A Classification of Headache according to the new System and the Rare headache types not so commonly encountered in clinical practice

1. Rare Headache Syndromes
Moderator: Dr Rohit Bhatia
Presenter: Dr Sucharita Ray

2. SEARCH STRATEGY:
PubMed
GOOGLE SCHOLAR
HANDBOOK OF CLINICAL NEUROLOGY: Headache; Volume 97
International Classification of Headache Disorders 2nd edition (ICHD-2) criteria
T J Schwedt, J P Gladstone, R A Purdy and D W Dodick

3. Acute
• Subarachnoid hemorrhage
• Cervical artery dissection
• Acute hypertensive crisis
• Ischemic stroke
• Pituitary apoplexy
• Colloid cyst of the third
ventricle
• Primary thunderclap
headache
• Intracranial infection
Episodic
• Hemiplegic migraine
• Basilar-type migraine
•Cluster headache
•Episodic paroxysmal
hemicrania
•SUNCT syndrome
•Chiari malformation
•Trigeminal neuralgia
• Subacute angle-closure
glaucoma
•Primary stabbing headache
•Primary headache associated
with sexual activity
•Primary cough headache
Chronic
•Giant cell arteritis
•Cerebral venous sinus
thrombosis
•Spontaneous intracranial
hypotension
• Idiopathic intracranial
hypertension
• Intracranial neoplasm
•Sinus headache
•Medication-overuse headache
•Post-traumatic headache
•Cervicogenic headache
•Occipital neuralgia
•Hypnic headache

4. Miscellaneous Syndromes that are truly rare !!!
Interactive case studies.

5. CASE 1:
A 26-year-old pregnant woman presented with nausea, vomiting, and headache. There
was no past medical history of note and she was not taking any medication. O/E she was
drowsy and uncooperative. No E/o focal neurology.

6. CASE 2:
A 62-year-old woman presented with a 3-day history of generalized headache of gradual
onset associated with neck stiffness and malaise. On the day of admission, she had
awoken covered in blood with no memory of what had happened. She was taking
tamoxifen for breast cancer diagnosed 6 months previously for which she had had a
mastectomy. On examination, GCS was 14/15 and there was a laceration over the
occiput. There was no focal neurological abnormality.

7. CASE 3:
A 19-year-old woman presented with diarrhoea and vomiting, headache and visual
disturbance. She was a smoker and was on OCPs. O/E there was papilloedema and a right
VI nerve palsy. She subsequently deteriorated developing bilaterally reduced visual acuity
and bilateral VI nerve palsies and then right arm weakness.

8. CASE 4
A 31-year-old man presented with acute onset headache a/w right arm weakness. There
was a past H/O hepatic abscess and hepatic vein thrombosis. O/E he was alert and there
was no papilloedema but he had a right CN VI and right-sided pyramidal weakness. He
had 2 generalized motor seizures in the ED.

9. The diagnosis is cerebral venous sinus thrombosis (CVT).
A schematic figure of the cerebral venous sinus system is shown in Fig. 2.1.
In CVT, the superior sagittal sinus (see Figs. 2.2 and 2.3) and the transverse
sinuses (see Figs. 2.3 and 2.4) are the most commonly affected followed
by the straight sinuses (see Figs. 2.5 and 2.6) and the cavernous
sinuses. More than one sinus is usually affected. Thrombosis of the
Galenic system (see Fig. 2.6) or isolated involvement of the cortical veins
is infrequent. CVT is often accompanied by raised intracranial pressure
since the dural sinuses contain most of the arachnoid villi and granulations
in which CSF absorption takes place.
Occlusion of one of the larger venous sinuses without involvement of
cortical veins or the Galenic venous system generally causes raised
intracranial pressure in the absence of focal neurological signs (

11. Hence it is important to know that a single clinical phenomenon may
have an acute, chronic and episodic presentation. Hence management
should be guided by a proper history, observation of the clinical signs
and clinical examination to aid in the diagnosis
Still for the most part, certain clinical syndromes can present with some
very typical clinical and case scenarios. A knowledge of these signs and
symptoms can help clinch the diagnosis at presentation itself.

13. 52 year old female presented with acute onset of headache, 4 to 5
episodes of vomiting, retroorbital pain and blurring of vision in
both eyes since early morning.
By the time she was rushed to the ER, she was found to be
confused about his name and address, was seen to have a BP of
77/43mmHg and was able to count fingers at three feet.
Past history was only significant for hypertension

18. The patient was a 36-year-old retropositive woman with a H/O multiple opportunistic
infections, including P carinii and M kanasii pneumonias, cerebral toxoplasmosis, and
cryptococcal meningitis. She developed unexplained fever and headache with nuchal
rigidity with progressive deterioration of her mental status.
O/E: Cushing response (hypertension with bradycardia), b/l nonreactive and small pupils
(1mm), decorticate rigidity and extensor plantar reflexes.
An emergent CT Head was done

20. The patient was intubated and continued on broad-spectrum
antibiotic therapy. An emergency ventriculostomy was
performed, which relieved the increased intracranial pressure
but clinical improvement was not seen.

