Healthcare and Medicine Reference
In-Depth Information
Chapter 4
CHARLIN'S SYNDROME
demyelinating disease ( Figure 4-2 ). Magnetic resonance angiog-
raphy (MRA) also may be useful in helping identify aneurysms,
which may be responsible for the patient's neurological findings.
In patients who cannot undergo MRI, such as a patient with a
pacemaker, computed tomography (CT) is a reasonable second
choice. Radionuclide bone scanning and plain radiography are
indicated if fracture or bony abnormality such as metastatic dis-
ease is considered in the differential diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of Char-
lin's syndrome is in question. Intraocular pressure should be mea-
sured if glaucoma is suspected.
ICD-9 CODE 350.1
ICD-10 CODE G50.0
T he C liniCal S yndrome
Charlin's syndrome, also known as nasociliary neuralgia, is an
uncommon cause of head and face pain. As with most headache
syndromes, the exact cause of the pain of Charlin's syndrome is
unknown. However, the pathogenesis of this uncommon cause
of head and face pain is thought to be dysfunction of the naso-
ciliary ganglion in a manner analogous to the dysfunction of the
sphenopalatine ganglion thought to be the source of cluster head-
ache. The pain of Charlin's syndrome has a rapid onset to peak,
with attacks lasting 45 to 60 minutes. In some patients, these
attacks can be triggered by sensory stimulation of the affected
areas. Although in many ways similar to cluster headache (e.g.,
retroorbital location of pain, profuse unilateral rhinorrhea, rapid
onset to peak, and short duration of attacks), many dissimilarities
also exist. In contrast to cluster headache, alcohol consumption
does not appear to trigger attacks of Charlin's syndrome and the
seasonal and chronobiological patterns so characteristic of cluster
headache do not seem to be a factor ( Table 4-1 ). Blockade of the
sphenopalatine ganglion, which is so effective in the treatment of
cluster headache, is of little value in the treatment of Charlin's
syndrome. Patients suffering from Charlin's syndrome uniformly
respond to daily nasociliary nerve blocks with local anesthetic, as
described subsequently.
d ifferenTial d iagnoSiS
Charlin's syndrome is a clinical diagnosis supported by a com-
bination of clinical history, normal physical examination, radi-
ography, and MRI. Pain syndromes that may mimic Charlin's
syndrome include cluster headache, temporal arteritis, trigemi-
nal neuralgia involving the first division of the trigeminal nerve,
demyelinating disease, and chronic paroxysmal hemicrania (see
Table 4-1 ). Trigeminal neuralgia involving the first division of
the trigeminal nerve is uncommon and is characterized by trig-
ger areas and tic-like movements. Demyelinating disease is gener-
ally associated with other neurological findings, including optic
neuritis and other motor and sensory abnormalities. The pain of
chronic paroxysmal hemicrania lasts much longer than the pain of
Charlin's syndrome.
T reaTmenT
The treatment of Charlin's syndrome is analogous to the treat-
ment of trigeminal neuralgia. The use of anticonvulsants such as
carbamazepine and gabapentin represents a reasonable starting
point. High-dose steroids tapered over 10 days also have been
anecdotally reported to provide relief. For patients who do not
respond to the previously mentioned treatments, daily nasociliary
ganglion block with local anesthetic and steroid is a reasonable
next step. Underlying sleep disturbance and depression associated
with the pain of supraorbital neuralgia are best treated with a tri-
cyclic antidepressant compound, such as nortriptyline, which can
be started at a single bedtime dose of 25 mg.
S ignS and S ympTomS
Patients suffering from Charlin's syndrome present with the com-
plaint of severe paroxysms of ocular or retroorbital pain that radi-
ates into the ipsilateral forehead, nose, and maxillary region. This
pain is associated with voluminous ipsilateral rhinorrhea and con-
gestion of the nasal mucosa and significant inflammation of the
affected eye ( Figure 4-1 ).
T eSTing
Magnetic resonance imaging (MRI) of the brain provides the best
information regarding the cranial vault and its contents. MRI is
highly accurate and helps identify abnormalities that may put the
patient at risk for neurological disasters secondary to intracranial
and brainstem pathological conditions, including tumors and
C ompliCaTionS and p iTfallS
Failure to diagnose Charlin's syndrome correctly may put
the patient at risk if an intracranial pathological condition or
9
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