Seminar rare headache syndromes

RARE HEADACHE SYNDROMES

by

SUCHARITA RAY

MODERATOR: DR ROHIT BHATIA

SOURCES AND BIBLIOGRAPHY:

1. Pubmed

2. Handbook in Clinical Neurology Vol. 97 (3rd series); G. Nappi and M.A.

Moskowitz, Headache, ©2011 Elsevier.

3. Atlas of Migraine and other headaches. Second Edition:2005; Stephen

D Silberstein, M Alan Stiles, William B Young.

4. The Green Journal of Neurology Continuum Series focusing on

headache, 2011 and 2005 various editions

5. Neurologic Clinics of North America various editions

6. Bradley’s Neurology in Clinical Practice. Sixth Edition

7. The International Classification of Headache Disorders, 3rd edition (beta

version) available online at www.ihs.org

8. www.google.co.in and wikipedia

http://www.ihs.org/

http://www.google.co.in/

TREPANATION 7000-3000

BC

Atlas of Migraine and other headaches;

© 2005 Taylor & Francis

Papyrus from Thebes, Egypt (2500

BC). Now in a British museum and the

accompanying cartoon explaining the

papyrus.

Atlas of Migraine and other

headaches; © 2005 Taylor & Francis

When Should we suspect more in a headache?

• Recent de novo headache

• Unusual headache in a subject with primary

headaches

• Thunderclap headache

• Focal neurological deficits

• Impaired Consciousness /confusion

• Abnormal neurological examination

• Papilledema

• Neck stiffness

• Fever

• Raised blood pressure

• Painful and inflammatory temporal arteries

WORLD STATEMENT ON HEADACHE

Globally, the percentages of the adult population with

an active headache disorder

• 46% for headache in general

• 11% for migraine

• 42% for tension-type headache and

• 3% for chronic daily headache

Stovner Lj et al :The global burden of headache: a documentation of headache prevalence

and disability worldwide. Cephalalgia March 2007 27: 193-210

THE NEED FOR DIAGNOSIS OF RARE HEADACHE

SYNDROMES

• Hitherto uncommon headache syndromes (SUNCT, hemicrania continua,

hypnic headache, and RCVS) are nowadays widely reported .*

• When considered as a group these headaches become sizeable in

number*

• Important also to identify rare manifestations of common headache

disorders**

* Bigal ME. Expert commentary-unusual headache syndromes. Curr Pain Headache

Rep.2012;16:287-8

**Casucci G, d’Onofrio F, Torelli P. Rare primary headaches: Clinical insights. Neurol Sci.

2004;25 (Suppl. 3):S77-S83.

ICHD-3 beta Cephalalgia 2013 © International Headache society 2013/4

CLASSIFICATION

Part 1:

Primary headache disorders

Part 2:

Secondary headache disorders

Part 3:

Painful cranial neuropathies and other facial pains


CLASSIFICATION

Part 1: The primary headaches

1. Migraine

2. Tension-type headache

3. Trigeminal autonomic cephalalgias

4. Other primary headache disorders


PART 2: THE SECONDARY HEADACHES

5. Headache attributed to trauma or injury to the head and/or

neck

6. Headache attributed to cranial or cervical vascular disorder

7. Headache attributed to non-vascular intracranial disorder

8. Headache attributed to a substance or its withdrawal

9. Headache attributed to infection

10. Headache attributed to disorder of homoeostasis


Part 3: Painful cranial neuropathies, other facial

pains and other headaches

13. Painful cranial neuropathies and other facial

pains

14. Other headache disorders


1. MIGRAINE

1.1 Migraine without aura

1.2 Migraine with aura

1.3 Chronic migraine

1.4 Complications of migraine

1.5 Probable migraine

1.6 Episodic syndromes that may be associated with

migraine


1.4 COMPLICATIONS OF MIGRAINE

1.4.1 Status migrainosus

1.4.2 Persistent aura without infarction

1.4.3 Migrainous infarction

1.4.4 Migraine aura-triggered seizure


1.4.1 STATUS MIGRAINOSUS

A. A headache attack fulfilling criteria B and C

B. In a patient with migraine with/without aura typical of

previous attacks except for its duration and severity

C.Both of the following characteristics:

