The traA gene of the Enterococcus faecalis conjugative plasmid pPD1 encodes a negative regulator for the pheromone response

Cranial nerve palsies are rare complications of internal carotid artery (ICA) dissections. The aim of this study is to evaluate the incidence of cranial nerve palsies in consecutive patients with ICA dissection and to describe clinical and radiological characteristics and their evolution over time. This study was conducted in 52 consecutive patients with dissection of the ICA. We have analyzed clinical data of patients with cranial nerve palsy as complication of ICA dissection. We defined ICA dissection as angiographic evidence of a string sign, double lumen, or internal flaps or visualization on magnetic resonance imaging (MRI) or computed tomographic scans of an enlarged arterial wall due to the hematoma. Of 52 consecutive patients with ICA dissection 7 had cranial nerve palsies: 2 had an involvement of the Vth cranial nerve and 5 had lower cranial nerve palsies. Five patients totally recovered while 2 did not after a 2 to 10-month period. The frequency of cranial nerve palsies associated with ICA dissection is higher in our study than in those of the literature. Many patients presenting with cranial nerve palsies due to ICA dissection without any ischemic event are probably not referred to stroke units. Angiography is less sensitive than cervical MRI to detect such patients. Cranial nerve palsies could either be due to compression by the enlarged ICA wall or an ischemia of the nerve.     

Multiple myeloma presenting as parasellar syndrome and cranial nerve palsies

Cranial and intracranial locations have been rarely reported in multiple myeloma. Their occurrence as a harbinger of multiple myeloma seems to have a particular significance. In this report, we discuss a case of multiple myeloma presenting as parasellar syndrome and cranial nerve palsies. A 75-year-old woman was admitted to the hospital in June, 1994, with a 3-month history of headache and a 3-week history of diplopia and photophobia. Physical examination revealed right third, fourth and sixth cranial nerve palsies. MRI scan demonstrated a homogeneous, voluminous mass, isointense in T1-weighted images with the cerebral parenchyma and hyperintense in T2-weighted images, occupying the sphenoid sinus and extending within the sella turcica and right cavernous sinus. Lying above the mass and apparently separated from it by a thin rim of hypointensity was a normal pituitary gland. X rays revealed destructive changes of the sella turcica. A minimal disturbance of endocrine function together with a radiologically abnormal pituitary fossa indicated that the primary lesion might lie outside the pituitary fossa. A diagnosis of IgG-kappa type multiple myeloma was made by pertinent laboratory studies. She received local radiation to the intracranial mass (50 Gy) and conventional chemotherapy. Sixteen months after the therapy she is in good health.     

Detection of restricted junctional diversity of peripheral T cells in SLE patients by spectratyping

Three patients presented with unilateral sensori-neural hearing disturbance as the initial symptom of cerebellar tumors: a 19-year-old female with a medulloblastoma (Case 1), a 45-year-old male with a cerebellar low-grade glioma (Case 2), and a 49-year-old female with a cerebellaer hemangioblastoma (Case 3). In Cases 1 and 2, the whole length of the eight cranial nerve was intact according to magnetic resonance imaging and intraoperative findings. In Case 3, the intracerebellar tumor had bulged into the cerebellopontine cistern, compressing the eighth cranial nerve near the brainstem. Auditory evoked brainstem responses showed only the first wave in all three patients, and the following waves could not be discriminated. Unilateral sensori-neural hearing disturbance occurs very rarely in patients with intramedullary cerebellar lesions because the auditory neural pathway is bilaterally innervated. Intramedullary tumors may cause unilateral sensori-neural hearing disturbance by infiltrating or causing edematous changes of the eighth cranial nerve or the cochlear nucleus in the brainstem, or by compressing the nerve in the cistern. The symptoms are the same as those of acoustic neurinoma, so intramedullary cerebellar tumors should be considered in the differential diagnosis of unilateral sensorineural hearing disturbance.     

