 130
Dix-Hallpike test 45
Dizziness 41, 48
acute symptoms of 48
chronic subjective 48
Dolichoectatic basilar artery 209f, 210f
Down's syndrome 202
Dravet syndrome 19, 29
Dural arteriovenous fistula 148
Dural tears 194
Dysarthria 9, 13, 24
Dysautonomia 193
Dyskinesia, paroxysmal kinesigenic 20
Dyslipidemia 53
Dysphagia 193
Dysphasia 184, 185
fibromuscular 55
Dystonia 28
Dystonic episodes 28
E
Eclampsia 160f, 161, 162, 164
Electrocardiography 22, 92, 113
Electroencephalography 12, 14, 16, 22, 69, 80, 81, 83, 84, 86, 185
Encephalitis 13, 14, 40
Encephalomalacia 86
Encephalomyelitis, postinfective 164
Encephalopathy
hepatic 14, 164
hypertensive 164
metabolic 14, 164
syndrome, posterior reversible 157, 161
uremic 14
Endocrine disturbances 14
Endothelial cells 53
Endothelial dysfunction 53
Endothelial injury 54
Endothelium 38
Epicrania fugax 205, 206f
Epilepsy 13, 20, 24, 27, 69, 70, 73, 73t, 74, 77, 78
benign familial infantile 20
childhood 74
classification of 71
focal migratory 18
migraine interface 83
syndrome 71
Epileptic encephalopathies, infantile-onset 28
Eptinezumab 67
Erythrocyte sedimentation rate 172
Erythropoietin 164
Eye, ipsilateral 109
Eyelid edema 102, 110, 126
F
Facial
nerve fibers, intrapontine 211
nucleus hypothesis 211
pain 193
persistent idiopathic 206
sweating 102, 110, 126
twitching 208
Fatigue 67
Fever, recurrent 182
Fluctuating motor
signs 28
symptoms 28
Fluid-attenuated inversion recovery 38, 156
Focal neurological signs 184
Foramen magnum margin 191
Forehead sweating 102, 110, 126
Four seizure, sign of 84, 85
Fremanezumab 67
Functional magnetic resonance imaging 2, 112, 139
Fusiform dolichoectatic basilar artery 211
G
Gait, ataxia of 10
Galcanezumab 67
Gamma-aminobutyric acid 76
Ganglion
cell, retinal 120
trigeminal 67, 95
Gastrointestinal tract 68
Genes, mutations of 25
Giant cell 180
arteritis 172, 178, 179
Glasses syndrome, hands full of 175
Glaucoma 59
chronic congestive 214
types of 59
Glia 53
Glossodynia 211
Glucose transporter defects 28
Glutamate
neurotransmission 2
transporter disorders 28
Gout 12
Granular osmiophilic material, accumulation of 38
H
HANDL syndrome 175, 187
Hazy cornea 213
Head
impulse test 45
trauma 50, 194
Headache 5, 1214, 26, 33, 45, 50, 55, 69, 70, 70b, 73, 74, 81, 86, 87, 90, 100, 116, 121123, 125127, 138, 143, 145, 152154, 159, 161, 168, 171173, 178, 190, 204, 215
airplane 33
associated with sexual activity 145, 153, 154
attacks, recurrent 166
attributed to airplane travel 33
bath-related 156
disorders 87, 144, 171
epilepsy interface 84
episodes, migraine-like 184
exacerbation of 119
external-pressure 145
fulfilling criteria 138
hemicranial 167
holocranial pulsatile 82
hypnic 145, 165
ictal epileptic 69, 71
intractable 177, 193
left hemicranial 214
moderate to severe 185
nummular 145
persistent 145
postdural puncture 154
post-endarterectomy 114
postictal 70
primary 171
cough 145, 168
exercise 145
stabbing 145
psychogenic 127
recurrent 8, 198
resolves immediately after seizure 70
secondary 90, 171
cough 168
sentinel 148
severe 130, 213
side locked 125
side shifting 137
spell of right hemicranial 85f
syndrome of transient 23
tension-type 70, 71, 171
throbbing 182
types of 59
typical 63
vascular 190
very severe squeezing-type 116
worst 147