1. unremitting for >72 h

2. pain and/or associated symptoms are debilitating

D.Not better accounted for by another ICHD-3 diagnosis


Marion Beltramone, Anne Donnet, Cephalalgia;

December 2013,(0)0, 1–5

Background: About the prevalence of Status migrainosus

(SM) and migraine aura status (MAS) in patients with migraine

in one tertiary care center

Methods: 11-year retrospective study

Duration: October 2002 through June 2013, in 8821 patients

enrolled in the participating in the French Observatory of

Migraine and Headaches (OMH)

Five migraine complications were considered:

1. Status migrainosus (>72 hours)

2. Persistent aura without infarction

3. Migrainous infarction

4. Migraine aura-triggered seizure, and

5. Migraine aura status (MAS) (2 auras/day over at least three

consecutive days in the MA patient)

RESULTS FROM THE STUDY:

Among 8821 patients studied:

24 had SM, three had MAS and one had both forms. (<3%,

SM>MAS)

Mean duration: SM---4.8 weeks and MAS---4 weeks.

Stress and menstruation were the main precipitating factors for SM

(68.8% and 31.3%, respectively).

No precipitating factor was found for MAS.

For a majority of patients, the frequency of migraine attack was the

same before and after SM or MAS.

SM and MAS occurred more frequently in patients with initial low-

frequency migraine attacks.

Conclusions: SM and MAS are rare complications of migraine

Similar frequency of migraine attacks occurs before and after SM.

1.2.2 MIGRAINE WITH BRAINSTEM AURA

A. At least 2 attacks fulfilling criteria B and C below, and

criteria C and D for 1.2.1 Migraine with typical aura

B. Aura of fully reversible visual, sensory and/or

speech/language symptoms, but not motor or retinal

C. 2 of the following brainstem symptoms:

1. dysarthria

2. vertigo

3. tinnitus

4. hypacusis

5. diplopia

6. ataxia

7. decreased level of consciousness

ICHD-3 beta Cephalalgia 2013 © International Headache society

2013/4

1.2.2 Migraine with brainstem aura

Terminology and coding changes 1988-2013

1.2.2 Migraine with brainstem aura was previously classified

as

1.2.6 Basilar-type migraine in ICHD-II

1.2.4 Basilar migraine in ICHD-I

There is little evidence that the basilar artery or, basilar-artery

territory is involved.

The name change was done to preclude the involvement of

basilar artery in the pathogenesis of the headache.


The differential diagnosis of migraine with brainstem aura:

• Hemiplegic migraine:

• Presence of unilateral weakness/paralysis

• Transient ischemic attack (TIA)/stroke

• Think if similar picture occurs in adults

• Symptoms begin simultaneously ( sequential in

migraine)

• CADASIL (CADASIL-Coma):

• High burden of T2/FLAIR hyperintensity in the

periventricular and deep WM and particularly in the

anterior temporal pole and external capsule

*Basilar-type migraine typically decreaes in frequency and severity

in adulthood and may disappear, (like hemiplegic migraine) by age

40. Often the attacks give way to typical migraine with/without aura.

HEMIPLEGIC MIGRAINE

A. At least 2 attacks fulfilling criteria B and C

B. Aura consisting of both of the following:1. fully reversible motor weakness; 2. fully reversible visual, sensory and/or speech/language symptoms

C. 2 of the following 4 characteristics:1. 1 aura symptom spreads gradually over ≥5 min,

and/or 2 symptoms occur in succession2. each individual non-motor aura symptom lasts 5-60min, and motor symptoms last <72 h3. 1 aura symptom is unilateral4. aura accompanied or followed in <60 min by headache

D. Not better accounted for by another ICHD-3 diagnosis, and TIA excluded


FAMILIAL HEMIPLEGIC MIGRAINE

Initially described in 1910

Recurrent headache associated with transient hemiparesis.

