inTervieW - Green Cross Publishing

32
case sTudy
Cluster headache in a 24
year old woman
a
24-year-old female patient
presented with a four year history
of daily headaches. She reported
8-10 attacks per day and the
duration of attacks lasted from 30-
90 minutes. Most of these attacks
would occur spontaneously but she always got
an attack awakening her from sleep at around
2am. When she awoke with this attack it would
make her restless and agitated, forcing her to
pace the bedroom floor until it subsided after
approximately 30 minutes. The typical clinical
characteristics of her headaches and associated
symptoms were:
• Abrupt in onset and occurred without warning
• Always unilateral in location in the left
periorbital/frontal region and extending to
the temple
• Duration 30-90 minutes
• Sharp, piercing and burning in character
• Very severe
• Forehead sweating
• Associated with restlessness, agitation and a
feeling like ‘banging’ her head off a wall
• Ipsilateral conjunctival tearing
• Ipsilateral conjunctival reddening
• Ipsilateral nasal congestion.
• Ipsilateral nasal rhinorrhoea.
• Ipsilateral ptosis of left upper eye lid.
• Ipsilateral miosis of left pupil.
Other symptoms associated with some
attacks included intermittant photophobia,
Prevalence Common: 10-12 per cent
population
phonophobia, nausea and occasional vomiting.
The rest of the physical and neurological
examination was normal.
These headaches were having a major impact
on her life. She was unable to get any relief from
the attacks and as an acute therapy she was
consuming up to 10 paracetamol daily without
benefit. She was also on amitriptylline 25mg
nocte as a preventive therapy, with no resultant
change in the frequency of attacks. Over the
years she had tried many other analgesics and
preventive therapies without benefit. In addition
she had been prescribed courses of antibiotics
for a presumptive diagnosis of chronic sinusitis.
Her life was being controlled by the headaches
and she was unable to pursue a career or get a
job. She previously had been working as a clerical
officer but was forced to give up her job. Her
social life had become non-existent. During this
four-year period she had attended emergency
departments and GP out-of-hours services on
many occasions, all of whom either diagnosed
‘severe migraine’ or ‘sinusitis’ and for immediate
pain relief she was treated acutely with either
parenteral courses of nSAIDs or narcotic agents,
all of which were ineffective.
Investigations carried out over the four-year
period included a CTScan brain, CTScan sinuses,
MRI scan brain and many blood tests, all of which
were entirely normal.
This patient has a diagnosis of chronic cluster
headaches, a benign primary headache disorder.
Migraine Cluster headache
Femalea:Male 3:1 1:5
Rare:0.5 per cent Population
Frequency of attacks 2 attacks per month 2-8 attack per day
Duration 4-72 hours 15 minutes –180 minutes
Headache Unilateral (70 per cent) or bilateral
(30 per cent)
Always unilateral
Character of headache Throbbing and Pounding Sharp/piercing or ‘boring’
Location Vary from frontal to temporal,
parietal and occipital locations.
Periobital, orbital and
temporal in location
severity Severe Excruciatingly painful
autonomic symptoms Seldom Frequent
nausea / vomiting Common Rare
Photophobia and
Phonophobia
Common Rare
impact Patient needs to lie down in a
quiet dark room
Patient becomes restless,
agitated and paces the floor
issue 10 volume 12 • novemBeR 2010
dr edwArd M. o’sullIvAN
ClINICAl dIreCtor HeAdACHe/MIGr AINe
dePArtMeNt of NeuroloGy
Cork uNIversIt y HosPItAl
ANd GP Cork CIt y.
At her initial consultation, the following
management plan was put in place:
• Keep a headache diary
• Discontinue paracetamol and amitriptylline
• As an acute therapy, the patient was
commenced on 100 per cent oxygen through
a non re-breathing mask at a high flow rate of
7-10 litres per minute for 15 minutes for each
attack.
• As a preventive therapy, she was prescribed
verapramil 240mg daily.
• Patient was advised to stop smoking.
Ten days after presentation the patient was
followed-up with a telephone consultation. She
was much improved. Her cluster attack rate was
now two per day and the attacks were relieved
by the oxygen therapy within 10 minutes. The
attacks were also less severe.
At a subsequent telephone consultation
another week later the patient was experiencing
her first headache-free days in four years. The
frequency of attacks was 1-2 per day and with
the oxygen therapy, the attack was aborted in 10
minutes.
discussion
Cluster headache is a rare primary benign
headache disorder which exists in acute and
chronic forms. It is characterised by a headache
described as being excruciatingly painful and
known as one of the ‘worst pains known to man’.
Women have described it as being more painful
then childbirth. Many patients have been driven
to suicide because of their severity.
The headache is always a unilateral headache
typically located in the periorbital/orbital and
temporal regions with ipsilateral autonomic
symptoms: conjunctival tearing, reddening, nasal
congestion, rhinnorhoea, ptosis and miosis. The
case study outlined is a text book description
of the disorder, nevertheless the patient went
for many years without a correct diagnosis
and as a consequence the most appropriate
treatment choices to optimise her care were
never prescribed. The delay in diagnosis I would
attribute to the rarity at which cluster headache
is encountered in clinical practice. Many doctors
will only see a few cases during the course of
their career. It is most often wrongly diagnosed
as migraine which, by comparison, is the most
frequent headache disorder for which a patient
attends their doctor. The clinical symptoms
which differentiate between migraine and cluster
headache are tabulated below.
neurophysiology
The understanding of the neuro/
pathophysiology mechanisms of cluster
headaches is hypothesised on the activation
and integration of the trigeminovascular
and cranial parasympathetic systems. The
ophthalmic division of the trigeminal nerve is
the pain sensitive pathway of the intracranial
structures: cerebral arteries, meninges. The
driving mechanism for trigeminovascular
activation is hypothalamic dysfunction. On
activation, neurotransmitters (CGRP, neurokinin
and substance P) are released via a retrograde
transmission from the peripheral end of the
trigeminal nerve which innervate the proximal