Garcin syndrome in a patient with rhinocerebral mucormycosis]

Garcin syndrome is characterized by an unilateral cranial nerves involvement without sensory or motor long-tract disturbances. It is usually caused by tumor infiltrating in the skull base with osteolytic changes on radiological study. We report a case of 64-year-old man with history of alcohol overintake, who admitted local hospital, because of right periorbital edema and facial swelling. He noted right ptosis 2 weeks prior to admission. Neurological examination revealed right multiple cranial nerves involvement including II, III, IV, V, and VI cranial nerves. MR imaging of the brain showed marked paranasal sinusitis and abnormal infiltration of right orbital fat. Orbital apex syndrome related to paranasal sinusitis was diagnosed, and antibiotics was administered. But a few days after admission, he developed a right VII, IX, X cranial nerve palsy. He was transferred to our hospital because of acute development of left hemiparesis and deteriorated consciousness. MR imaging of the brain showed right internal carotid artery (ICA) occlusion, and infarction in right middle cerebral artery (MCA)'s territory. The diagnostic biopsy of the paranasal sinus showed mucorales hyphae, indicating that the pathological diagnosis was mucormycosis. Despite of antibiotic therapy included of amphotericin-B administration and strict control of diabetic mellitus, his sinusitis was gradually spread. His condition progressively deteriorated, and finally died of sepsis. Post-mortem examination revealed a widespread mucor infiltration in the dura mater without skull bone invasion. This case presented with unilateral multiple cranial nerve involvements (Garcin syndrome) followed by left hemiparesis associated with rhinocerebral mucormycosis. It is suggested that mucormycosis should be considered in case of Garcin syndrome without osteolysis in the skull base.     

Garcin syndrome resulting from a giant cell tumor of the skull base in a child

Garcin syndrome is characterized by a progressive ipsilateral involvement of cranial nerves, culminating in paralysis of all or at least seven of them, without sensory or motor long-tract disturbance, with no intracranial hypertension, and with osteoclastic involvement in the skull base on radiographic computed tomography. Giant cell tumor is a primary bone tumor rarely affecting the skull base. An 8-year-old female presented with a 3-month history of increasingly worsening right otalgia, tinnitus, hearing loss, right facial numbness, and diplopia. She was admitted with a 2-week history of swallowing difficulties, voice change, and right shoulder pain. Neurologic examination disclosed unilateral paralysis of the right fifth through twelfth cranial nerves, with no other abnormal neurologic findings. Skull radiographic computed tomography revealed lytic lesions in the right temporal petrous portion. Computed tomographic scan indicated a destructive mass involving the right greater wing of the sphenoid bone and temporal petrous apex. Magnetic resonance imaging demonstrated a tumor arising from the temporosphenoidal region, infiltrating neither the brain nor the brainstem. No hydrocephalus was observed. Biopsy revealed giant cell tumor. Posterior treatment consisted of radiotherapy. At an 8-year follow-up, the patient was well but with functional sequelae. There is no magnetic resonance imaging evidence of tumor growth. No other giant cell tumor presenting as Garcin syndrome is known to have been reported.     

Unilateral multiple cranial nerve palsies mimicking Garcin syndrome as an atypical symptom of brainstem encephalitis: a case report]

We report a 56-year-old Japanese man who was admitted because of dysphagia and left side facial dysesthesia. On admission, his general conditions were unremarkable. Neurological examination revealed that he was alert and well oriented. He exhibited left-side cranial nerve involvement such as the trigeminal(sensory and motor), glossopharyngeal and potential vagal nerve palsy. He exhibited neither long-tract signs, such as motor weakness, sensory disturbance in his exremities and pathological reflex, nor ataxia. By a few days after admission, his symptoms had worsened. Vertigo and diplopia appeared, and his consciousness level became drowsy. Bilateral third and sixth nerves, left fourth, fifth, eighth, ninth and tenth nerves were involved. Results of laboratory tests and CSF studies were within normal. Results of examination of the skull base X-ray and MRIs of the brain were normal. Administration of corticosteroid and intravenous administration of a high dose of gamma-globulin were not effective. His symptoms gradually recovered three months after admission. Based on clinical symptoms and results of physiological examination, i.e., an involvement of his consciousness and abnormal findings in blink reflex test that suggest involvement of the brainstem, he was diagnosed as having brainstem encephalitis. Although the exact pathophysiological mechanisms were unclear, it is clinically important to note that an atypical brainstem encephalitis may present a subacute progressive appearance of unilateral multiple cranial nerve palsies mimicking Garcin syndrome.     

