STUDY OBJECTIVE:

During the first phase of the Fundus Photography vs Ophthalmoscopy Trial Outcomes in the Emergency Department study, 13% (44/350; 95% confidence interval [CI] 9% to 17%) of patients had an ocular fundus finding, such as papilledema, relevant to their emergency department (ED) management found by nonmydriatic ocular fundus photography reviewed by neuro-ophthalmologists. All of these findings were missed by emergency physicians, who examined only 14% of enrolled patients by direct ophthalmoscopy. In the present study, we evaluate the sensitivity of nonmydriatic ocular fundus photography, an alternative to direct ophthalmoscopy, for relevant findings when photographs are made available for use by emergency physicians during routine clinical care.

METHODS:

Three hundred fifty-four patients presenting to our ED with headache, focal neurologic deficit, visual change, or diastolic blood pressure greater than or equal to 120 mm Hg had nonmydriatic fundus photography obtained (Kowa nonmydriatic α-D). Photographs were placed on the electronic medical record for emergency physician review. Identification of relevant findings on photographs by emergency physicians was compared with a reference standard of neuro-ophthalmologist review.

RESULTS:

Emergency physicians reviewed photographs of 239 patients (68%). Thirty-five patients (10%; 95% CI 7% to 13%) had relevant findings identified by neuro-ophthalmologist review (6 disc edema, 6 grade III/IV hypertensive retinopathy, 7 isolated hemorrhages, 15 optic disc pallor, and 1 retinal vascular occlusion). Emergency physicians identified 16 of 35 relevant findings (sensitivity 46%; 95% CI 29% to 63%) and also identified 289 of 319 normal findings (specificity 91%; 95% CI 87% to 94%). Emergency physicians reported that photographs were helpful for 125 patients (35%).

CONCLUSION:

Emergency physicians used nonmydriatic fundus photographs more frequently than they performed direct ophthalmoscopy, and their detection of relevant abnormalities improved. Ocular fundus photography often assisted ED care even when results were normal. Nonmydriatic ocular fundus photography offers a promising alternative to direct ophthalmoscopy.

BACKGROUND AND PURPOSE:

Changes at the optic nerve head on DWI in the presence of papilledema have not been systematically studied. The purpose of this study was to evaluate if hyperintensity of ON heads on DWI is associated with papilledema.

MATERIALS AND METHODS:

In this retrospective study, 19 patients (4 men, 15 women; median age, 32 years) with papilledema and 20 control participants (7 men, 13 women; median age, 48 years) who had undergone prior MR imaging of the brain were identified. Two neuroradiologists blinded to the diagnosis independently reviewed the DWI for the presence of hyperintense signal at the ON head of each eye. If present, they graded the signal as mild or prominent. Groups with and without papilledema were compared for the prevalence of ON head hyperintensity by using the Fisher exact test, with analyses performed both for groups of patients and for individual eyes. Presence of ON head hyperintensity was also studied as a function of Frisen papilledema grade on fundoscopy, when available.

RESULTS:

Hyperintensity of the ON heads on DWI was significantly associated with papilledema (P = .001). For the 2 readers, hyperintensity at both ON heads was 26.3% and 42.1% sensitive and 100% specific in the detection of papilledema. The presence of unilateral ON head hyperintensity was not specific for papilledema and was invariably graded as mild when seen in the control group. Patients with higher papilledema grades had a higher prevalence of hyperintensity at the ON heads.

CONCLUSIONS:

Hyperintensity of the ON heads on DWI can serve as a useful imaging marker for papilledema, especially if bilateral. Its absence, however, does not exclude papilledema.

