Lumbar Epidural Cavernous Hemangioma: A Case Report and Review of the Literature.

Pure epidural cavernous hemangioma (ECH) of the spine are rare and account for only 4% of all epidural spinal lesions. We report a case of epidural cavernoma at L3/4 presenting with L4 radiculopathy. Radiological, intraoperative findings and histopathology are presented. We present the case of a 56-year-old man who was admitted with a right L4 radiculopathy including an M4 paresis of the right leg, hypoesthesia L4, and radicular pain. Magnetic resonance imaging (MRI) confirmed an extradural lesion L3/4 partially expanding into the right intervertebral foramen. The lesion had a heterogeneous signal, isointense on T1-weighted and hyperintense on proton density (PD) and T2-weighted images. At surgery, an epidural, ovoid, gray-red, soft mass, lightly adherent to the dura and extending to the right L4 foramen was observed. Findings in the histological examination indicated a cavernous hemangioma without signs of hemorrhage. Symptoms and paresis improved rapidly after surgery. The follow-up MRI showed complete resection of the lesion with no signs of radicular compression. Spinal ECH should be considered as a cause of chronic lumbar radiculopathy with atypical radiological findings. Early diagnosis and total removal of the spinal ECH might prevent hemorrhage and neurological deficits. Fewer than 50 cases of lumbar epidural spinal hemangioma have been reported until today, and our case report is adding valuable knowledge to the existing literature.

The "Eyelet Sign" as an MRI Clue for Inflammatory Brown Syndrome.

BACKGROUND: Brown syndrome is characterized by a restrictive elevation deficit of the affected eye in adduction. Besides the well-known congenital form, different acquired etiologies including inflammation, trauma, and surgery may prevent the superior oblique (SO) tendon from gliding freely through the trochlea on attempted upgaze. We present MRI findings in pediatric and adult patients with inflammatory acquired Brown syndrome. METHODS: Retrospective review of clinical and MRI findings of 6 patients (4 children: median age 8.4 years [range 6.1-8.7]; 2 adults: age 46.4 and 51.1 years). Median follow-up was 23 months (range 1-52). RESULTS: In all 6 patients, orbital MRI demonstrated inflammatory changes of the SO tendon-trochlea complex. A striking feature was circumferential contrast enhancement of the trochlea with central sparing where the tendon passes, reminiscent of an eyelet. In all cases, the motility restriction improved either spontaneously or with systemic anti-inflammatory treatment. Although both adult patients had a history of known seronegative spondyloarthritis, there was no associated systemic condition in the children in our series. CONCLUSIONS: Both in children and in adults, MRI can provide evidence of inflammatory changes located at the trochlea-tendon complex in acquired Brown syndrome here referred to as the "eyelet sign," which may be helpful in confirming the clinical diagnosis and guide appropriate treatment.

Retinal Ganglion Cell Topography in Patients With Visual Pathway Pathology.

BACKGROUND: To investigate and quantify the impact of intracranial lesions at different locations within the visual pathway on the ganglion cell layer-inner plexiform layer (GCL-IPL) complex and the retinal nerve fiber layer (RNFL). METHODS: Patients with intracranial lesions affecting the optic chiasm (Group I) or the optic tract and/or lateral geniculate nucleus (Group II) were included. All patients received kinetic visual field assessment and underwent spectral domain optical coherence tomography. Peripapillary and papillomacular bundle (PMB) RNFL and macular GCL-IPL thickness in 4 perifoveal areas were measured and compared with normal values derived from 52 age-matched healthy control subjects. Z-scores for each parameter of every patient were calculated and compared with the normative data. Z-scores less than -2.0 (e.g., -2.5) were considered as being statistically significant. RESULTS: Twenty-two patients (Group I and II: 13 and 9, respectively) were included. Ten of 13 patients in Group I showed significant binasal GCL-IPL thinning, with associated temporal sector thinning in 8 patients. In Group II, all 9 patients showed significant reduction of the GCL-IPL corresponding to the homonymous visual field defect, but only 4 demonstrated RNFL thinning. Contralateral RNFL thinning within the PMB clinically similar to bow-tie atrophy was evident in all patients in Group II. GCL-IPL and RNFL thinning varied in severity from mild (isolated PMB RNFL thickness reduction) to severe (bilateral asymmetrical reduction of PMB RNFL associated with asymmetric, predominantly nasal reduction of GCL-IPL) in Group I. CONCLUSION: Clinical abnormalities in patients with visual pathway lesions are more likely to demonstrate abnormalities of GCL-IPL than global peripapillary RNFL thickness. However, PMB thickness measurement appears to be a valuable tool to detect abnormalities of the anterior visual pathways. If peripapillary RNFL measurements are performed in such patients, PMB thickness should be considered the most useful quantitative parameter.