Garcin syndrome. Clinical aspects and diagnosis of a rare cranial nerve syndrome with special reference to computerized tomography and nuclear magnetic resonance image findings]

Garcin syndrome is an ipsilateral step-by-step deterioration of all 12 brain cranial nerves, first described in 1927. The underlying cause is usually a sarcoma or carcinoma of the skull base. The pathogenesis of the Garcin syndrome is chiefly determined by the slow localized growth of these tumors, rather than by their histology and primary localisation. The prognosis is as a rule unfavourable. The findings on computed and magnetic resonance tomography are important for an early diagnosis. Two cases of Garcin syndrome are reported, one of which was probably due to a tonsillar carcinoma, while the other was caused by a mucinous adenocarcinoma of the nasopharynx.     

An extremely unusual presentation of varicella zoster viral infection of cranial nerves mimicking Garcin syndrome

We report a patient with the varicella zoster viral (VZV) infection of multiple cranial nerves mimicking Garcin syndrome, who initially presented with Ramsay Hunt syndrome (herpes zoster oticus). A 78-year-old man showed left facial palsy with zosteric eruptions in his left auricle and dysphagia, followed by left total ophthalmoplegia. His serum anti-VZV antibody titer was elevated. Cerebrospinal fluid examination revealed pleocytosis with a slightly elevated protein level. He was treated with intravenous acyclovir and corticosteroids. His tongue weakness resolved, and then ocular movement improved. The improvement of facial palsy and swallowing difficulty was delayed. VZV infection should be considered even in patients who show unilateral multiple cranial neuropathy mimicking Garcin syndrome because it is treatable.     

Polyneuritis of cranial nerves and acquired immunodeficiency syndrome (AIDS): 5 cases

Cranial neuropathies were present in 5 patients with positive serology for the human immunodeficiency virus. Two patients presented with abnormalities of ocular movements (3rd, 4th), two with an isolated unilateral facial nerve palsy and one with a lesion of the accessory nerve. Neurological symptoms and signs are present in approximately 60 to 80 p. 100 of cases of the acquired immunodeficiency syndrome, but cranial neuropathies affect only 2 to 3 p. 100 of the patients. The isolation of the human immunodeficiency virus from the nerve suggest a direct role, but an indirect immune mechanism may also be present. Some of the patients with aseptic meningitis or subacute encephalopathy have demonstrated involvement of cranial nerves, mainly 2nd, 7th and 8th. Systemic tumors (lymphoma) may involve the central nervous system by diffuse meningeal invasion with lesions of 3rd, 4th and 6th nerves. Opportunist infections like Herpes zoster or Cytomegalovirus may produce cranial neuropathies (2nd, 5th). Isolated mononeuropathies or cranial nerves palsies have also been reported in patients treated with chemotherapeutic agents like vinca alkaloids.     