A 21-year-old man presented to his local emergency department with 5 days of headache, which was dull, occipital, bilateral, nonthrobbing, and progressively worsening. It was associated with mild fever, photophobia, and neck pain and stiffness. He had no history of headache, chronic illness, recent vaccinations, cutaneous rash, cough, diarrhea, arthralgia, or myalgia. He was from Ecuador and had been living in the United States for less than 1 year. He had been incarcerated while in Ecuador. Sublingual temperature on admission was 102.6°F. Other vital signs were within normal limits. On physical examination, he appeared thin but not cachectic. He had meningismus and photophobia, but no papilledema and his mental status was alert and attentive. There were no focal neurologic deficits. CSF contained red blood cells: 24 × 10(3)/μL; white blood cells: 85/μL (lymphocytic predominant); protein: 128 mg/dL; and glucose: 48 mg/dL (CSF/serum glucose ratio = 0.53). CSF Gram stain and cultures, PPD test, and blood and urine cultures were all negative. CT scan of the head on day of admission was entirely normal. MRI without gadolinium contrast showed a single punctate T2 hyperintensity in the left frontal periventricular white matter. Chest radiograph was clear. He received empiric vancomycin, ceftriaxone, and acyclovir. Corticosteroids were not given. The patient did not improve with antibiotics and continued to be intermittently febrile. On day 5, he became abruptly more somnolent, then comatose, opening eyes only to pain, his pupils were 5 mm and reactive, he had intact brainstem reflexes, withdrawing both arms and legs. Emergent head CT showed development of hydrocephalus and a ventriculoperitoneal shunt was emergently placed. The neurologic examination did not improve after shunt placement, and repeat head CT showed increased hydrocephalus with bilateral cerebral infarcts. On day 11, he was transferred to Columbia University Medical Center for intensive care. He was febrile and comatose. He did not open his eyes to pain, pupils were 7 mm minimally reactive, brainstem reflexes were intact, and he exhibited extensor posturing to pain. Mannitol was given, corticosteroid therapy was started, and an extraventricular drain was placed. The next day, his right pupil was 8 mm and nonreactive. MRI showed diffuse contrast enhancement of the arachnoid, extensive infarction of basal ganglia, midbrain, and pons, and small ring-enhancing lesions in the cerebellum (figure 1, A-D). Repeat lumbar puncture showed red blood cells: 550 × 10(3)/μL; white blood cells: 250/μL (14% neutrophils, 80% lymphocytes, 6% monocytes); protein: 65 mg/dL; and glucose: <10 mg/dL (CSF/serum glucose ratio = 0.08). CSF testing for Cryptococcus and toxoplasmosis was negative. CSF acid fast bacilli (AFB) smear was negative ×2, and CSF nucleic acid amplification test was also negative for tuberculosis. Serum HIV test was negative. Not until 14 days after initial presentation and 3 days after transfer to the intensive care unit was antituberculosis therapy finally started, because the pattern of infarcts on the MRI suggested basilar meningitis and he had not improved on broad-spectrum antibiotics. That same day, the first sputum AFB smear was positive, as were all succeeding daily sputum AFB smears. Tuberculosis nucleic acid amplification was positive from the sputum, but persistently negative from the CSF. Daily portable chest radiographs had been normal (read as likely atelectasis), but chest CT showed dense consolidations in the left lung and diffuse micronodular opacities throughout both lungs. Two days later, only 21 days after the onset of his headache, the patient died of cardiopulmonary arrest secondary to transtentorial cerebral herniation. Thirteen days later, the CSF culture became positive for Mycobacterium tuberculosis sensitive to streptomycin, isoniazid, ethambutol, rifampin, and pyrazinamide.

To investigate the clinical characteristics of Leber hereditary optic neurology (LHON) patients with different primary site mutation.Four hundred and fourteen patients with optic neuropathy were divided into three groups: clinically diagnosed LHON group (group A), probable LHON group (group B), optic neuropathy of unknown reason group (group C). Visual acuity (VA), colour vision, Intraocular pressure (IOP), virual field and visual evoked potential (VEP) were tested for all the patients. Some (64 cases) had optical coherence tomography (OCT) measurement. Mutations of mtDNA were detected for all the groups, and clinical analysis were carried out emphatically in the patients with the 11778 mutation confirmed by gene assessment. T paired test was used to evaluate two group patients of different Mitochondrial DNA mutation.Gene mutations were found in 215 of the 414 patients (52%). Approximately 93% (199/255) of the patients were caused by the common primary mutations (11778, 14484, 3460 mutation), in which 100% mutation (106/106) in group A, 65% (91/139) in group B, and 11% (18/169) in group C. No cases were diagnosed with confirmed LHON in the patients with unilateral optic neuropathy. Fundus examination in 334 eyes of 167 cases showed pseudo papilledema (54 eyes), normal (67 eyes), pale disc or plae on the teperal side of the optic disc (213 eyes). On the basis data of OCT from 64 patients and 84 normal person, RNFL was found thickening at the early stage and thinning gradually at the later stage in the LHON patients. But, the RNFL thickness of patients with 1-2 years history was not significantly different from the patients with over 2 years history(P = 0.051), and there was no difference among the patients with different mitochondrial DNA mutations. The initial mean VA of patients with the 14484 mutation and 11778 mutation were 3.6 ± 0.65, 3.75 ± 0.54 (t = 0.536, P > 0.05), but the follow-up VA were 4.29 ± 0.55 (t = 4.034, P < 0.001) and 3.93 ± 0.49 respectively (t = 1.857, P > 0.05).The symptoms and fundus manifestation were similar in the LHOH patients with different primary site muation. Gene mutation analysis is helpful to assess the prognosis of visual acuity.