AD

Those with family history ( FHM-AD) and without i.e. Sporadic

Hemiplegic migraine

For the familial form:

FHM1: Chr 19p13; CACNA1A. Also seen with Episodic ataxia

type 2, spinocerebellar ataxia type 6, and benign paroxysmal

torticollis of infancy

FHM2: Chr 1q23; ATP1A2 gene which codes for a Na+K+

ATPase pump.

FHM3: Chr 2q24; SCN1A gene coding for a voltage gated

sodium channel

Rohit Bhatia, Soaham Desai, Manjari Tripathi, Ajay Garg ,

M. V. Padma, Kameshwar Prasad, Mamta B. Singh

Case scenario:

30-year-old male with headache and left hemiparesis with

visual aura and systemic signs preceding the headache.

There was no history of fever, trauma, seizure, or

dehydration preceding the episode.

In the preceding 16 years, he had about 50 such episodes,

age of onset at 14 years.

No family history.

Possible differential diagnosis of recurrent hemiplegia

1> Transient ischemic attacks and recurrent strokes

2> Complex partial seizure with and without secondary generalization

(with post-ictal Todd’s palsy)

3> One of the migraine with aura subtypes:

(a) Hemiplegic migraine.(sporadic or familial)

(b) Migraine with brainstem aura (Basilar-type migraine)

(c) Migrainous infarction (complication of migraine)

4> Mitochondrial encephalopathy with lactic acidosis and stroke-like

episodes (MELAS) syndrome

5 >Pseudomigraine with lymphocytic pleocytosis with transient

neurologic deficits

6> Alternating hemiplegia of childhood

(a) T1-weighted axial MRI brain, reveals right cerebral hemispheric

sulcal effacement and cortical thickening which is hyperintense on

T2WI and (b) FLAIR sequence

Followup MR at 4 weeks shows resolution of changes

SPORADIC HEMIPLEGIC MIGRAINE

•Most patients with SHM have ‘typical’ aura symptoms (visual, sensory

and/or aphasic) associated with motor weakness during the acute

attack.

•The auras are more prolonged in sporadic hemiplegic migraine as

compared to a typical migraine with aura.

•Absence of infarction on imaging helps in differentiating it from

migrainous infarction or stroke associated with migraine.

Whitty CWM (1953) Familial hemiplegic migraine. J NNeurosurg Psychiatry 16:172-77

Thomsen LL, Olesen J (2004) Sporadic hemiplegic migraine. Cephalalgia 24:1016–1023

IMAGING FINDINGS OF SPORADIC HEMIPLEGIC

MIGRAINE

•Restricted diffusion, normal or increased diffusion based on

DWI and ADC values involving a hemisphere opposite to the

side of deficit associated

• Normal T2W, T1W images, angiography .

•Abnormalities have been found to be resolve over 4–12

weeks.

Restricted diffusion probably due to cell swelling leading to

ATP dysfunction.

Jacob A, Mahavish K et al (2006) Imaging abnormalities in sporadic hemiplegic

migraine on conventional MRI, diffusion and perfusion MRI and MRS. Cephalalgia

26(8):1004–1009.

MANAGEMENT OF SPORADIC HEMIPLEGIC MIGRAINE

•Treatment strategy : Flunarizine, Naloxone and Verapamil

•Sympathomimetic drugs like ergotamine derivatives and triptans

are avoided in acute attacks (vasospasm may lead to permanent

sequelae)

•Beta-blockers are not used for prophylaxis in patients with

hemiplegic or basilar-type migraine: risk of prolonged aura

/migrainous infarction.

• SHM is a diagnosis of exclusion.

