Concurrent sino-orbital aspergillosis and cerebral nocardiosis.

A 79-year-old man with myelodysplastic syndrome developed a right optic neuropathy with optic disc edema and intractable periocular pain, one month after undergoing removal of a gangrenous gallbladder. Although results of a temporal artery biopsy were negative, he was treated with prednisone for presumed temporal arteritis. Attempts at tapering the prednisone dose led to recurrence of periocular pain. On neuro-ophthalmologic evaluation six months after the prednisone treatment was begun, he had developed right fourth and sixth cranial nerve palsies, and magnetic resonance imaging demonstrated a right orbital apex mass. Trans-sphenoidal biopsy revealed Aspergillus fumigatus. During treatment of aspergillosis, the patient developed a left hemiparesis. Magnetic resonance imaging disclosed multiple ring-enhancing cerebral masses. Biopsy revealed Nocardia asteroides. The patient was successfully treated for both infections with recovery of neurologic function except for the right optic neuropathy. Although immunocompromised patients are known to be subject to multiple infections, this may be the first reported case of concurrent sino-orbital aspergillosis and cerebral nocardiosis.     

Solitary intracranial extra-osseous plasmacytoma presenting with ophthalmic signs.

Solitary plasmacytomas rarely develop in the skull, meninges, or brain. Ophthalmic signs as the initial manifestations of solitary intracranial plasmacytoma have rarely been described. We report the neuro-ophthalmologic, imaging, and pathologic findings for two patients. One patient presented with optic neuropathy, the second with bilateral sixth nerve palsies. Plasmacytoma is a treatable intracranial tumor that should be considered in the differential diagnosis of patients who present with optic neuropathy or sixth nerve palsy.     

The pattern electroretinogram: a long-term study in acute optic neuropathy

We report a 2-year prospective study of the electroretinographic response to reversal of checkerboard patterns (P-ERG) obtained in 63 eyes with acute optic nerve lesions. The aim of the study was to document the value of P-ERG regarding diagnosis and prognosis of four types of optic neuropathy: optic neuritis, compressive or hereditary optic atrophy, and traumatic optic neuropathy. We documented visual loss by neuro-ophthalmologic examination and recorded pattern-reversal visual evoked potentials (P-VEP). The initial P-ERG was normal to large- and medium-sized checks in 89% and the P-VEP abnormal in 94% of eyes with acute optic nerve lesions. Forty-six eyes were followed for up to 2 years. Two groups emerged. Group A (n = 17) gradually and permanently had significant reduction of the P-ERG to three separate check sizes. All 17 had no improvement in acuity better than 20/100, retained centrocecal scotomas, and developed optic atrophy. In group B (n = 29) the P-ERG remained within normal limits to one or more check sizes. Twenty-two of these eyes recovered acuity to 20/25 or better and had resolution of the field defect. The data showed that P-VEP was superior to P-ERG in diagnosis of acute and chronic optic nerve lesions. However, significant reduction of the b-wave of the P-ERG to three separate check sizes correlated closely with failure of visual recovery and the eventual development of severe optic atrophy, suggesting a prognostic value for P-ERG in optic neuropathy.     

Patterns of white matter diffusivity abnormalities in Leber’s hereditary optic neuropathy: a tract-based spatial statistics study

Leber's hereditary optic neuropathy (LHON) is a mitochondrial disease characterized by retinal ganglion cell degeneration and optic nerve atrophy, leading to a loss of central vision. The aim of this study was to explore the topographical pattern of damage to the brain white matter (WM) tracts from patients with chronic LHON using diffusion tensor (DT) MRI and tract-based spatial statistics (TBSS). Brain dual-echo and DT MRI scans were acquired from 13 patients with chronic LHON and 25 matched controls using a 3.0?T scanner. TBSS analysis was performed using the FMRIB's Diffusion Toolbox. A complete neuro-ophthalmologic examination, including standardized automated Humphrey perimetry as well as average and temporal peripapillary retinal nerve fiber layer thickness (PRNFL) measurements, was obtained in all patients. Mean average and temporal PRNFL thicknesses were decreased significantly in LHON patients. Compared to controls, TBSS analysis revealed significant diffusivity abnormalities in these patients, which were characterized by a decreased fractional anisotropy (FA) and an increased mean diffusivity and radial diffusivity, affecting exclusively the optic tracts and optic radiations (OR). In patients, a significant correlation was found between optic tract average FA and mean visual acuity (r?=?0.57, p?=?0.04). In LHON patients, DT MRI reveals a microstructural alteration of the WM along the entire visual pathways, with a sparing of the other main WM tracts of the brain. Damage to the OR may be secondary either to trans-synaptic degeneration, which in turn is due to neuroaxonal loss in the retina and optic nerve, or to local mitochondrial dysfunction.     

Optic neuropathy associated with periostitis in relapsing polychondritis.

Optic neuropathy is an uncommon manifestation of relapsing polychondritis (RPC), a rare systemic disease affecting cartilaginous and proteoglycan-rich structures. The optic neuropathy has been attributed to ischemia, intrinsic inflammation of the optic nerve, or spread of inflammation to the nerve from adjacent intraconal orbital tissues. We report a case of recurrent corticosteroid-responsive optic neuropathy in which MRI did not show ocular, optic nerve, or intraconal orbital abnormalities but did show periosteal thickening and enhancement in the apical orbit and adjacent intracranial space consistent with periostitis. The periostitis, which is a manifestation of a systemic vasculitis or an autoimmune reaction to progenitors of cartilage, probably caused the optic neuropathy by compression or inflammation. It is important to recognize this mechanism of optic neuropathy as its imaging features may be a subtle yet critical clue to an underlying systemic condition that can be life-threatening if not properly managed.     