Hearing loss, sensorineural 27
Hematoma, retroclival 146
Hemicrania
chronic paroxysmal 99, 111, 142
continua 88, 108, 123, 125, 126, 134, 142, 170
epileptica 70
episodic paroxysmal 110
paroxysmal 88, 92, 110, 134
Hemifacial spasm 208
Hemiparesis 24, 184, 185
Hemiparesthesia 184, 185
Hemiplegia 27, 130
Hemiplegic episodes 28
Hemorrhage 13, 202
intracerebral 164
intracranial 146, 150
nonaneurysmal perimesencephalic 148
subarachnoid 76, 146, 147, 150, 153, 155
Herpes simplex virus 187
Hydrocephalus 167
Hypercalcemia 14
Hyperexcitability, neuronal 1, 20
Hyperglycemia 14
Hyperintensities
bilateral symmetrical 160f
occipital 161f
Hypernatremia 14
Hypertension 53, 146, 164
Hyperthyroidism 14
Hypoacusis 9, 13
Hypocalcemia 14
Hypoglycemia 14
Hyponatremia 14, 164
Hypoperfusion 54
Hypophysitis, lymphocytic 175
Hypotension 167
spontaneous intracranial 146, 154, 194
Hypothalamus 2, 96
Hypothyroidism 14
Hypoxia 14
I
Immunoglobulin, intravenous 149, 164
Infection, intracranial 146
Inflammatory bowel disease 68
Intermittent porphyria, acute 164
International Classification of Headache Disorders 7, 26, 46, 70, 82, 83, 85, 92, 102, 105t, 131, 134, 137, 146, 153, 155, 165
International Headache Society 1, 24, 122
International League Against Epilepsy 71, 80
Intoxication 13
Intracranial hypertension, idiopathic 90
Intractable trigeminal autonomic cephalalgias 94
Intraocular pressure 119
Ischemia 13
Ischemic attacks, transient 13, 38, 43, 201
J
Jaw claudication 179
Joint swelling 179
L
Lacrimal gland 93
Lacrimation 103
Leukoaraiosis 39, 60
Leukoencephalopathy 24, 37, 39, 55
posterior 164b
syndrome, reversible posterior 146, 150, 151, 161
Lhermitte's sign 193
Limb
paralysis 193
weakness, transient 21
Linier burning sensation on face 204
Long-lasting autonomic symptoms with associated hemicrania 118, 142
Lumbar puncture 148, 175, 194
Lymph nodes
paratracheal 186f
subcarinal 186f
Lymphadenopathy, necrotic mediastinal 186f
Lymphocytes 180, 183
M
Magnetic resonance 158
angiography 90, 121, 151
cerebral angiography 26
imaging 8, 80, 82, 84, 90
contrast-enhanced 136, 165
Marfan's syndrome 55
Mean flow velocity 150
Medical myelopathies, types of 35
Medullary syndrome, lateral 90
Melatonin 100
Ménière's disease 4244
Ménière's syndrome 118
Meningioma, trigeminal 90
Meningitis
bacterial 164
carcinomatous 175
Meningoencephalitis 14
Mental retardation 24
Merkel's ganglion 97
Methylenetetrahydrofolate reductase gene 55
Methylprednisolone, high-dose 208
Microvascular disease, age-related 39
Migraine 1, 9, 15, 22, 3032, 37, 41, 43, 52, 53, 5558, 65, 6971, 73, 73t, 77, 78, 119, 123, 134136, 167
acute confusional 13, 14b, 15
acute treatment of 65
attack of 1, 46, 51
aura 11, 72
status 15
triggered seizure 15, 70
basilar-type 41
brain hyperexcitability 54
cascade 2
chronic 65, 184
complications of 15
epilepsy interface 80, 81
epileptic 70
episodic 184
familial hemiplegic 17, 23, 28, 77, 186
family history of 14
features of 70
headache 43, 54, 70, 74, 189
hemiplegic 20, 2224, 32, 78
history of 55, 174
normal 78
ophthalmoplegic 63
past medical history of 14
postdrome 7
specific picture of 71
sporadic hemiplegic 2224
typical 63
vestibular 4144, 48
visual aura, pathophysiology of 32
Migrainous infarction 15, 52
Migralepsy 70
Miosis 102, 110