21. POST OPERATIVE CT SCAN

22. Another ventriculostomy was inserted . Despite appropriate medical therapy, the
patient’s neurologic function continued to decline. Two additional ventriculostomies
yielded no clinical benefit.
Owing to the absence of cortical activity and poor prognosis, life support was withdrawn,
and the patient expired 7 days after last admission

25. Rare lesions comprising 0.5–1% of primary brain tumors.
Occur in the third to fifth decades of life ,mc site anterior third ventricle>
lateral> fourth ventricle>outside ventricular system.
C/F: Headaches are brief, lasting seconds to minutes, and are usually relieved
by standing.
A well recognized cause of sudden death. Other symptoms include
progressive dementia, drop attacks, and spells of transient loss of
consciousness.
Cyst size does not appear to be a reliable predictor of outcome
Histologically benign, but may obstruct the foramina of Monro to produce
acute hydrocephalus. Intermittent foraminal obstruction due to attachment
to roof of third ventricle.
Attachment to the third ventricular roof may impart a pendulous character.

27. 24-year-old female c/o left-sided throbbing headaches associated
with blurring of vision followed later by development of right-sided
hemiparesis and aphasia and later started having right focal motor
seizures.
Past H/O similar type of headache 12 years back with right sided
weakness alternating with left sided weakness
Duration of few hours and then reverting back to normal.
Imaging (CT, MRI,MRA), csf lactate serum lactate all were normal
No family history

29. Hemiplegic migraine was initially described in 1910 as a type of
migraine consisting of recurrent headache associated with transient
hemiparesis.
AD form of migraine with aura in which some degree of hemiparesis
is present during attacks
Aura typically lasts longer than in migraine with aura and usually
comprises visual, sensory, aphasic, and motor symptoms
Family History maybe absent.
Two subforms of FHM families exist—pure FHM in 80% and FHM
families with cerebellar symptoms in 20%
Responsible gene CACNA1A encoding a neuronal calcium channel,
are present in 50% of families with hemiplegic migraine including
those with cerebellar signs

30. A 56-year-old woman c/o 5-year history of daily multiple (upto 5)
episodic short-lasting (15-20 seconds each)
Attacks occurred up to five times a day and lasted 15–20 s each.
The attacks were left-sided, moderately severe and retro-orbital a/w
marked tearing and redness of the ipsilateral eye with mild
rhinorrhoea but no nausea, photophobia or phonophobia.
No history of migraine nor family history of headache.
Clinical examination normal.
MRI of the brain was normal.
No particular response to any of the medications ( TCAs/PPnl/
methysergide/ lithium/verapamil, trial of steroids
/ergots/triptans/indomethacin, valproate/CBZ)

31. subtype of the trigeminal autonomic cephalalgias (TACs) . It is characterized by frequent
(up to 200/day), strictly unilateral, severe, neuralgiform attacks in the ophthalmic division
of the trigeminal nerve which are brief in duration (60 seconds) and occur in association
with conjunctival injection and tearing
The diagnosis of SUNCT syndrome is based on operational
diagnostic criteria (Box 17.1)
. The differential diagnosis of SUNCT includes trigeminal
neuralgia (see Chapter 19), primary stabbing headache
(see Chapter 21), paroxysmal hemicrania, and cluster
headache (see Chapter 15 and Fig. 17.1)
. Clinically similar to trigeminal neuralgia; however, SUNCT is
almost always confined to V1 while trigeminal neuralgia is
confined to V1 in less than 5% of patients. In addition, SUNCT
attacks are longer, associated with autonomic symptoms, and
have a refractory period
. Secondary causes of SUNCT have been reported with lesions
occurring most commonly in the pituitary gland, parasellar
region, and posterior fossa
. MRI brain with coronal enhanced

32. images of the pituitary is
required to rule out a secondary cause
. A trial of indomethacin is helpful to exclude an indomethacinresponsive
headache
n Treatment
. SUNCT is more refractory to treatment than other primary
headache disorders
. The pharmacologic treatments with reported success in case
reports and case series include: anticonvulsants (lamotrigine,
gabapentin, carbamazepine, and topiramate), corticosteroids,
and intravenous lidocaine
n
n
n

33. SUNCT – International Headache Society diagnostic
criteria
A. At least 20 attacks fulfilling criteria B–E
B. Attacks of unilateral, orbital, supraorbital or temporal stabbing
or pulsating pain lasting 5–240 seconds
C. Pain is accompanied by ipsilateral conjunctival injection and
lacrimation
D. Attacks occur with a frequency from 3 to 200 per day
E. Not attributed to another disorder
10
0
10
0
10
0
CPH
Cluster
SUNCT
1 h
Time