Isolated trochlear nerve palsy secondary to dural carotid-cavernous sinus fistula.

Ophthalmoplegia associated with dural carotid-cavernous sinus fistula typically involves the third, fourth, and sixth cranial nerves. Occasionally, isolated palsy of the oculomotor or abducens nerve is noted. We report a patient with bilateral dural carotid-cavernous sinus fistulas who presented with an isolated trochlear nerve palsy.

Parésie trochléaire transitoire comme signe neurologique inaugural d'une panartérite noueuse [Transient trochlear nerve palsy as the presenting neurological sign of panarteritis nodosa]

INTRODUCTION: Panarteritis nodosa (PAN) is a systemic vasculitis affecting small and medium-sized arteries. Neuro-ophthalmological complications of PAN are rare but numerous, and may affect the eye, the visual and the oculomotor pathways. Such complications occur mainly in patients previously diagnosed with PAN. OBSERVATION: A 51-year-old woman presented with an isolated right trochlear (IV) palsy, in the setting of headaches and fluctuating fever of unknown etiology. Erythrocyte sedimentation rate was 13 mm and full blood cell count was normal. Previous chest X-ray and blood studies were negative for an infection or inflammation. Orbital and cerebral CT scan was normal. Spontaneous recovery of diplopia ensued over four days. Two days later, paresthesia and sensory paresis of the dorsal portion of the left foot were present. Lumbar puncture revealed 14 leucocytes (76 percent lymphocytes) with elevated proteins, but blood studies and serologies were negative. A diagnosis of undetermined meningo-myelo-radiculoneuritis was made. Because of a possible tick bite six weeks previously the patient was empirically treated with 2 g intravenous ceftriaxone for 3 weeks. Fever rapidly dropped. Six weeks after the onset of diplopia, acute onset of blindness in her right eye, diffuse arthralgias and fever motivated a new hospitalization. There was a central retinal artery occlusion of the right eye. Blood studies now revealed signs of systemic inflammation (ESR 30 mm, CRP 12 mg/L, ANA 1/80, pANCA 1/40, leucocytosis 12.4 G/L, Hb 111 g/L, Ht 33 percent). Biopsy of the left sural nerve revealed arterial fibrinoid necrosis. A diagnosis of PAN was made. CONCLUSIONS: Transient diplopia can be the heralding symptom of a systemic vasculitis such as PAN, giant cell arteritis and Wegener granulomatosis. In this patient the presence of accompanying systemic symptoms raised a suspicion of systemic inflammation, but the absence of serologic and imaging abnormalities precluded a specific diagnosis initially. A few weeks later, the presence of a second ischemic event (retinal) and positive blood studies led to a further diagnostic procedure. Oculomotor and abducens palsies have rarely been reported in association with PAN. We report the first case of trochlear nerve paresis as the inaugural neurological sign of PAN. This case highlights the importance of considering inflammatory systemic disorders in patients with acute diplopia particularly when they are young, lack vascular risk factors or cause, and complain of associated systemic symptoms.

Isolated IVth (trochlear) nerve palsy due to basilar artery dolichoectasia.

BACKGROUND: Dolichoectasia (elongation, dilatation and tortuosity) of the basilar artery can cause an isolated cranial neuropathy. The trigeminal nerve and facial nerve are most frequently affected. Dysfunction of one of the ocular motor cranial nerves due to basilar artery dolichoectasia is uncommon, and an isolated IVth (trochlear) nerve palsy has not been previously described in the literature. HISTORY AND SIGNS: Two men, ages 70 and 59 years, respectively, presented with vertical diplopia due to a IVth nerve palsy. In one patient, the onset of the IVth nerve palsy was painless and gradual and in the other patient, the onset was acute and associated with periorbital pain. Neuroimaging in both patients revealed pathological tortuosity of the basilar artery around the midbrain and displacement of the artery toward the side of the affected trochlear nerve. THERAPY AND OUTCOME: The patients were observed clinically. One patient had gradual worsening of his palsy for three and one-half years then suffered a stroke. The second patient whose IVth nerve palsy had an acute onset experienced spontaneous resolution of his palsy but later developed dysfunction of other cranial nerves. CONCLUSIONS: Basilar artery dolichoectasia should be considered in the differential diagnosis of an isolated IVth nerve palsy. The clinical course may be variable, and the prognosis is not always benign.