Mitochondrial disorders 28
Mood disturbances 50
Moyamoya disease 90, 202
Moyamoya vessels, typical 200f
Muller's muscle 93
Muscles
interscapular 179
pupillary 93
Mycotic aneurysm 148
Myoclonic epilepsy, severe 29
Myopia, acute 59
N
Nasal congestion 102, 110, 126
Nasopharyngitis 66
Nausea 67, 82
Neck arteries, angiogram of 34
Neoplasm 13
Nerve origin hypothesis 211
Neuralgia
glossopharyngeal 114
secondary 175
trigeminal 101, 105t, 106, 106t
Neuritis, vestibular 42, 48
Neurological deficit 23, 204
transient 182
Neuromodulatory therapies 95
Neuromyelitis optica antibody test 207
Neurons 53
Neurovascular disorders 51
Nitric oxide 93, 99
synthase 162
Nociceptive signals, modulation of 3
Noninvasive vagus nerve stimulation 98
Nonsteroidal anti-inflammatory drugs 99
Nonvascular intracranial disorders 146
Nucleus complex, trigeminal 3
Nystagmus, downbeat 193
O
Obesity 67
Obstructive airways disease, chronic 167
Obstructive sleep apnea syndrome 12, 140
Occipital nerve 96
stimulation 97
Ocular pain 119, 120f
Oculosympathetic system, role of 114
Oligemia 51
spreading 54
Ophthalmic evaluation 213
Ophthalmoplegia 60, 61, 63
Optic
atrophy 27
neuropathy, anterior ischemic 179
Optokinetic stimulation 48
Orbital myositis 61
Organ transplantation 164
P
Pachymeningitis, hypertrophic 194, 195
Pain 179
central neuropathic 189
injection-site 66
intractable abdominal 135
localization of 105
periorbital 207, 208
Painful ophthalmoplegia, differential diagnosis of 61
Panic disorder 127
Papilledema 193
Paralytic episodes 28
Parenchymal signals 209
Paresthesias 73
Parieto-occipital lobes 160f
Patent foramen ovale 56
Pes cavus 27
Pheochromocytoma 149
Phobia, specific 127
Phobic postural vertigo 48
Phonophobia 82
Phosphenes 10
Photophobia 15, 47, 82, 182
persistent 47
severe 116
Pituitary adenylate cyclase-activating polypeptide 5, 93, 95
Polyarteritis nodosa 164
Polymyalgia rheumatica 179
Pontine hyperintense lesions 160f
Pontine vascular angioma 90
Porphyria 14, 164
Positron emission tomography 11, 94, 139
Preeclampsia 161, 162
Proline-rich transmembrane protein 2 78
Psychiatric disorder 49, 127
Psychosis, acute 14
Ptosis 102, 110, 126
Puerperium 164b
Pupillary function, sparing of 61
Pyruvate dehydrogenase deficiency 28
R
Rapid eye movement 166
Restless leg syndrome 193
Retinostriate pathways 47
Rhinorrhea 102, 110, 126
S
Salivatory nucleus, superior 93, 120
Sarcoidosis 61, 194
Sclerosis, multiple 189
Seizures 78, 86, 164f
cephalgic 72
classification of 71
complex partial 86
epileptic 70b
focal 71
sensory 86
unaware 86
generalized, epileptic 70
migraine-triggered 70
partial epileptic 70
Sensorium, altered 164b
Serum angiotensin-converting enzyme 193
Shock, electric 101
Short-lasting bursting holocranial headaches, episodic 158
Short-lasting unilateral neuralgiform headache attacks 88, 102, 103, 105, 105t, 106, 106t, 108
chronic 101, 103
episodic 103
Single-nucleotide polymorphisms 77
Sinus barotrauma 34
Sinusitis, frontal 90
Skin biopsy 37, 38
Sleep
apnea 193
disturbance 138
post-traumatic 50
Somatostatin 99
Spectrum disorder 142
Sphenopalatine ganglion 93, 95, 97, 120
Spinal cord stimulation, high-frequency 96
Status epilepticus, nonconvulsive 14
Status migrainosus 15
Stress 43
disorder
acute 127
post-traumatic 127
Stroke 40, 52