Mechanisms of efferent neuronal control of the reflex nicitating membrane response in rabbit (Oryctolagus cuniculus)

Efferent mechanisms controlling the nicitating membrane (NM) reflex response to air puff in the albino rabbit were analyzed using stimulation, lesions, and recording techniques. In brief, stimulation of the sixth nerve (abducens) yields short-latency NM extension. Stimulation of the fourth and seventh nerves and the superior cervical ganglion has essentially no effect on the NM. Stimulation of the third nerve causes short-latency retraction of the NM. Lesions and recording data are consistent with this result - the sole efferent neuronal control of NM extension is the sixth cranial nerve and of NM retraction is the third cranial nerve. The NM extension response appears to be mediated by mechanical actions via retraction of the eyeball by the retractor bulbi muscle, and NM retraction appears to result from direct activation of muscle fibers in the NM by the third nerve. The superior cervical ganglion appears to play no role in reflex NM retraction in the rabbit, in contrast to its action in the cat.     

Low incorporation of soleus tendon: a potential diagnostic pitfall on MR imaging

Head pain is one of the main presenting symptoms of internal carotid artery (ICA) dissection, usually in association with ischemic and/or local signs such as Horner's syndrome, lower cranial nerve palsies, or tinnitus. In rare cases, head pain remains isolated and mimics other conditions. We report a patient who suffered isolated prolonged orbital pain as the only sign of intrapetrous ICA dissection. Early recognition of such unusual facial pain may be crucial in decreasing the risk of secondary cerebral or retinal ischemia.     

Relationship among social and intrapersonal risk, alcohol expectancies, and alcohol usage among early adolescents

We evaluated 3 patients with biopsy-proven hypertrophic cranial pachymeningitis apparently unrelated to any systemic disease. Each patient had chronic headache, cranial neuropathy, an elevated ESR, and a mild CSF pleocytosis. Neuro-ophthalmic findings included bilateral sixth nerve palsies in two patients and the third had bilateral optic neuropathies. MR imaging revealed thickened dura that enhanced with Gd-DTPA administration. Histologic examination showed thickened, fibrotic dura with a sterile, chronic, nongranulomatous inflammation. The response to treatment was variable with corticosteroids, immunosuppressive drugs, or radiation. The distinctive MR appearance should help physicians recognize this rarely reported disease.     

Isolated third nerve palsy due to sarcoidosis

We present a 28-year-old man with isolated third nerve palsy as the initial manifestation of sarcoidosis. MRI demonstrated abnormal enhancement of the cisternal portion of the right third nerve. One month after steroid therapy, he showed almost complete recovery with resolution of the contrast enhancement on MRI. This patient illustrates the usefulness of enhanced MRI for detection of cranial nerve lesions of sarcoidosis.     

Hypertrophic cranial pachymeningitis with spinal epidural granulomatous lesion

A 67-year-old woman with a one-year history of tinnitus and headache had multiple cranial nerve palsies of V, VII, VIII, IX, X, XI and spastic paraparesis. She also had a secretory otitis media. Gd-DTPA-enhanced magnetic resonance imaging (MRI) revealed hypertrophy of the dura of the posterior fossa and spinal epidural mass which extended from C7 to T10. A biopsy of the epidural mass showed chronic granulomatous change. These lesions were completely cured with administration of antibiotics. We believe this case of double-lesion of hypertrophic cranial pachymeningitis and spinal epidural granulomatous lesion originated from a bacterial infection secondary to the secretory otitis media.     

Clinical studies of ocular motility disturbances. Part 1. Ocular motility disturbances: causes and incidence

Between January 1985 and December 1994, 500 cases of ocular motility disturbances were encountered in our department; we analyzed these statistically. There were 48 cases of supranuclear palsy (10%), 281 of ocular motor nerve palsies (56%), 19 with myasthenia gravis (4%), 29 with myogenic palsy (6%), 57 with abnormal innervation (11%), and 63 instances of mechanical disturbances (13%). There were more men than women in all groups except myasthenia gravis. Eighty-four ocular motor nerve palsy and 15 blowout fracture patients recovered spontaneously. Spontaneous recovery occurred within 6 months in 80 (93%) patients with ocular motor nerve palsy. The abnormality detection rate, using diagnostic imaging, was high for supranuclear and myogenic palsy and the mechanical disturbance groups, but very low for the ocular motor nerve palsy groups. In 3 supranuclear palsy patients, small infarctions of the brainstem were discovered by magnetic resonance imaging (MRI) but were not found with computed tomography (CT). MRI thus proved useful in detecting abnormalities of brainstem lesions.     