OBJECTIVES:

To report values of optic nerve sheath diameter (ONSD) and optic disc elevation (ODE) obtained with optic nerve sonography (US) in the diagnosis and monitoring of treatment efficacy in an adult with idiopathic intracranial hypertension (IIH).

METHODS:

Serial measurements of the ONSD and ODE using B mode US were performed in a 45-years-old woman with IIH before and during after treatment with acetazolamide and diet.

RESULTS:

At first evaluation US showed a significantly enlarged ONSD (.68 cm right; .66 cm left side) and the presence of increased ODE (.1 cm right; .15 cm left side). Post-punctural assessments showed a bilateral decrease of ONSD (.58 cm right; .58 cm left side), without changes in ODE values. After 12 months of treatment with acetazolamide and diet ODE completely normalized (0 cm on both sides). ODE values correlated directly with ONSD, and both ODE and ONSD values correlated directly with BMI. Correlations were statistically significant. ONSD changes occurred rapidly after the lumbar puncture, whereas the papilloedema required longer to reduce.

CONCLUSIONS:

US of ONSD and ODE was useful to support the diagnosis of IIH and to monitor the efficacy of diet and pharmacological treatment. Further studies are required to evaluate whether this promising technique may be considered a reliable and accurate method to longitudinally evaluate patients with increased ICP secondary to IIH.

Hydrocephalus is one of the most common complications of tuberculous meningitis. The present study evaluated the incidence, predictive factors and impact of hydrocephalus on overall prognosis of tuberculous meningitis.In a prospective cohort study, all patients fulfilling the inclusion criteria of tuberculous meningitis underwent clinical and cerebrospinal fluid evaluation, together with magnetic resonance imaging of the brain. Patients were treated with antituberculosis drugs and dexamethasone. Follow up neuroimaging was done after 6 months. Hydrocephalus was assessed using Evan's index.Of 80 patients with tuberculous meningitis, 52(65%) had hydrocephalus at presentation. During follow up, 8 new patients developed hydrocephalus. Factors associated with hydrocephalus included advanced stage of disease, severe disability, duration of illness > 2 months, diplopia, seizures, visual impairment, papilledema, cranial nerve palsy, hemiparesis, CSF total cell count > 100/cu.mm, CSF protein > 2.5 g/l. Neuroimaging factors that were significantly associated with hydrocephalus included basal exudates, tuberculoma and infarcts. Multivariate analysis revealed visual impairment, cranial nerve palsy and the presence of basal exudates as significant predictors of hydrocephalus. In 13 patients, with early tuberculous meningitis, there was complete resolution of hydrocephalus. Hydrocephalus was significantly associated with mortality and poor outcome.Hydrocephalus occurs in approximately two-third of patients with tuberculous meningitis and has an unfavorable impact on the prognosis. Hydrocephalus in early stages of tuberculous meningitis may resolve completely.

ABSTRACT:

: An 18-year-old man with gliomatosis cerebri (GC) developed tumor infiltration of the optic chiasm and right optic nerve including the optic disc. Although papilledema often is seen with GC, tumor invasion of the optic nerve head is observed.