Centonze V, Brucoli C, Macinagrossa G, Attolini E, Campanozzi F, Albano O (1983) Non-

familial hemiplegic migraine responsive to naloxone. Cephalalgia 3:125–127

Yu W, Horowitz SH (2003) Treatment of sporadic hemiplegic migraine with calcium-channel

blocker verapamil. Neurology 60:120–121

Tobita M, Hino M, Ichikawa N, Takase S, Ogawa A (1987) A case of hemiplegic migraine

Pseudomigraine with lymphocytic pleocytosis and

transient neurologic deficits*

Very rare condition with single/multiple attacks of migraine like

headaches, weakness, sensory and visual auras with confusion

and sometimes meningismus lasting minutes to hours.

CSF lymphocytic pleocytosis (10–760 cells/mm3); Normal protein

and sugar and have a normal brain imaging. No infectious etiology

Also known as Headache and Neurological Deficits with

cerebrospinal fluid (CSF) Lymphocytosis (HaNDL) syndrome

Visual auras are very uncommon (12%) as compared to sensory

symptoms (78%). This helps it to differentiate from migraine with

brainstem aura.

* Filina T, Feja K N, Tolan R W: An Adolescent With Pseudomigraine, Transient Headache, Neurological

Deficits, and Lymphocytic Pleocytosis (HaNDL Syndrome). Clinical Pediatrics: 2013 Jun;52(6):496-502.

Cephalalgia; 2010; 30(11) 1284–1289:

Cerebral autosomal dominant arteriopathy with

subcortical infarcts and leukoencephalopathy

(CADASIL)

NOTCH3 gene ( ? Migraine susceptibility gene)Recurrent stroke, cognitive decline, psychiatric disturbances and

migraine.

The prevalence of migraine in CADASIL is slightly higher than in the

general population, The proportion of migraine with aura is much

higher

• increased susceptibility to cortical spreading depression (CSD)

• different expression of CSD

Total of 27 articles on migraine in CADASIL

Prevalence of headache: European studies: 14% to 72%.

Four studies from Asian countries: low migraine prevalence of 5%.

CADASIL and migraine: A narrative review. Liem MK, Oberstein SA, van der grond J, Ferrari

Cerebral autosomal dominant arteriopathy with subcortical

infarcts and leukoencephalopathy (CADASIL)

Pooling of all results: Overall migraine prevalence of migraine is 38%

(151/510) with a 95% CI of 33–42%.

When excluding Asian countries, the pooled prevalence is 43%

(187/434) with a 95% CI of 38–48%.

Cause for low incidence of Migraine in CADASIL patients:

? R544C mutation associated with less headache in Asian patients*

*Headache among CADASIL patients withR544C mutation: Prevalence, characteristics, and

associations. CC Jay et al. Cephalalgia January 2014 vol. 34 no. 1 22-28

3. Trigeminal autonomic cephalalgias (TACs)

3.1 Cluster headache

3.2 Paroxysmal hemicrania

3.3 Short-lasting unilateral neuralgiform headache attacks

3.4 Hemicrania continua

3.5 Probable trigeminal autonomic cephalalgia


3.2 Paroxysmal hemicrania

A. At least 20 attacks fulfilling criteria B-E

B. Severe unilateral orbital, supraorbital and/or temporal pain

lasting 2-30 min

C.1 of the following ipsilateral symptoms or signs:

[1.conjunctival injection and/or lacrimation, 2.nasal congestion and/or

rhinorrhoea, 3. eyelid oedema,4.forehead and facial sweating, 5.

forehead and facial flushing, 6.sensation of fullness in the ear, 7.

miosis &/or ptosis]

D. Frequency >5/d for > half the time

E. Prevented absolutely by therapeutic doses of indomethacin

F. Not better accounted for by another ICHD-3 diagnosis


3.3 Short-lasting unilateral neuralgiform headache

A. At least 20 attacks fulfilling criteria B-D

B. Moderate/severe unilateral head pain (orbital, supraorbital, temporal

and/or other trigeminal distribution) lasting 1-600 seconds as single

stabs, series of stabs or in a saw-tooth pattern

C. 1 of the following ipsilateral cranial autonomic symptoms or signs ( as in

PH)