acute ischemic 52
causes of 51
greater risk of 55
hemorrhagic 57
history of 40
ischemic 52, 53, 56, 146
migraine-induced 51
migraine-related 52, 53
risk, migraine-related 53
Subcortical infarcts 24, 37, 39, 184
Sulfa drug allergy 138
Sulfonamides 140f
Sumatriptan 140f
Syphilitic pachymeningitis 194
Syringomyelia
reduction of 192f
spontaneous resolution of 193
Systemic lupus erythematosus 164, 175
T
Tacrolimus 149, 164
Temporomandibular disorder 91
Thrombotic-thrombocytopenic purpura 164
Thunderclap headache 125, 146, 152, 155, 158, 173
bath-related 156
differential diagnosis of 146b
primary 145, 147
Thyroid ophthalmopathy 61
Tinnitus 9, 13, 193
Tolosa-Hunt syndrome 61
Tongue, part of 208
Tonic-clonic seizure, generalized 82
Topiramate therapy 59
Tortuous basilar artery 209
Transcutaneous electrical stimulation 129f
Transesophageal echocardiography 22
Trigeminal autonomic cephalalgias 87, 92, 95, 108, 112, 114, 118, 123, 133, 134, 142, 213
differential features of 88t
non-sellar causes of 90b
Trigeminovascular pathway 5
activation of 5
Tuberculosis 194
Tumor 206
intracranial 14
parasellar 61
U
Upper motor neuron 34
Upper respiratory tract infection 66, 67
Urinary tract infection 67
V
Vagus nerve stimulation 98
Vanillylmandelic acid 158
Varicella zoster virus 187
Vascular disorders 28
Vascular smooth muscle cells 38
Vasculitis 61, 194
Vasoactive intestinal polypeptide 93
Verapamil 130, 204
Vertebrobasilar dolichoectasia 211
Vertebrobasilar system 181
Vertigo 9, 13, 24, 41, 44, 45, 47, 193
acute symptoms of 48
benign paroxysmal positional 26, 4244
causes of 44
cluster 118
migrainous 41
nonrotational 48
Vestibular diseases, acute 48
Vestibular migraine, pathophysiology of 43
Vestibular nerve, right 47
Vestibulocochlear nerve, right 46f
Vestibulo-ocular reflex 45
Vestibulopathy, migraine-related 41
Vestibulo-thalamocortical pathway 47
Vestibulo-visual symptoms 47
Video electroencephalography 80
Viral encephalitis 40, 164
Vision
recurrent blurring of 8
sudden onset blurring of 80
Visual aura 10, 37, 72
absence of 23
typical 58
Visual disturbance 12, 73
Visual loss 8, 178, 207, 213
irreversible 215
Visual migrainous symptoms 55
Visual tasks, precision 48
Vitamin
B1 14
B12 deficiency 14
D3 supplementation 177
Vomiting 67
von Willebrand factor
during migraine attacks 51
levels of 54
W
White matter lesions, asymptomatic 56
Z
Zonular fibers, relaxation of 59
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Chapter Notes

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Case Studies in UNCOMMON HEADACHE DISORDERS
Case Studies in UNCOMMON HEADACHE DISORDERS
Author Ambar Chakravarty MD FRCP Honorary Professor and Emeritus Consultant in Neurology Vivekananda Institute of Medical Science Kolkata, West Bengal, India Foreword G Arjundas
Jaypee Brothers Medical Publishers (P) Ltd
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Case Studies in Uncommon Headache Disorders / Ambar Chakravarty
First Edition: 2019
9789352706716
Printed at
To the memory of
Professor Shyamal Kumar Das
(1954–2018)
a dear friend and a very learned colleague
Contributors
AUTHOR
CO-AUTHORS
Foreword
Headaches still remain the commonest complaint for which patients seek neurologist advice. The fear of brain lesions is very high and rightly so.