Brainstem lesions and click lateralization in patients with multiple sclerosis

The ability to lateralize dichotic clicks with either interaural time delays (ITD) or interaural level differences (ILD) was tested in seven multiple sclerosis (MS) subjects who had normal audiograms. Along with the psychoacoustical tests, magnetic resonance images (MRI) of the subjects' brainstem were obtained. After matching each MRI section with the corresponding section of a computerized atlas of the brainstem, the parts of the auditory pathway affected by each MS lesion were determined. Of the seven subjects two performed normally with both types of interaural asymmetry and had no brainstem lesions involving the auditory pathway. Two subjects performed normally only with level differences, but perceived all the dichotic clicks with different ITDs in the center of the head; both had lesions involving the trapezoid body. Three subjects could not perform normally with either task, perceiving the clicks to the sides and never in the center for both ITDs and ILDs; all three had unilateral lesions of the lateral lemniscus. A multi-level decision making model is proposed to account for these results.     

Clivus chordoma

Chordomas are slow growing, locally invasive tumors that most commonly present as midline masses in the sacrococcygeal or clival regions. The case presented in this paper demonstrates the typical MRI appearance of a clival chordoma in a patient presenting with classic symptoms of cranial nerve compression and headache. While the exact signal characteristics of these lesions seen on MRI may vary with pathologic subtypes, MRI has proven essential in the diagnosis, surgical planning and post-treatment evaluation of patients with these lesions.     

Triple dose of gadolinium-DTPA and delayed MRI in patients with benign multiple sclerosis

OBJECTIVES: To evaluate whether a triple dose of gadolinium-DTPA (Gd-DTPA) or delayed MRI increase the number, size, and conspicuousness of enhancing lesions in patients with benign multiple sclerosis. METHODS: T1 weighted brain MRI was carried out on 20 patients with benign multiple sclerosis (expanded disability status scale < 3 with a disease duration > 10 years) in two sessions. In the first session, one scan was obtained before and two scans five to seven minutes and 20-30 minutes after the injection of 0.1 mmol/kg Gd-DTPA (standard dose). In the second session, six to 24 hours later, the same procedure was repeated with 0.3 mmol/kg Gd-DTPA (triple dose). RESULTS: Nine enhancing lesions were found in seven patients (35%) using the standard dose of Gd-DTPA. The numbers of enhancing lesions increased to 13 (P = 0.03) and the number of patients with such lesions to eight (40%) on the delayed standard dose scans. On the early triple dose scans, we found 19 enhancing lesions in 10 patients (50%). The number of enhancing lesions was significantly higher (P = 0.01) than that obtained with the early standard dose. The number of enhancing lesions was 18 and the number of "active" patients 11 (55%) on the delayed triple dose scans. The enhancing areas increased progressively from the early standard dose scans to the delayed triple dose scans. The contrast ratios of the lesions detected in early standard dose scans was lower than those of lesions present in the early (P = 0.01) and delayed (P = 0.04) triple dose scans. CONCLUSIONS: More enhancing lesions were detected in patients with benign multiple sclerosis with both delay of MRI and the use of triple dose of Gd-DTPA suggesting that the amount of inflammation in the lesions of such patients is mild and heterogeneous.     

Clinical, neurodiagnostic, and MR findings in children with spinal and brain stem multiple sclerosis

PURPOSE: To describe the clinical, neurodiagnostic, and MR findings in seven children with brain stem and spinal multiple sclerosis. METHODS: Spinal or brain stem multiple sclerosis was diagnosed in seven children between 1986 and 1992. All patients had neurologic and MR examinations as well as neurodiagnostic testing, including spinal fluid analysis and brain stem and auditory evoked potentials. RESULTS: Three children had clinical findings and masslike lesions in the brain stem (two) or spinal cord (one) suggestive of neoplasm, which prompted biopsy (two) or radiation therapy (one). Five of six patients with spinal involvement had cord swelling with increased signal on T2-weighted images over at least three cord segments, and two children had essentially holocord involvement. Three children had normal cranial MR at presentation. CONCLUSIONS: Multiple sclerosis involvement of the brain stem and spinal cord may be associated with extensive swelling and MR signal changes suggestive of neoplasm without typical cerebral white matter abnormalities. Serial clinical and neuroimaging examinations may be necessary to make a definitive diagnosis of multiple sclerosis in children.     