D. Frequency 1/d for > half the time when active

E. Not better accounted for by another ICHD-3 diagnosis

First described by Sjaastad et al; Slight higher prevalence in males*

* Sjaastad O, Russell D, Horven I, Bunaes U. Multiple neuralgiform unilateral headache attacks associated with conjunctivalinjection and appearing in clusters. A nosological problem. Proceedings of the Scandinavian Migraine Society 1978; 31

3.3 SHORT-LASTING UNILATERAL NEURALGIFORM HEADACHE

ATTACKS

Two subtypes recognized:

3.3.1 Short-lasting unilateral neuralgiform headache attacks

with conjunctival injection and tearing (SUNCT)

3.3.2 Short lasting unilateral neuralgiform headache attacks

with cranial autonomic symptoms (SUNA)

3.3.1 SUNCT may be a subform of 3.3.2 SUNA

Mean age of onset 50 years (10 to 77 years)


•To aid diagnosis of SUNCT/SUNA an indomethacin test and

oxygen trial, which will rule out PH and CH, respectively.

•Intravenous lidocaine is effective for short-term prevention

• Lamotrigine and topiramate are recommended as preventives,

and gabapentin may have a beneficial effect in SUNA.

•Greater occipital nerve injections may play a role.

SUNCT and SUNA: Recognition and Treatment. JA Pareja et al. Current Treatment Options

in Neurology (2013) 15:28–39

Duration versus frequency attacks of TACs :-

3.4 HEMICRANIA CONTINUA

A. Unilateral headache fulfilling criteria B-D

B. Present >3 mo, with exacerbations of moderate or greater

intensity

C.Either or both of the following:

1. 1 of the following ipsilateral symptoms or signs as in

PH

2. a sense of restlessness or agitation, or aggravation of

pain by movement

D. Responds absolutely to therapeutic doses of

indomethacin

E. Not better accounted for by another ICHD-3 diagnosis


4. OTHER PRIMARY HEADACHE DISORDERS

4.1 Primary cough headache

4.2 Primary exercise headache

4.3 Primary headache associated with sexual activity

4.4 Primary thunderclap headache

4.5 Cold-stimulus headache

4.6 External pressure headache

4.7 Primary stabbing headache

4.8 Nummular headache

4.9 Hypnic headache

4.10New daily persistent headache (NDPH)


PART 2: THE SECONDARY HEADACHES

5. Headache attributed to trauma or injury to the head and/or neck

6. Headache attributed to cranial or cervical vascular disorder

7. Headache attributed to non-vascular intracranial disorder8. Headache attributed to a substance or its withdrawal9. Headache attributed to infection10. Headache attributed to disorder of homoeostasis 11. Headache or facial pain attributed to disorder of cranium,

neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cranial structure

12. Headache attributed to psychiatric disorder


6.7 Headache due to other acute intracranial arterial

disorder

6.7.1 Headache attributed to an intracranial endovascular

procedure

6.7.2 Angiography headache

6.7.3 Headache attributed to reversible cerebral

vasoconstriction syndrome (RCVS)

6.7.4 Headache attributed to intracranial arterial

dissection


6.7.3 Headache attributed to RCVS

A. Any new headache fulfilling criterion C

B. Reversible cerebral vasoconstriction syndrome (RCVS)

diagnosed

C.Evidence of causation demonstrated by ≥1 of:

1. headache, with or without focal deficits and/or seizures,

has led to angiography and diagnosis of RCVS

2. headache has ≥1 of the following characteristics:

a) recurrent during ≤1 mo, and with thunderclap onset

b) triggered by sexual activity, exertion, Valsalva, emotion,

bathing and/or showering

3. No new significant headache occurs >1 m after onset

D.Not better accounted for by another ICHD-3 diagnosis, and

aneurysmal SAH excluded


REVERSIBLE CEREBRAL VASOCONSTRICTION

SYNDROME

• Diffuse, multifocal narrowing of the cerebral arteries, typically

heralded by a sudden, severe headache with or without neurologic

deficit

•Females slightly more than males, mean age of onset around 45

years.