And yet the detailed histories and deductions, both from patients and busy neurologists are not intense and quick.
This leads to premature and nonlogical resort to expensive imaging and other tests.
Conditions like chronic daily headaches, primary increased intracranial tension and migraine syndromes can almost be resolved with experience-based algorithms and minimal tests especially for those who are unable to afford expensive tests.
Case-based studies by senior neurologists and headache centers will be of great help to younger and less experienced neurologists than didactic reviews and updates to a clinician.
Professor Ambar Chakravarty and his colleagues are bringing out this most important volume on this very basis to help enlarge more accurate algorithms in headaches based on case reports of difficult cases of headaches of various etiologies.
It will help all neurologists as a referral book in practice.
It will enable logical choices for investigations and treatments and finally help the patients.
Professor G Arjundas
Chennai, India
Preface
The present volume is a unique one, the type of which is being published for the first time from India and contributed solely by Indian authors about cases encountered by them in their practice in India. This would be a case-based study being written about 51 uncommon cases of headache disorders, the types of which are not encountered in routine day-to-day practice by most neurologists. In addition, there are 10 commentaries, incorporating most recent information on subjects relevant to the cases discussed.
The cases are discussed in a very interactive way with the case history and findings detailed first followed by the investigations performed and the results of therapy, when applicable. This clinical note is followed by a series of questions which are likely to crop up in the minds of the readers after going through the case. The final section is the discussion, embedded in which would lie the answers to all the questions raised, along with other relevant and up to date information on the subject discussed. Not very detailed referencing had been done considering the time constraint for busy medical practitioners and trainees, and only few major ones are listed at the end of each case/chapter for those interested in more detailed discourses. All cases have been discussed in conformity with the current classification proposed by the International Headache Society in the International Classification of Headache Disorders (ICHD-3 2018) and published in Cephalalgia 2018; 38(1):1-211. This reference will not be repeated again and again in the text.
I have no hesitation in admitting that in preparation of this book, I had been influenced by a somewhat similar style adopted by Professor Alan Purdy of Nova Scotia, Canada and his colleagues in their book “Advanced Treatment of Migraine” written quite a few years back and currently not available in the market. I felt this would be the best form of learning for busy practicing clinicians and the trainees.
Headache medicine is becoming more and more popular in India and I am confident the present volume would be a source of inspiration for our younger generation to take up this subspeciality to master in. An attempt will be made to distribute this book free of any charge to all members of the Indian Academy of Neurology and to all residents in Neurology in the country to this end.
I am grateful to my collaborators Dr Angshuman Mukherjee and Dr Debasish Roy for their part contribution and constructive criticism of my thinking and writing. I must also thank the Publishers for their untiring efforts to bring out the volume in time as desired by me. Lastly, I must pay my tribute and thanks to Professor G Arjundas for kindly writing the Foreword of the book highlighting the very reason for publishing such a volume.
Ambar Chakravarty