Fourth cranial nerve palsy and brown syndrome: two interrelated congenital cranial dysinnervation disorders?

Based on neuroimaging data showing absence of the trochlear nerve, congenital superior oblique palsy is now classified as a congenital cranial dysinnervation disorder. A similar absence of the abducens nerve is accompanied by misinnervation to the lateral rectus muscle from a branch of oculomotor nerve in the Duane retraction syndrome. This similarity raises the question of whether some cases of Brown syndrome could arise from a similar synkinesis between the inferior and superior oblique muscles in the setting of congenital superior oblique palsy. This hypothesis has gained support from the confluence of evidence from a number of independent studies. Using Duane syndrome as a model, we critically review the accumulating evidence that some cases of Brown syndrome are ultimately attributable to dysgenesis of the trochlear nerve.

Results of Anterior Tucking of the Superior Oblique Muscle Tendon in Bilateral Fourth Nerve Palsy.

Background: Bilateral fourth nerve palsy is characterised by excyclotorsion, which can be corrected by reinforcement of the anterior tendon fibres of the superior oblique muscle. Patients and Methods: A retrospective study of 40 consecutive patients with bilateral acquired fourth nerve palsy operated by a selective tuck of the anterior portion of the superior oblique tendon between 1994 and 2012 was undertaken. Horizontal, vertical and torsional deviations were measured in 9 diagnostic positions of gaze and the field of binocular single vision was evaluated with the Harms tangent screen. Postoperative follow-ups took place at 1 week, 6 months, and ≥ 3 years. Results: Preoperative mean excyclotorsion was 9° in the primary position and 15° in downgaze. These values decreased to 2° and 5° 6 months after surgery, and 2.5° and 6° at ≥ 3 years. Immediate post-operative incyclotorsion in upgaze (28 patients) and Brown syndrome (15 patients) regressed spontaneously. The median score of field of binocular single vision improved from 4 % preoperatively to 76 % postoperatively. Conclusions: The selective tuck of the anterior tendon fibers of the superior oblique tendon enables an efficient and long-lasting correction of the ocular torsion induced by bilateral trochlear palsy.

Heerfordt syndrome with unilateral facial nerve palsy: a rare presentation of sarcoidosis.

BACKGROUND: Heerfordt syndrome is rare and is characterized by fever, uveitis, parotid gland enlargement, and facial nerve palsy. We hereby present a case of Heerfordt syndrome with unilateral facial nerve palsy as a presentation of sarcoidosis. HISTORY AND SIGNS: A 29-year-old male patient from Sri Lanka presented with eye redness OU, blurred vision OD, fever, headache, night sweat, fatigue, and weight loss (5 kg over 1 month). Examination revealed mild anterior uveitis OU, mild vitritis OD, fundus whitish lesions OU, left otalgia, taste disorders, bilateral parotid gland enlargement, and left facial nerve palsy. Work-up for infection or tumour was negative. Chest computed tomography and transbronchial lymph node biopsy set the diagnosis of sarcoidosis. THERAPY AND OUTCOME: The patient recovered completely within 2 months under therapy with prednisone and azathioprine. One year after onset of treatment, no recurrence was noted. CONCLUSIONS: Heerfordt syndrome is a rare manifestation of neurosarcoidosis and has to be included in the differential diagnosis of facial nerve palsy.

Patterns of extraocular muscle weakness in vasculopathic pupil-sparing, incomplete third nerve palsy.