•Underlying pathological basis is unclear, probably due to alterations

in cerebral vascular tone, either spontaneously or evoked by

exogenous or endogenous factors.

• Most cases resolve within a 1-3 month period,

• Major complications: Subarachnoid hemorrhage (20-25%) and

ischemic or hemorrhagic stroke (30%) can occur.

Eponyms for reversible cerebral vasoconstriction

syndrome

Isolated benign cerebral vasculitis

Benign acute cerebral angiopathy

Call–Fleming syndrome

Central nervous system pseudovasculitis

Benign angiopathy of the central nervous system (BACNS)

Post-partum angiopathy

Migrainous vasospasm

Fatal vasospasm in migrainous infarction

Migraine angiitis

Thunderclap headache with reversible vasospasm

Idiopathic thunderclap headache

Primary thunderclap headache

Drug-induced cerebral vasculopathy/angiopathy

Reversible Cerebral Vasoconstriction Syndrome. Holly Yancy et al, Headache Currents; 2013;

570-7

Conditions associated with RCVS-I

•Pregnancy and puerperium

•Exposure to illicit drugs and alcohol

•Cocaine, ecstasy, marijuana, methamphetamine, lysergic acid

diethylamide (LSD), binge ETOH drinking

•Medications:

•Nasal decongestants (eg, phenylpropanolamine

pseudoephedrine, ephedrine)

•ergotamine tartrate, methergine

• bromocriptine, lisuride,

• SSRIs, triptans, isometheptine

•amphetamine derivatives

•tacrolimus (FK-506), cyclophosphamide, erythropoietin,

•ginseng, human placenta extract

Reversible Cerebral Vasoconstriction Syndrome. Holly Yancy et al, Headache Currents; 2013;

570-7.

Conditions associated with RCVS-II

•Exposure to blood products

•Intravenous immune globulin, red blood cell transfusion

•Idiopathic

•Catecholamine secreting tumors:

•Pheochromocytoma, bronchial carcinoid tumor, glomus

tumors

•Vascular disorders

• Unruptured cerebral aneurysm, post-carotid

endarterectomy, cervical dissection, dysplasia

•Miscellaneous:

•Hypercalcemia, porphyria

•Neurosurgical and neurointerventional procedures

•Head trauma

CLINICAL FEATURES OF REVERSIBLE CEREBRAL

VASOCONSTRICTION SYNDROME

•Recurrent thunderclap headaches over several days to weeks are

highly characteristic of RCVS.

•Women aged 20 to 50 and slightly older age than males who present

in third decade.

•Complications: focal or generalized seizures in up to 20%.

•Permanent or transient focal neurologic deficits may represent

transient ischemic attack, infarct, or intracranial hemorrhage

•The vascular abnormalities of RCVS are reversible and this defines

the diagnosis.

• Vasoconstriction typically outlasts the headaches by several weeks

Yancy, H., Lee-Iannotti, J. K., Schwedt, T. J. and Dodick, D. W. (2013), Reversible Cerebral

vasoconstriction Syndrome. Headache: The Journal of Head and Face Pain, 53: 570–576.

doi: 10.1111/head.12040

Cerebral angiography is the gold standard

Characteristic “string of beads” sign

MRA or CTA is recommended to display the diffuse

alternating pattern of vasodilation and vasoconstriction.

Fig 1 and 2: Diagnostic angiogram with multiple areas of vasoconstriction in anterior

circulation.