A case of crushing head injury with bilateral abducens and facial nerve palsies

A 20-year-old male was admitted to our hospital suffering from a crushing head injury. At accident, his head had been compressed by the printing machine on both temporal regions. He remained at dull conscious. On admission one hour after the injury, he showed bilateral sixth-nerve and seventh nerve palsies and bleeding from the nose. CT scan showed marked pneumocephalus, traumatic subarachnoid hemorrhage, fluid collections in the bilateral sphenoid sinuses and right mastoid air cells. Bone CT disclosed bilateral temporal bone fractures. MRI did not show cerebral parenchymal damage. He recovered fully conscious at four hours after the injury, but cranial nerve palsies sustained over 30 days after the injury. Bilateral decompression of the facial canal were performed at day 31. At one year after the injury, bilateral abducens nerve palsies and facial nerve palsies recovered incompletely (grade III). The case report and the mechanism of such cranial nerve injuries by low-velocity crushing head injury is described.     

Spontaneous bilateral carotid and vertebral artery dissection presenting as a Collet-Sicard syndrome

Spontaneous dissection of the internal carotid arteries usually presents with unilateral headache, neck pain, focal ipsilateral cerebral ischaemic symptoms and a Horner's syndrome. Lower cranial nerve palsies are only rarely observed. We report a case of carotid and vertebral dissections presenting as a unilateral palsy of the ninth to twelfth cranial nerves (Collet-Sicard syndrome).     

Defective gas mixers, a cause of retro-pollution of medical gas distribution pipelines]

PURPOSE: To study the distribution and mechanism of traumatic injuries to the nerves supplying the eye and muscles protecting the visual apparatus. METHODS: Brain autopsy was carried out in 12 consecutive patients who died within three days after closed head injury. A segment of the brainstem with the entire intracranial portion of nerves II-VII was dissected out in each case and fixed in formalin. The specimens were stripped of the leptomeninges and inspected thoroughly under magnification. RESULTS: Injuries to the nerves were seen in nine subjects. The oculomotor nerve was completely torn off from the midbrain unilaterally in three and bilaterally in two cases. In one patient only a portion of the superficial fibres on the medial aspect of the nerve was ripped out from the brainstem. In two patients the fourth nerve was ruptured. The root of the fifth cranial nerve was contused and the fibres between the brainstem and Gasserian ganglion crushed and separated in one case. Bilateral avulsion of the root of the sixth nerve from the brainstem was found in two cases. The initial segment of the facial nerve was crushed in two subjects. No visible injury to the optic nerves was found. CONCLUSIONS: Cranial nerves related to the visual system are subject to serious injury in a large proportion of cases of severe head trauma resulting from automobile accidents. In the majority of cases damage results from ripping the roots of these nerves out of the brainstem.     

The superior orbital fissure: a microanatomical study

The superior orbital fissure (SOF) is a small (3 x 22 mm), but functionally very important, region. The microsurgical anatomy of the SOF was studied on five adult, formalin-fixed cadavers. The vascular structures of three of them were injected with latex. The SOF contains the third, fourth, and sixth nerves, the ophthalmic branch of the fifth nerve, and the superior orbital vein. It is divided by the two tendons of the lateral rectus muscle: the superior part contains the fourth nerve, the frontal and the lacrimal branches of the ophthalmic division of the fifth nerve, and the superior orbital vein; the inferior part contains the superior and inferior branches of the third, the nasociliary, and the sixth nerves. In regard to surgical access to lesions involving the SOF, the question is often raised as to whether the dissection should be started from the cranial or the orbital side. The following procedure is recommended: 1) frontotemporo-orbital craniotomy; 2) resection of the lesser wing of the sphenoid bone, of the anterior clinoid, and of the superolateral part of the orbital roof and opening of the dura along the Sylvian fissure, with an extension to the frontal lobe and another extension to the temporal lobe; 3) incision of the periorbita in its superolateral part and identification of the frontal nerve; and 4) dissection of the frontal nerve in an anteroposterior direction. The fourth nerve will be found medially and inferiorly to the frontal nerve. The third nerve will be found inferomedially to the frontal nerve in the SOF, and the sixth nerve will be found inferiorly to the inferior branch of the third nerve.     