OBJECTIVE: To determine the pattern of extraocular muscle (EOM) paresis in incomplete vasculopathic third nerve palsies (3NP) that have normal pupillary function. METHODS: A retrospective study in a private practice and academic neuro-ophthalmic practice. Patients diagnosed with vasculopathic 3NP within 4 weeks of symptom onset were identified. The chart of each patient was reviewed to determine pupillary function and the pattern and degree of EOM and levator palpebrae paresis at the time of presentation. RESULTS: Of 55 patients with vasculopathic 3NP, 42 (76%) had normal pupillary function. Of these 42, 23 (55%) demonstrated an incomplete EOM palsy, defined as partially reduced ductions affecting all third nerve-innervated EOMs and levator (diffuse pattern) or partially reduced ductions that involved only some third nerve-innervated EOMs and levator (focal pattern). Twenty (87%) of these 23 patients showed a diffuse pattern of paresis; only three (13%) showed a focal pattern of paresis, one that affected only the superior rectus and levator muscles (superior division weakness). CONCLUSIONS: Based on our series, most patients with EOM/levator involvement in pupil-sparing, incomplete 3NP of vasculopathic origin have a diffuse pattern of paresis. In contrast, our review of the literature suggests that pupil-sparing 3NP of aneurysmal origin usually have a focal pattern of paresis. We propose that distinguishing these two patterns of EOM paresis may be helpful in differentiating between vasculopathic and aneurysmal 3NP. Future studies will be needed to confirm the clinical utility of this hypothesis.

Left recurrent laryngeal nerve palsy associated with silicosis.

Left recurrent laryngeal nerve palsy usually results from invasion or compression of the nerve caused by diseases localized within the aortopulmonary window. This study reports the case of a 76-yr-old male with vocal cord paralysis due to lymph node involvement by silicosis. This rare entity was identified by video-mediastinoscopy, which revealed a granulomatous and fibrosed recurrent lymph node encasing the nerve. The nerve was dissected and released from scar tissues. Progressive clinical improvement was observed followed by total and durable recovery of the voice after 15 weeks follow-up.

Long-term prognosis in patients with vasculopathic sixth nerve palsy.

PURPOSE: To better define the long-term prognosis in patients with a vasculopathic sixth nerve palsy (6NP), specifically addressing the degree of recovery and incidence of recurrent similar episodes. DESIGN: Observational case series. METHODS: Retrospective chart review. SETTING: An outpatient neuroophthalmic practice. STUDY POPULATION: Patients with one or more vascular risk factors and an acute, isolated 6NP that spontaneously recovered. OBSERVATION PROCEDURE: Information regarding resolution of the 6NP, subsequent vascular events and recurrent ocular motor nerve palsy was obtained from chart review of follow-up clinic visits, mailed questionnaires and telephone interviews. The duration of follow-up ranged from 2 to 13 years. MAIN OUTCOME MEASURES: Resolution of 6NP (complete or incomplete) and incidence of recurrent ocular motor nerve palsy. RESULTS: Fifty-nine patients were identified with a mean age of 65.3 years +/- 11.6 (range 34-90 years). Fifty-one patients (86%) experienced complete resolution of their first episode of vasculopathic 6NP and eight patients (14%) had incomplete resolution. A subsequent episode of ocular motor mononeuropathy occurred in 18 of 59 (31%) patients. The number of recurrences ranged from one (in 14 patients) to four (in one patient). There was no association between any risk factor and recurrence of ocular motor nerve palsy. Similarly, incomplete resolution of the vasculopathic 6NP was not associated with any risk factor. CONCLUSIONS: Patients with a vasculopathic 6NP usually have complete resolution of their ophthalmoplegia, but nearly one third of patients in our study later experienced at least one episode of recurrent vasculopathic ocular motor nerve palsy.

Absence of the fourth cranial nerve in congenital Brown syndrome.

PURPOSE: To elucidate the aetiology of congenital Brown syndrome. METHODS: Four consecutive patients diagnosed with unilateral congenital Brown syndrome had a comprehensive standardized ocular motility examination. Any compensatory head posture was measured. Brain magnetic resonance imaging (MRI) with regard for the IV cranial nerve (CN) was performed in all patients. Orbital MRI was performed in 2/4 patients, with images acquired in eight directions of gaze and superior oblique (SO) muscle areas compared. RESULTS: CN IV could not be identified bilaterally in two patients, but was absent only on the side of the Brown syndrome in the two other patients. On the normal side, orbital MRI revealed a smaller SO muscle area in upgaze than in downgaze, demonstrating normal actions of this muscle. On the side of the Brown syndrome, the SO area remained the same in upgaze and in downgaze and approximately symmetric to the area of SO in downgaze on the normal side. CONCLUSIONS: These cases add further anatomical support to the theory of paradoxical innervation in congenital Brown syndrome. CN IV was absent in two patients on the side of the Brown syndrome, but without muscle hypoplasia. SO muscle size did not vary in up- and downgaze, which we interpreted as a sign of constant innervation through branches of CN III.