Giant parasellar aneurysm simulating pituitary tumor

Seven cases of compromised pituitary fossa at the conventional skull X-ray, who had the final diagnosis of giant aneurysm of the intracavernous portion of the carotid artery (6 cases) and one of the anterior communicating artery, are reported. The main findings were: headache (7/7), complex ophthalmoplegia involving the III, IV and VI cranial nerves (5/7), compromised V cranial nerve (4/7) and eyeball pain (4/7). Other manifestations were: meningeal signs (2/7), unilateral blindness (1/7), hemiparesis (1/7), cacosmia (1/7) and inferior bitemporal quadrantanopsia (1/7). Five patients with intracavernous carotid artery aneurysm showed benefits with progressive occlusion of the internal carotid artery at the cervical level. One died before surgery. The case with anterior communicating artery aneurysm improved after its surgical clipping. Our data, in accord with the literature, support the conclusion that the differential diagnosis of aneurysms in the parasellar region remains a very difficult task. The accurate final diagnosis requires cerebral angiography and the surgical treatment with progressive occlusion at the cervical portion of the internal carotid artery has a relatively low risk with promising results.     

A clinicopathological study of a patient with anti-Hu-associated paraneoplastic sensory neuronopathy with multiple cranial nerve palsies

Only a few cases of paraneoplastic neurologic syndrome with multiple cranial palsies have been reported. This is the case report of a patient with small-cell lung cancer and a high titer of anti-Hu antibodies who developed a tonic left pupil and multiple cranial nerve palsies, including palsies of the left fifth through tenth nerves and both twelfth nerves, as in Garcin syndrome showing at least more than seven ipsilateral cranial nerve palsies, in the course of paraneoplastic sensory neuronopathy (PSN). Pathologic examination revealed no metastasis or direct invasion of malignancy with gliosis and perivascular inflammation throughout the brainstem, indicating paraneoplastic encephalomyelitis (PEM). The numbers of EBM11+ cells (probably reactive microglia), CD8+ cells, and CD4+ cells increased. Intracellular adhesion molecule-1 and lymphocyte function associated molecule-1 were expressed intensely on the endothelia of microvessels and were found to have infiltrated mononuclear cells around microvessels in the brainstem. Multiple cranial nerve palsies and their effects including the tonic pupil are likely due to the paraneoplastic effect of the primary systemic malignancy.     

Osteopathia striata with cranial sclerosis. Report of a case and review of the literature

This article describes a case of osteopathia striata with cranial sclerosis. The patient also has multiple sclerosis. The symptomatology includes a right sided conduction deafness and a left maxillar nerve deficit, which were both attributed to the bone disorder. The authors review the literature of this rare genetic syndrome and pay special attention to the neurological manifestations. These mainly consist of hearing loss, mental subnormality and occasionally the involvement of other cranial nerves. The bone scan in their patient shows hyperactivity in the left skull base region. This finding provides further evidence that, at least in some instances, the bone disorder has a progressive course.     

Renal cell carcinoma presenting as Garcin''s syndrome.

A 50 year old man presented with progressive unilateral cranial nerve palsies (Garcin's syndrome) for one year. Skull radiography and computed tomography (CT) showed intracranial extension of a soft tissue tumour from the right base of the skull. Necropsy revealed a papillary cell carcinoma of the right kidney and metastases in the base of the skull.     

Gone in the blink of an eye – A Tolosa-Hunt syndrome variant

Tolosa-Hunt syndrome is a rare disorder characterized by severe peri-orbital headache and ophthalmoplegia resulting from pseudotumour in the cavernous sinus compressing structures within it, namely cranial nerves III, IV, and VI and the superior divisions of cranial nerve V. We report the case of a 47 year old female who presented with painless left unilateral ptosis and complete external ophthalmoplegia. Magnetic Resonance Imaging (MRI) identified an enhancing heterogeneous mass filling the left cavernous sinus, following the course of the oculomotor nerve. After 2 weeks symptoms and signs resolved and there was a parallel resolution of the MRI findings, without the administration of corticosteroids. As far as we are aware this is one of the first reports of Tolosa-Hunt syndrome variant, with painless neurological involvement confined solely to the oculomotor nerve, and with complete resolution without pharmacological intervention.     

Primary Non-Hodgkin Lymphoma of the Skull Base Presenting with Garcin Syndrome: MRI Manifestations

Primary non-Hodgkin lymphoma of the skull base is a rare disorder. We report a case of primary non-Hodgkin lymphoma of the skull base presenting with Garcin syndrome. MRI revealed peculiar lesions in the cavernous sinus, clivus, and occipital bone. Diagnosis was made by biopsy of the tumor in the cavernous sinus.     

