Surgical Neurolog

Neoplasm

Garcin’s syndrome—a case report

Jacob Paul Alapatt, MCh, FRCS4, Sasi Premkumar, MBBS, MS,

Remesh Chirayil Vasudevan, MBBS, MSDepartment of Neurosurgery, Medical College Hospital, Calicut, Kerala, India 673017

Received 14 October 2005; accepted 22 March 2006

Abstract Background: Garcin’s syndrome in its complete form is very rare, and hence, this case is reported.

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doi:10.1016/j.surneu.2

Abbreviations: CT

tion cytology; MRI, m

4 Corresponding

Case Description: A 45-year-old patient presented to us with proptosis of the right eye and

progressive weakness of all cranial nerves on the right side without associated features of raised

intracranial pressure or long tract dysfunction. He had a bulge in the right buccolabial fold, from which

fine needle aspiration cytology was done. Cytology report came as a poorly differentiated carcinoma.

Conclusions: Garcin’s syndrome is a very rare neurosurgical entity. The course of the condition

depends on the nature of underlying pathology.

D 2007 Published by Elsevier Inc.

Keywords: Garcin’s syndrome; Cranial nerves

1. Case report

A 45-year-old man presented with complaints of pain

and numbness on the right side of his face for 1 year,

proptosis of the right eye, and diplopia, followed by

progressive vision reduction of the right eye. With these

complaints, the patient was treated in another institution

with systemic steroids and antituberculous drugs with a

working diagnosis of Tolosa-Hunt syndrome/tuberculosis

for 5 months. Because his neurologic status was gradually

deteriorating, these drugs were stopped. The patient

reached our hospital with development of deviation of

angle of mouth to the left side and difficulty in swallowing,

followed by nasal regurgitation of food. There was no

associated history suggestive of raised intracranial pressure,

weakness, or sensory impairment in any limbs. On

examination, all cranial nerves on the right side were

dysfunctional (Fig. 1). The patient’s higher mental func-

tions were normal, and there were no signs of cerebel-

lar dysfunction.

Magnetic resonance imaging scan of the head done at

the onset of his symptoms showed an ill-defined enhancing

lesion appearing isointense to white matter on T1 and T2,

nt matter D 2007 Published by Elsevier Inc.

006.03.041

, computed tomography; FNAC, fine needle aspira-

agnetic resonance imaging.

author. Tel.: +91 4952354353, +91 9447002931.

invading the right cavernous sinus and the right Meckel’s

cave. As the patient reached our hospital with development

of lower cranial nerve dysfunction, we repeated the MRI

scan of the head. It showed an extensive abnormal signal

in the right orbit with extension to the superior orbital

fissure, right cavernous sinus, right Meckel’s cave, and

posterior fossa (Fig. 2). Thickening of mucosa of para-

nasal sinuses and erosion of hard palate were observed.

Extension was also seen on the right cheek. We did

an FNAC from an obvious bulge seen on the right

y 67 (2007) 184–185

Fig. 1. Photograph of the patient revealing the right-sided palatal palsy,

wasting of tongue, and facial palsy.

Fig. 2. Magnetic resonance imaging scan of the head (sagittal view) of the

patient showing tumor infiltration along the middle cranial fossa floor,

extending to the orbit, cheek, and posterior fossa.

J.P. Alapatt et al. / Surgical Neurology 67 (2007) 184–185 185

buccolabial sulcus and reported poorly differentiated

carcinoma as the cause.

2. Discussion

Garcin’s syndrome is a rare syndrome of unilateral

paralysis of all or nearly all cranial nerves seen in the tumors

of nasopharynx [1-3] and the base of the skull, which does

not affect the brain itself. The syndrome is progressive and,

in its complete form, is seen only very rarely.

In Europe, many cases of multiple cranial nerve palsies

(even b7) that do not fit into any of the other named

syndromes were described as Garcin’s syndrome. Another

term used for multiple cranial nerve palsies due to skull

base lesions is hemibase syndrome [4]. Some reported

cases have other symptoms such as headache and signs

of hypopituitarism.

Prognosis as a rule is unfavorable. The findings on the

CT scan and MRI scan are important in early diagnosis.

Many cases of Garcin’s syndrome have been reported.

These include cases that are caused by tonsillar carcinoma,

nasopharyngeal carcinoma, sarcoma of the skull base,

metastasis, giant internal carotid aneurysm, chemodectoma,

tuberculosis, carcinomatous leukemic meningitis, and para-

nasal and parotid tumors. Nasopharyngeal carcinoma

frequently involves cranial nerves because of its proximity

to the skull base.

Our patient presented with history of numbness and pain

involving the right side of the face, weakness of extraocular

muscles, and, later, proptosis of the right eye, implying a

lesion originating near the paracavernous area spreading to

the right orbit. Over a period of 10 months, the lesion

gradually spread along the skull base to the posterior fossa,

producing paralysis of the lower cranial nerves.

References

[1] Goel A, Bhayani R, Satoskar A, Nagpal RD. Massive nasopharyn-

geal carcinoma mimicking Garcin syndrome. Neurol India 1995;43(2):

120 -1.

[2] Greulich W, Sackmann A, Schlichting P. Garcin syndrome. Clinical

aspects and diagnosis of a rare cranial nerve syndrome with special

reference to computerized tomography and nuclear magnetic resonance

image findings. Nervenarzt 1992;63(4):228 -33.

[3] Guillain G, Alajouanine Th, Garcin R. Le syndrome paralytique

unilateral globale des nerts craniens. Bull Med Hop (Paris) 1926;

50:456.

[4] Brazis PW, Masden JC, Billier J, editors. Cranial nerve XI, 4th ed.

Localization in clinical neurology. USA7 Lippincott, Williams and

Wilkins; 2001. p. 342.

Commentary

This case report describes a rare problem involving the

skull base. Multiple unilateral cranial nerve palsies involv-

ing cranial nerves of the cavernous sinus and jugular

foramen are rare and are associated with malignancy. This

report of Garcin’s syndrome is a nice review of this disease.

John B. Delashaw, Jr., MD

Portland, OR 97201, USA