Cryptococcal induced visual loss is a devastating complication in survivors of cryptococcal meningitis (CM). Early detection is paramount in prevention and treatment. Subclinical optic nerve dysfunction in CM has not hitherto been investigated by electrophysiological means. We undertook a prospective study on 90 HIV sero-positive patients with culture confirmed CM. Seventy-four patients underwent visual evoked potential (VEP) testing and 47 patients underwent Humphrey's visual field (HVF) testing. Decreased best corrected visual acuity (BCVA) was detected in 46.5% of patients. VEP was abnormal in 51/74 (68.9%) right eyes and 50/74 (67.6%) left eyes. VEP P100 latency was the main abnormality with mean latency values of 118.9 (±16.5) ms and 119.8 (±15.7) ms for the right and left eyes respectively, mildly prolonged when compared to our laboratory references of 104 (±10) ms (p<0.001). Subclinical VEP abnormality was detected in 56.5% of normal eyes and constituted mostly latency abnormality. VEP amplitude was also significantly reduced in this cohort but minimally so in the visually unimpaired. HVF was abnormal in 36/47 (76.6%) right eyes and 32/45 (71.1%) left eyes. The predominant field defect was peripheral constriction with an enlarged blind spot suggesting the greater impact by raised intracranial pressure over that of optic neuritis. Whether this was due to papilloedema or a compartment syndrome is open to further investigation. Subclinical HVF abnormalities were minimal and therefore a poor screening test for early optic nerve dysfunction. However, early optic nerve dysfunction can be detected by testing of VEP P100 latency, which may precede the onset of visual loss in CM.

PURPOSE:

We describe the clinical, neuroimaging and pathological features and therapeutic outcome in a large cohort of 39 patients with tumefactive demyelination.

MATERIALS AND METHODS:

A retrospective audit of 39 patients with 'tumefactive demyelination' was performed. The demographic, clinical, MR imaging and pathological details were reviewed.

RESULTS:

The clinical course was monophasic (n = 22) or relapsing-remitting (n = 17). Common neurological manifestations at presentation included hemiparesis - 27; ataxia - 11; vomiting - 10; headache -9; ophthalmoplegia - 7; seizure - 5; impaired vision - 4; aphasia - 4; visual field defects - 3; papilloedema - 5; extrapyramidal - 5; intellectual decline - 5; behavioural disturbances - 3; altered sensorium - 5. MRI revealed fronto-parietal lesions, which were isolated in 14 (36%) patients. Moderate perilesional oedema and/or mass effect was noted in 12 (30.8%) patients. Post-contrast MR sequences revealed partial ring enhancement in 15, complete ring in seven, patchy enhancement in six, uniform enhancement in two and lack of enhancement in nine cases. Clinical and MR characteristics did not help distinguish between monophasic and relapsing-remitting subgroups. In the monophasic group, 53.8% had complete recovery, while 38.5% had partial improvement (follow-up duration, 8.31 ± 9.3 months). In the relapsing-remitting subgroup, the median time to relapse was 4 months (n = 12, follow-up, 37.8 ± 39.4 months). Patients with monophasic course or single relapse received steroids. Patients with more than one relapse received cyclophosphamide (2), mycophenolate (1), azathioprine (1) or methotrexate (1).

CONCLUSIONS:

A high proportion of cases of tumefactive demyelination follow a relapsing course, thus necessitating a long-term follow-up. MRI, although helpful in diagnosis, does not predict monophasic or relapsing-remitting course. Guidelines for the management of acute episodes and prevention of relapses are required.

We believe that there is a need to increase awareness, particularly among foundation year doctors, of the importance of performing a full neurological examination, including ophthalmoscopy, in medical inpatients. Following a serious unexpected incident (missed papilloedema), we implemented a multifaceted intervention, including ensuring greater availability of equipment for neurological/ ophthalmological assessment, education and curriculum redesign in two large teaching hospitals in the UK.Following the results of our initial intervention, we introduced a patient assessment scoring system to evaluate patient recollection of the completeness of neurological examination by medical staff in the two Trusts over a four-month period.Of the 93 patients referred to neurology during this period, 33% could not recollect being examined with a tendon hammer and 48% said they had not been examined with an ophthalmoscope. In contrast, the majority (95.7%) remembered the use of a stethoscope in their examination. The data were fed back to medical staff which resulted in greater awareness of the importance of a complete neurological examination. No further adverse incidents of missed papilloedema were reported in the following 12 months, although it would be premature to state that this situation has been resolved.A patient assessment score can be used by medical staff to raise awareness of the importance of a complete neurological examination from referring physicians.