B-cell neurolymphomatosis confined to the peripheral nervous system

Patients with neurolymphomatosis show lymphoma cells within the peripheral nerves, nerve root/plexus, or cranial nerves. However, most neurolymphomatosis patients show lymphomatous infiltration not only in the peripheral nervous system (PNS), but also in the meninges, Virchow–Robin space, and brain parenchyma. Here, we report a 74-year-old woman with diffuse large B-cell lymphoma presenting with motor–sensory–autonomic polyneuropathy and multiple cranial neuropathies. A diagnosis of neurolymphomatosis was made by sural nerve biopsy. Postmortem examination indicated that lymphoma cell infiltration in the nervous system was confined to the PNS with no involvement of the central nervous system, including the meninges. This was a very rare case of B-cell neurolymphomatosis with lymphomatous infiltration confined to the PNS, suggesting specific affinity of the lymphoma cells for the PNS in this patient.     

Gradenigo syndrome as presenting sign of T-cell lymphoma

Gradenigo syndrome is an uncommonly observed neurologic complex consisting of cranial nerve VI palsy associated with the loss of the sensory component of cranial nerve V. We report a patient whose presentation with Gradenigo syndrome led to the unexpected diagnosis of T-cell lymphoma and briefly discuss Gradenigo syndrome and intracranial lymphomas.     

Lymphoma and peripheral neuropathy: a clinical review

Lymphoma occasionally affects the peripheral nervous system. When it does, the diagnosis can be elusive since many patients present without known lymphoma. Most peripheral nerve complications are due to non-Hodgkin's lymphoma (NHL), which infiltrates nerves causing axonal damage. This disorder can affect nerve roots and cranial nerves, often associated with lymphomatous meningitis. NHL may also infiltrate peripheral nerves and cause plexopathy, mononeuropathy, or generalized neuropathy. These neuropathies may resemble an asymmetric mononeuropathy multiplex or a generalized disorder such as chronic inflammatory demyelinating polyradiculoneuropathy. When NHL infiltrates diffusely, the term neurolymphomatosis is used. Hodgkin's lymphoma (HL), by contrast, rarely infiltrates nerves. More often, HL causes immunological disorders of the peripheral nervous system such as inflammatory plexopathy or Guillain-Barré syndrome. Other rare lymphomas such as intravascular lymphoma and Waldenstrom's macroglobulinemia can also affect peripheral nerves in specific ways. In addition, other malignant and nonmalignant lymphoproliferative disorders enter into the differential diagnosis of lymphomatous neuropathy. This review discusses the multiple peripheral nerve presentations of lymphoma from the clinician's point of view and provides a guide to the evaluation and diagnosis of these uncommon, challenging disorders.     

Ungewöhnliche Manifestation eines Zoster sine herpete als unilaterales kaudales Hirnnervensyndrom

Multiple lower cranial nerve palsies are a rare complication following varicella zoster virus (VZV) reactivation, especially if typical herpetic eruptions are lacking. We report a case of a 45-year-old, immunocompetent male with unilateral involvement of the cranial nerves VIII, IX, X, and XI without skin lesions. Cerebrospinal fluid (CSF) studies revealed mononuclear pleocytosis with intrathecal antibody synthesis against VZV, while polymerase chain reaction (PCR) did not detect VZV or HSV (herpes simplex virus). The patient almost completely recovered after aciclovir administration. VZV reactivation without rash (zoster sine herpete) may lead to multiple cranial nerve palsies. PCR is a useful tool to detect VZV-DNA in CSF, but negative results do not exclude a reactivation. In case of multiple cranial nerve palsies of unknown etiology with mononuclear pleocytosis in CSF tumors of the skull base, meningitis tuberculosis, and meningeosis have to be excluded, and antiviral therapy should be discussed.     