Lymphomatoid granulomatosis in a renal transplant patient.

Lymphomatoid granulomatosis is a rare angiocentric and angiodestructive pulmonary angiitis considered as a variant of the lymphoproliferative disorder group. Patients with organ transplantation are at an increased risk for post-transplant lymphoproliferative disorders secondary to their immunosuppression. However, lymphomatoid granulomatosis has rarely been described in patients with renal transplantation. It often presents with severe pulmonary signs. We describe a case whose initial presentation was an isolated VIth nerve palsy. We review the radiological and pathological findings and discuss the etiopathogenesis and therapeutic options of this particular lymphoproliferative disorder. With careful and stepwise reduction in her immunosuppression, our patient showed a complete disappearance of her lymphomatoid granulomatosis, and she is clinically well more than 3 years after the diagnosis, with good kidney function.

Surgical approach for total hip arthroplasty: direct lateral or posterior?

OBJECTIVE: To determine the risks of prosthesis dislocation, postoperative Trendelenburg gait, and sciatic nerve palsy after a posterior approach compared to a direct lateral approach for adult patients undergoing total hip arthroplasty (THA) for primary osteoarthritis (OA). METHODS: Medline, Embase, CINHAL, and Cochrane databases were searched until August 2003. All published trials comparing posterior and direct lateral surgical approaches to THA in adults with a diagnosis of primary hip osteoarthritis were collected. Retrieved articles were assessed independently for their methodological quality. RESULTS: Four prospective cohort studies involving 241 participants met the inclusion criteria. Regarding dislocation rate, no significant difference between posterior and direct lateral surgical approach was found (relative risk 0.35). The presence of postoperative Trendelenburg gait was not significantly different between surgical approaches. The risk of nerve palsy or injury was significantly higher with the direct lateral approach (relative risk 0.16). However, there were no significant differences when comparing this risk nerve by nerve, in particular for the sciatic nerve. Of the other outcomes considered, only the average range of internal rotation in extension of the hip was significantly higher (weighted mean difference 16 degrees ) in the posterior approach group (mean 35 degrees, SD 13 degrees ) compared to the direct lateral approach (mean 19 degrees, SD 13 degrees ). CONCLUSION: The quality and quantity of information extracted from the trials performed to date are insufficient to make a firm conclusion on the optimum choice of surgical approach for adult patients undergoing primary THA for OA.

Management of diplopia secondary to neurosurgical injury of the orbital roof

BACKGROUND: Diplopia related to neurosurgical procedures is often consecutive to oculomotor nerve lesions. We hereby report an oculomotor dysfunction secondary to an orbital roof effraction and its treatment. HISTORY AND SIGNS: Following surgery for a left anterior communicating artery aneurysm, a 45-year-old woman reported vertical diplopia associated with a left orbital hematoma. The diagnosis of third cranial nerve palsy was excluded by orbital imaging which revealed an orbital roof defect with incarceration of the levator palpebrae and superior rectus. THERAPY AND OUTCOME: As neurosurgeons advised against muscle adhesiolysis, diplopia was corrected by a two-step procedure on the oculomotor muscles. We first corrected horizontal and torsional deviations by operating on the healthy eye, before correcting the vertical deviation on the fellow eye. This two-step extraocular muscle surgery allowed restoration of binocular single vision in a useful field of gaze. CONCLUSIONS: Diplopia can occur as a rare orbital complication during neurosurgical procedures. Surgery of extraocular muscles can provide good functional results

Carotid artery stenting compared with endarterectomy in patients with symptomatic carotid stenosis (International Carotid Stenting Study): an interim analysis of a randomised controlled trial.

