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Garcin syndrome associated with metastatic colorectal carcinoma

2004; Elsevier BV; Volume: 117; Issue: 3 Linguagem: Inglês

10.1016/j.amjmed.2004.02.040

1555-7162

Surmeet Bedi, Vijay Krishnamoorthy, Lijo Simpson, John N. Sheagren,

Head and Neck Surgical Oncology

A 59-year-old-man presented to the emergency department with right-sided facial pain and difficulty swallowing of about 2-weeks duration. He had a history of disseminated colorectal carcinoma. Initial examination showed motor and sensory involvement of the right V cranial nerve. All other cranial nerves, including the gag test for IX and X, were intact. Video swallow studies and esophagogastroduodenoscopy suggested motor dysphagia. Over the next few days of hospitalization, the patient progressively developed cranial nerves palsies of VII, VIII, IX, X, XI, and XII, which completed the clinical picture of Garcin syndrome. Magnetic resonance imaging (MRI) of the brain showed an extra-axial enhancing lesion in the vicinity of the right cerebellopontine angle that extended along the posterior aspect of right temporal lobe, suggesting a metastatic lesion. A survey of the literature indicates that this may be one of the first reported cases of Garcin syndrome caused by colorectal carcinoma. Garcin syndrome is a progressive ipsilateral involvement of the cranial nerves resulting in paralysis of all or most cranial nerves without involvement of long tracts or the cerebellum, and without signs of increased intracranial pressure (1Adams R. Victor M. Ropper A.H. Principles of Neurology: The Major Categories of Neurologic Diseases.6th ed. The McGraw-Hill Companies, Inc, 1997Google Scholar). It has been reported in association with primary nasopharyngeal tumors and a variety of metastatic tumors. Radiation is the preferred mode of treatment for palliation, but the prognosis is poor (2Greelich W. Sackmann A. Schlichling P. Garcin syndrome. Clinical aspects and diagnosis of a rare cranial nerve syndrome with special reference to computer topography and nuclear magnetic resonance image findings.Nervenarzt. 1992; 63: 228-233PubMed Google Scholar). Garcin syndrome can also occur because of infections and as a paraneoplastic syndrome (with increased anti-Hu antibodies in patients with small cell lung cancers) (3Fujimoto S. Kumamoto T. Ito T. Sannomiya K. Inuzuka T. Tsuda T. A clinicopathological study of a patient with anti-Hu- associated paraneoplastic sensory neuropathy with multiple cranial nerve palsies.Clin Neurol Neurosurg. 2002; 104: 98-9102Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar). This patient had Garcin syndrome caused by a metastasis from his colorectal carcinoma. The patient was diagnosed with colorectal carcinoma metastatic to the liver in March 2002. He initially refused treatment; however, he presented 3 months later with an intestinal obstruction, at which time he underwent exploratory laparotomy with a colostomy. He subsequently received three cycles of chemotherapy with 5FU, leucovorin, and irinotecan. Six months, later the patient was admitted with a 1-week history of worsening right-sided facial pain and dysphagia, both for solids and liquids. He also complained of nasal fluid regurgitation and voice changes. He did not have odynophagia or visual, hearing, or olfactory problems. On examination, the patient appeared emaciated. Chest and cardiovascular system examinations were normal. Abdominal examination showed a functioning colostomy bag. Neurological examination revealed right temporalis and massater muscle wasting and deviation of the jaw to the right on opening the mouth. Corneal and conjunctival reflexes were lost on the right side. All sensory modalities were decreased on the right side of the face. He had atrophy of the right sternocleidomastoid muscle compared with the left side and also atrophy and fasciculation of the right half of the tongue with deviation of the tongue to the right. He had a poor gag reflex. The remainder of the neurological examination was normal. Laboratory tests were notable for mild normochromic, normocytic anemia and elevated alkaline phosphatase levels. The coagulation profile and cerebrospinal fluid analyses were normal. An MRI of the head (Figure) revealed an extra-axial enhancing lesion in the vicinity of the right cerebellopontine angle; MRI had been normal 1 month before, indicating a rapidly growing tumor. While in the hospital, the patient developed progressive right-sided facial weakness and deafness, indicating new 7th and 8th cranial nerve palsies. The patient was started on palliative radiation therapy to the base of the skull and showed symptomatic improvement in cranial nerve function and a decrease in right-sided facial pain. Garcin syndrome was first described by the French physician, Raymond Garcin, in 1926 as “Syndrome paralytique unilateral global des nerfs craniens” also known as the hemibasal syndrome, Guillain-Garcin syndrome, or Bertolletti-Garcin syndrome. The patient we observed presented with progressive involvement of cranial nerves culminating in total unilateral paralyses of all the cranial nerves, with an absence of motor or sensory signs in the limbs, no increase in intracranial pressure, and abnormal radiographs of the base of the skull (1Adams R. Victor M. Ropper A.H. Principles of Neurology: The Major Categories of Neurologic Diseases.6th ed. The McGraw-Hill Companies, Inc, 1997Google Scholar). In many cases, isolated cranial nerves are spared (2Greelich W. Sackmann A. Schlichling P. Garcin syndrome. Clinical aspects and diagnosis of a rare cranial nerve syndrome with special reference to computer topography and nuclear magnetic resonance image findings.Nervenarzt. 1992; 63: 228-233PubMed Google Scholar). Not all patients reported as having Garcin syndrome, including those described by Garcin himself, have shown total unilateral cranial nerve palsies. Garcin syndrome can now be diagnosed before the clinical criteria are met (4Kaya H. Nakamura S. Matano S. et al.Acute nonlymphocytic leukemia complicated by GS.Acta Hematol. 1995; 94: 142-143Crossref PubMed Scopus (4) Google Scholar) because of possible early diagnosis with MRI, as was the case in our patient. Garcin syndrome has been described in association with cancers of nasopharyngeal and paranasal carcinomas; rhabdomyosarcoma cylindromas; craniopharyngiomas; and metastases from cancers of the breast, lung, liver, or uterus (5Mubaidin S.I. Sunna J.B. Beiruti M.A. Shennak M.M. Ayoub M.S. Renal cell carcinoma presenting as GS.J Neuro Psych. 1990; 53: 613-614Crossref Scopus (13) Google Scholar). It also can be a paraneoplastic syndrome. Garcin syndrome should be in the differential diagnosis of unilateral progressive multiple cranial nerve palsies. The work-up should be directed towards finding an underlying malignancy or infection. The prognosis is usually unfavorable.