Multiple cranial neuropathy as a feature of internal carotid artery dissection

The traditional presentation of spontaneous internal carotid artery dissection includes ipsilateral hemicranial headache, oculosympathetic paresis, and contralateral focal cerebral ischemic deficits. However, we describe two cases with multiple cranial nerve involvement ipsilateral to the dissection as the principal feature. The first patient, a 36-year-old man, had involvement of the 9th, 10th, 11th, and 12th cranial nerves. The second case was a 53-year-old man with abnormalities of the 5th, 7th, 9th, 10th, and 12th cranial nerves. In both, magnetic resonance imaging revealed a ring-like area of abnormal signal intensity surrounding the carotid artery at the skull base. Carotid angiography was consistent with the suggestion of dissection on the magnetic resonance studies in both cases. The patients recovered without anticoagulation. Internal carotid artery dissection may thus present with multiple cranial nerve palsies, which could be mistaken for an infiltrating tumor of the skull base. Magnetic resonance imaging is useful in identifying the condition.     

Lower cranial nerve palsies due to internal carotid dissection

A 41-year-old man experienced intense headache and neck pain, bruits, and a complete unilateral cranial nerve palsy IX-XII (Collet-Sicard syndrome) after a trivial back trauma. Magnetic resonance imaging and angiography demonstrated features of bilateral internal carotid artery dissection with aneurysm formation at the base of the skull compressing the nerves at the level of the jugular foramen. Severe dysphagia persisted for 1 month but rapidly improved after occlusion of the carotid aneurysm with a detachable balloon.     

Dejerine-Sottas neuropathy with multiple nerve roots enlargement and hypomyelination associated with a missense mutation of the transmembrane domain of MPZ/P0

In a patient affected with a slowly progressive, severe form of Dejerine-Sottas syndrome, symmetric enlargement of cranial nerves and focal hypertrophy of cervical and caudal roots were detected following MRI. Neuropathological features of the sural nerve disclosed a dramatic loss of myelinated fibres, with skewed-to-the-left, unimodal distribution of the few residual fibres, consistent with the diagnosis of congenital hypomyelination neuropathy. Genetic analysis revealed this condition to be associated with a heterozygous G to A transition at codon 167 in the exon 4 of the MPZ/P0 gene causing a Gly138Arg substitution in the transmembrane domain of the mature MPZ/P0 protein. Focal enlargement of the nerve trunks in demyelinating, hereditary motor and sensory neuropathies (HMSN) was previously reported in both asymptomatic and symptomatic cases with root compression, but peculiar to this case is the diffuse involvement of both cranial and spinal nerves. We believe that the relevance of nerve trunk hypertrophy in HMSN is probably underevaluated: therefore MRI investigation of the head and spine should be included in the diagnostic study of selected HMSN patients. Molecular analysis of peripheral myelin genes will help to rule out misdiagnosed cases. Received: 9 January 2002, Received in revised form: 14 March 2002, Accepted: 19 March 2002     

Neurological complications of swine influenza vaccination

The emphasis upon the remarkably large number of cases of Guillain-Barre syndrome which resulted from the 1976 National Swine Influenza immunization program in the U.S.A. has obscured the fact that other neurological complications, involving the central nervous system also occurred. The anatomical distribution of lesions is almost identical with that seen following other types of vaccination: involvement of the brain, cerebellum, optic nerve, cranial nerves and spinal cord occurred with approximately the same frequency.
5 instances of the very rare subacute or chronic, progressive, post-vaccinal encephalopathy are described, a situation which is identical to the subacute and chronic forms of polyradiculoneuropathy. In a number of cases, in particular the myelopathies, a subclinical involvement of peripheral nerves was demonstrated by means of electrodiagnostic studies, illustrating the often overlooked fact that central nervous system involvement will mask peripheral nerve lesions.
The etiological significance of the swine influenza vaccination was overlooked and completely erroneous diagnoses were established in a surprisingly large number of the 26 new cases reported here.