A risk of developing multiple sclerosis following an outbreak of optic neuritis during a mean follow-up of 4.5 years

OBJECTIVE: The current study was designed to determine the risk of developing clinically definite multiple sclerosis (CDMS) after an isolated idiopathic optic neuritis (ON). PATIENTS AND METHODS: We retrospectively studied 28 patients (range from 18-45 years) who presented a unilateral acute ON between 1 st April and 31 st December. We excluded optic neuropathy of other causes, a previous diagnosis of MS or systemic diseases associated with ON. Patients underwent brain MRI, visual evoked potentials (VEPs), somatosensory evoked potentials (SEPs) and brainstem auditory evoked potentials (BAEPs). The mean interval between ON onset and MRI was 3.1 months. 24 patients were treated with corticosteroids in different ways: oral prednisone (14) and intravenous methylprednisolone (10). Mean duration of the follow-up was 4.5 years in 26 patients. RESULTS: Brain MRI detected white matter areas with increased signal in 10 of 25 patients (40%). Eight (30%) had bilateral anormalities on VEP, while SEPs and BAEPs revealed anormalities in one patient (5.5%). Two out 26 patients (7.7%) developed CDMS; one was treated with oral prednisone and the other with intravenous corticosteroids. No patients with normal MRI developed MS compared to 20% of patients with lesions on initial brain MRI. The only patient with abnormal SEPs and BAEPs did not develop MS. CONCLUSIONS: We found in our population low rate of developing CDMS with higher risk for those ON with abnormalities on initial brain MRI.     

Sudden hearing loss as the initial monosymptom of multiple sclerosis

Hearing loss is an uncommon symptom in multiple sclerosis. We report two patients in whom unilateral sudden hearing loss was the first monosymptomatic manifestation of multiple sclerosis. We confirmed the initial central auditory dysfunction suggested by audiometric findings and brainstem auditory evoked potentials by MRI, which showed a unilateral pontine lesion in one patient and a lesion in the medulla oblongata in the other.     

Adult brainstem gliomas

OBJECTIVE: To evaluate prognostic factors and survival of adult patients with brainstem gliomas. BACKGROUND: Brainstem glioma is a disease found primarily in children, with a median survival of only 9 to 12 months. However, the prognosis and survival of adults with this disease has not been determined with precision. METHODS: We conducted a retrospective analysis of patients older than 16 years at Memorial Sloan-Kettering Cancer Center with histologically proved or presumed brainstem glioma diagnosed between 1989 and 1997. We assessed the effect of gender, age at diagnosis, cranial nerve involvement, duration of symptoms, exophytic component, MRI enhancement, site of disease, treatment, and Karnofsky performance status on survival. RESULTS: Twenty-three patients were identified, but complete information was available in only 19 (12 males and 7 females). Patients ranged in age from 17 to 70 years (median, 40 years). Twelve patients were treated with radiotherapy at diagnosis and seven were observed, three of whom received subsequent radiotherapy. Median survival is 54 months (range, 3 to 98 months) and the 5-year survival is 45%. There was a trend for patients with a higher performance status at diagnosis to have longer survival, but this did not reach statistical significance. Other factors did not affect survival. CONCLUSION: Adults with brainstem gliomas may survive significantly longer than children, suggesting the disease may be less aggressive in adults. Furthermore, some patients with a long duration of symptoms or tectal or cervicomedullary tumors may be managed initially with observation alone.