Yancy, H., Lee-Iannotti, J. K., Schwedt, T. J. and Dodick, D. W. (2013), Reversible Cerebral

vasoconstriction Syndrome. Headache: The Journal of Head and Face Pain, 53: 570–576.

doi: 10.1111/head.12040

7. HEADACHE DUE TO NON-VASCULAR INTRACRANIAL

DISORDER

7.1 Headache attributed to increased cerebrospinal fluid

pressure

7.2 Headache attributed to low cerebrospinal fluid pressure

7.3 Headache attributed to non-infectious inflammatory

intracranial disease

7.4 Headache attributed to intracranial neoplasia

7.5 Headache attributed to intrathecal injection

7.6 Headache attributed to epileptic seizure

7.7 Headache attributed to Chiari malformation type I

7.8 Headache attributed to other non-vascular intracranial

disorder


7.2 HEADACHE ATTRIBUTED TO LOW CEREBROSPINAL

FLUID PRESSURE

7.2.1 Post-dural puncture headache

7.2.2 CSF fistula headache

7.2.3 Headache attributed to spontaneous intracranial

hypotension


Spontaneous Low CSF Pressure/Volume Headache

Orthostatic headache is the mc symptom

Other C/F : neck pain, nausea, emesis, interscapular pain,

diplopia, dizziness.

Pathogenesis : Esp in autonomic disorders (e.g POTS)

syndrome there may occur orthostatic CSF hypovolemia

Orthostatic pooling of the venous blood in the lower limbs

Decreased pressure in epidural venous plexus

Resultant decrease in CSF pressure

Mokri, B. (2013), Spontaneous Low Pressure, Low CSF Volume Headaches: Spontaneous

CSF Leaks. Headache: The Journal of Head and Face Pain, 53: 1034–1053.

doi: 10.1111/head.12149

Cerebrospinal fluid analyses:

Opening pressure: Low/Negative/normal

Color: Usually transparent, occasionally xanthochromic

Cells: maybe present, upto 50cells/cumm is common

NCCT Head

Limited utility.

Look for subdural fluid collection and/or tentorial herniation

Radioisotope Cisternography

Very useful for detecting csf leaks

M/c abnormality: Absence/paucity of radioactivity over the

cerebral convexities at 24-48 hrs

Site of csf leak: Zone of parathecal activity

Meningeal diverticula: Multiple zones of parathecal activity

Early activity in kidneys and urinnary bladder s/o extradural

csf

Magnetic resonance imaging

Meningeal enhancement: Pachymeningeal (not leptomeningeal)

Supratentorial as well as infratentorial

Linear & non-nodular; uninterrupted & bilateral, usually thick but

maybe subtle and thin.

Ferrante, E., Savino, A., Sances, G. and Nappi, G. (2004), Spontaneous Intracranial

Hypotension Syndrome: Report of Twelve Cases. Headache: The Journal of Head and Face

Pain, 44: 615–622. doi: 10.1111/j.1526-4610.2004.446012.x

•Descent of brain (sagging or sinking of the brain): Descent of

cerebellar tonsils ( like in type I Chiari malformation)

•Decrease in size of prepontine cistern

• Inferior displacement of the optic chiasm

• Effacement of perichiasmatic cisterns

• Crowding of the posterior fossa

Bilateral subdural collection with pachymeningeal thickening

and epidural thickening

Ferrante, E., Savino, A., Sances, G. and Nappi, G. (2004), Spontaneous Intracranial

Hypotension Syndrome: Report of Twelve Cases. Headache: The Journal of Head and Face

Pain, 44: 615–622. doi: 10.1111/j.1526-4610.2004.446012.x

Part 3: Painful cranial neuropathies, other

facial pains and other headaches

13.Painful cranial neuropathies and other facial pains

14.Other headache disorders


13. Painful cranial neuropathies and other facial pains

13.1 Trigeminal neuralgia

13.2 Glossopharyngeal neuralgia

13.3 Nervus intermedius (facial nerve) neuralgia

13.4 Occipital neuralgia

13.5 Optic neuritis

13.6 Headache attributed to ischaemic CN III palsy

13.7 Tolosa-Hunt syndrome

13.8 Paratrigeminal oculosympathetic (Raeder’s) syndrome

13.9 Recurrent painful ophthalmoplegic neuropathy

13.10 Burning mouth syndrome (BMS)

13.11 Persistent idiopathic facial pain (PIFP)

13.12 Central neuropathic pain


Glossopharyngeal neuralgia

Severe transient stabbing pain in the ear, base of the tongue, tonsillar

fossa, or beneath the angle of the jaw.