The cerebellar nodulus: perceptual and ocular processing of graviceptive input.

Current concepts postulate a decisive role of the cerebellar nodulus in the processing of otolith input. We hypothesized that nodular lesions abolish otolith-perceptual integration, predicting alignment of perceived direction of earth vertical with the z-axis of the head and not with gravity. In an 80-year-old patient with acute heminodular infarction, the subjective visual vertical deviated contralesionally by -21.1° when the patient was upright. After subtracting this offset, perceived vertical closely matched the patient's head orientation when the patient was roll-tilted. Otolith-ocular reflexes remained normal. This is the first report on abolished earth verticality perception in heminodular stroke and underlines the importance of the nodulus in spatial orientation.

The pivotal sign of CANVAS.

A 75-year-old woman complained about insecure gait since age 55. Clinical examination revealed signs of cerebellar ataxia, bilateral vestibulopathy, and peripheral sensory impairment. Sensory nerve action potentials were absent. The visually enhanced vestibulo-ocular reflex (VVOR) was impaired (video on the Neurology(®) Web site at www.neurology.org, figure 1) and the diagnosis of cerebellar ataxia (figure 2) with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) was made.(1) CANVAS is considered to be a recessive disorder with a mean age at onset of 60 years.(2) VVOR impairment is its characteristic clinical sign.(2) It can only be elicited if both smooth-pursuit eye movements and the vestibulo-ocular reflex are deficient. Normally, both are redundant at low head velocities.(2.)

[Not a tropical, but a non the less exotic fever]. / Kein tropisches, aber ein nicht minder exotisches fieber.

We describe a 26-year-old male patient with fever and elevated inflammatory markers of three months duration plus an initial episode of encephalitis. Since intensive search for infectious diseases gave no positive results and ANA and ANCA titers were negative, Still's disease was assumed. But as the patient developed a nodular erythema, polyarteritis nodosa (PAN) was revealed by histopathology. The case underlines, that PAN presents mostly with general symptoms. Therefore, the diagnosis is difficult. An encephalitis is a seldom manifestation of PAN.

Optic nerve sheath meningiomas in patients with neurofibromatosis type 2.

OBJECTIVE: To determine the prevalence of optic nerve sheath meningiomas (ONSMs) in patients with neurofibromatosis type 2 (NF2). METHODS: An observational retrospective case series of 30 consecutive patients with NF2 referred to an academic ophthalmology unit from November 1, 1991, through August 31, 2003. Twenty-six patients were followed up for a mean of 93 months (range, 3-150 months). One individual was lost to follow-up, and 3 had been referred recently. Diagnosis of ONSM was made based on typical neuroradiologic and clinical features in 7 patients and on histologic criteria in 1. RESULTS: Eight of 30 patients harbored unilateral (n = 6) or bilateral (n = 2) ONSMs. Six ONSMs were diagnosed at initial examination, and 4 during follow-up. CONCLUSIONS: There is a strong association between ONSMs and NF2 that parallels the well-known association of optic nerve gliomas with NF1. Physicians should be aware of the possibility that patients with ONSMs may also have NF2.

Reflexions about imaging technique and examination protocol 2. MR-examination protocol.

Adequate imaging protocol is crucial for any imaging technique. As MR is a time consuming examination, the physician serves the patient best, when he/she makes the best choice of sequences, which answer the question of the clinician and provide a definite diagnosis. Although any patient requires an individual protocol, some general rules should be known.