BACKGROUND: Stents are an alternative treatment to carotid endarterectomy for symptomatic carotid stenosis, but previous trials have not established equivalent safety and efficacy. We compared the safety of carotid artery stenting with that of carotid endarterectomy. METHODS: The International Carotid Stenting Study (ICSS) is a multicentre, international, randomised controlled trial with blinded adjudication of outcomes. Patients with recently symptomatic carotid artery stenosis were randomly assigned in a 1:1 ratio to receive carotid artery stenting or carotid endarterectomy. Randomisation was by telephone call or fax to a central computerised service and was stratified by centre with minimisation for sex, age, contralateral occlusion, and side of the randomised artery. Patients and investigators were not masked to treatment assignment. Patients were followed up by independent clinicians not directly involved in delivering the randomised treatment. The primary outcome measure of the trial is the 3-year rate of fatal or disabling stroke in any territory, which has not been analysed yet. The main outcome measure for the interim safety analysis was the 120-day rate of stroke, death, or procedural myocardial infarction. Analysis was by intention to treat (ITT). This study is registered, number ISRCTN25337470. FINDINGS: The trial enrolled 1713 patients (stenting group, n=855; endarterectomy group, n=858). Two patients in the stenting group and one in the endarterectomy group withdrew immediately after randomisation, and were not included in the ITT analysis. Between randomisation and 120 days, there were 34 (Kaplan-Meier estimate 4.0%) events of disabling stroke or death in the stenting group compared with 27 (3.2%) events in the endarterectomy group (hazard ratio [HR] 1.28, 95% CI 0.77-2.11). The incidence of stroke, death, or procedural myocardial infarction was 8.5% in the stenting group compared with 5.2% in the endarterectomy group (72 vs 44 events; HR 1.69, 1.16-2.45, p=0.006). Risks of any stroke (65 vs 35 events; HR 1.92, 1.27-2.89) and all-cause death (19 vs seven events; HR 2.76, 1.16-6.56) were higher in the stenting group than in the endarterectomy group. Three procedural myocardial infarctions were recorded in the stenting group, all of which were fatal, compared with four, all non-fatal, in the endarterectomy group. There was one event of cranial nerve palsy in the stenting group compared with 45 in the endarterectomy group. There were also fewer haematomas of any severity in the stenting group than in the endarterectomy group (31 vs 50 events; p=0.0197). INTERPRETATION: Completion of long-term follow-up is needed to establish the efficacy of carotid artery stenting compared with endarterectomy. In the meantime, carotid endarterectomy should remain the treatment of choice for patients suitable for surgery. FUNDING: Medical Research Council, the Stroke Association, Sanofi-Synthélabo, European Union.

Infection of the central nervous system caused by varicella zoster virus reactivation: a retrospective case series study.

BACKGROUND: Recent data suggest that varicella zoster virus (VZV)-associated complications of the central nervous system (CNS) are more common and diverse than previously thought. The main purpose of this article is to describe the clinical characteristics and the outcome of patients suffering from meningitis and encephalitis caused by VZV reactivation. METHODS: A retrospective case study of adult patients (≥16 years old) diagnosed with a VZV reactivation in the CNS was performed. The cases were identified by a qualitative PCR DNA assay of the cerebrospinal fluid (CSF) at the Regional Hospital of Lugano between January 1, 2003 and July 31, 2010. RESULTS: Eleven out of 519 CSF samples (2.1%), submitted from patients with a clinical diagnosis of viral meningitis or encephalitis, were positive for VZV. A vesiculo-pustular skin eruption was observed in only five patients (45%). In six cases (55%), a systemic inflammatory syndrome was absent. The clinical outcome was favorable in eight patients (73%). Only one out of 11 patients (9%) died. The four patients with encephalitis had a less favorable prognosis: one patient recovered without residual neurological sequelae; two had a chronic neuropsychological handicap, speech difficulties, facial nerve palsy, and focal seizures; one patient died. We estimated an annual incidence rate of VZV infection of the CNS of 1.02/100 000 inhabitants for southern Switzerland. CONCLUSIONS: Screening of CSF for VZV by PCR is recommended for all patients with encephalitis and for those with viral meningitis of unclear origin in order to better target antiviral treatment.