Younger patients than TN (40% of patients are under 50 years).**

Female (67%) > Male (33%) patients**

Occasionally vascular compression of the glossopharyngeal nerve by the

posterior inferior cerebellar artery at the root entry zone.

Other causes of Glossopharyngeal Neuralgia:1. Elongated or fractured styloid process

2. Calcified stylohyoid ligament (Eagle’s syndrome)

3. Cerebellopontine angle tumors

4. Parapharyngeal space lesions

5. Carcinoma of the parapharyngeal space, pharynx, nasopharyngeal

carcinoma, posterior fossa arteriovenous malformation

6. Multiple sclerosis

**Patel A, Kassam A, Horowitz M et al. (2002). Microvascular decompression in the

management of glossopharyngeal neuralgia: analysis of 217 cases.Neurosurgery;705-720

CLINICAL FEATURES

Hyperactivity of the CN IX afferents activation of the dorsal motor

nucleus of the vagus nerve vagal efferent response severe

bradycardia /asystole.

•Major difference is in location of pain

•Paroxysmal and lasts for seconds to minutes, with remission.

• Provoked by swallowing sharp foods, talking, or coughing

• Examination is unremarkable.

•The symptomatic forms are often secondary to intra- or extracranial

compressions near the jugular foramen.

•MRI or a panoramic radiograph is useful to exclude Eagle’s

syndrome. An electrocardiogram may be useful to rule out cardiac

abnormalities.

Patel A, Kassam A, Horowitz M et al. (2002). Microvascular decompression in the

management of glossopharyngeal neuralgia: analysis of 217 cases. Neurosurgery

50: 705–710.

Sampson JH, Grossi PM, Asaoka K et al. (2004). Microvascular decompression for

glossopharyngeal neuralgia: long-term effectiveness and complication avoidance.

Neurosurgery 54: 884–889.

MANAGEMENT

The first-line medical treatment: CBZ and all other treatment for

Trigeminal Neuralgia.

Other drugs like Baclofen, Gabapentin and pregabalin have been

seen to be effective in selected cases.

Important to rule out secondary causes.

Surgical treatment : Nerve sectioning to Microvascular

decompression .

NERVUS INTERMEDIUS NEURALGIA

•A rare condition characterized by brief paroxysms of

pain felt deeply in the ear.

•Pain paroxysms are intermittent, last for seconds or

minutes

• Triggered by touching the posterior wall of the auditory

canal.

•Pain is sometimes accompanied by disorders of

lacrimation, salivation, or taste.

•There is a common association with herpes zoster (HZ).

OPHTHALMIC POSTHERPETIC NEURALGIA

Localized infection caused by the VZV.

The main complication of HZ is postherpetic neuralgia (PHN), a

neuropathic pain persisting more than 3 months after skin eruption.

10% of those with HZ will develop PHN

Higher frequency in elderly and diabetics.

Thoracic dermatomal involvement : 50%

Ophthalmic division of the CN V: 22–25%

Pain usually constant or paroxysmal and dynamic allodynia is seen.

Local examination: Scarring and skin changes.

Both large and small sized trigeminal afferents are affected

Pain can be paroxysmal (demyelination of Aβ fibers) and constant

pain(Aδ and C axons)

Truini A, Galeotti F, Haanpaa M et al. Pathophysiology of pain in postherpetic

neuralgia: a clinical and neurophysiological study. 2008; Pain 140: 405–410.

Because of the eye involvement medical management is the

first line of treatment rather than using topical agents.

Recent EFNS guidelines recommend as first-line treatment

with the tricyclic antidepressants, gabapentin/pregabalin, and

topical lidocaine

Attal N, Cruccu G, Haanpaa M et al. (2006). EFNS guidelines on pharmacological

treatment of neuropathic pain. Eur J Neurol 13: 1153–1169.

Seminar rare headache syndromes

Health & Medicine

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