Choroidal and optic nerve infarction in hepatitis C-associated polyarteritis nodosa.

A 39-year-old man presented with headache, weight loss, bilateral subdural hematomas, pansinusitis, and visual loss. The neuro-ophthalmologic examination disclosed deep choroidal lesions and bilateral optic disc edema. Orchiectomy for testicular torsion showed acute vasculitis consistent with polyarteritis nodosa (PAN). Polymerase chain reaction (PCR) testing revealed hepatitis C. This is the first reported case of PAN due to hepatitis C with early findings of choroidal and optic nerve infarction.     

An epidemic of optic neuropathy and painful sensory neuropathy in Cuba: Clinical aspects

An epidemic of bilateral optic neuropathy and painful sensory neuropathy occurred in Cuba in 1991–1993. Over 45 000 individuals were stated to have been affected. We report a clinical study on 25 patients seen in Cuba in 1993–1994. Affected patients showed either bilateral optic neuropathy with caecocentral scotomata or a distal predominantly sensory neuropathy sometimes associated with deafness, or a combination of both optic and peripheral sensory neuropathy. The nature of the epidemic is discussed. The clinical features in patients with confirmed neurological deficits were consistent with a diagnosis of Strachan's syndrome, probably related to nutritional deficiency. Other patients with similar symptoms showed no evidence either of optic or peripheral neuropathy and were considered to represent disease mimicry on a psychoneurotic basis.     

Contemporaneous retinal and optic nerve infarcts, choroidal non-perfusion, and Hollenhorst plaque: are these all embolic events?

A 76-year-old man developed a sudden painless superior field defect in the right eye, retinal whitening along the inferior temporal arcade, and fluorescein angiographic evidence of lobular choroidal non-perfusion. One week later, ophthalmoscopy revealed inferior optic nerve edema with splinter hemorrhages consistent with an anterior ischemic optic neuropathy (AION) and a new cholesterol plaque near the macula. There was no clinical, serologic, or pathologic evidence of giant cell arteritis. Carotid ultrasound revealed no evidence of significant stenosis but did show an echolucent soft plaque in the right carotid artery. Transthoracic echocardiography demonstrated normal left ventricular function with no source of emboli. The presumed cause of the clinical findings in this patient was embolism, a rarely reported cause of AION. An embolic origin may be considered in non-arteritic AION associated with choroidal non-perfusion.     

Histoplasma meningitis with hyperactive suppressor T cells in cerebrospinal fluid.

Histoplasma meningitis usually occurs in conjunction with disseminated histoplasmosis. We studied a patient with common variable hypogammaglobulinemia who manifested meningitis without disseminated histoplasmosis. No histoplasma antibody was detected in cerebrospinal fluid (CSF) or blood. Evaluation of lymphocyte function in the blood revealed normal numbers of T cells with increased numbers of B cells. Most blood lymphocytes were identifiable, but most lymphocytes in CSF were null cells. Lymphocyte proliferative response to phytohemagglutinin or pokeweed mitogen was poor. T cells in CSF suppressed proliferative responses to histoplasma antigen by cells from blood or CSF, whereas T cells from blood did not. This difference suggested compartmentalization of T-cell function. The lack of humoral and cellular response to histoplasma in CSF may have allowed meningitis to develop, while the cellular response to histoplasma elsewhere prevented development of disseminated histoplasmosis.     

An autopsy case with cerebral histoplasmoma: case report]

Histoplasma capsulatum infection is, for the most part, asymptomatic or of little clinical consequence. Disseminated infection due to H. capsulatum is rather uncommon. Clinically apparent infection of central nervous system (CNS) is rare, and involves in 10 to 50% of patients with disseminated histoplasmosis. Although CNS histoplasmosis is frequently fetal or only discovered as an autopsy, some patients can be effectively treated with anti-fungal agents. We describe a 44-year-old civil man who is engineering contractor with headache without evidence of systemic infection. Magnetic resonance imaging showed enhancing masses in the third and forth ventricles, right interpeduncular cistern, and right cerebello-pontine angle. After biopsy of what was presumed to be a malignant lymphoma, the patient died of rapidly progressive multiple cerebral infarctions. The autopsy revealed the CNS histoplasmoma disseminating systemically. And we finally diagnosed him as histoplasmoma by gene analysis. It was extremely difficult to make a diagnosis based on his physical and radiological findings because it should be included in the differential diagnosis of a well or ring enhanced lesion. It is very important to ask patients about their birthplace, past illness, occupation, and where they had traveled. In the present case, the patient working for the construction has visited many countries including the African Continent and Central America. Clinicians should maintain a high index of suspicion in patients who are from any area endemic for histoplasmosis. The clinical, radiological and pathological features of this infection were reviewed in this report.     

Optic neuropathy and sixth cranial nerve palsy caused by compression from a dolichoectatic basilar artery.

A 53-year-old man with progressive visual loss in the right eye and diplopia manifested dysfunction of the right optic nerve and the right sixth cranial nerve. MRI revealed a markedly enlarged and tortuous basilar artery, its proximal portion compressing the right sixth cranial nerve at the exit from the pons and its distal portion elevating and compressing the right optic nerve. This is the first report of optic neuropathy and sixth cranial nerve palsy caused by a dolichoectatic basilar artery.     

Anterior ischemic optic neuropathy associated with rickettsia conorii infection.

A 43-year-old man with fever, headache, and skin rash developed unilateral acute anterior ischemic optic neuropathy. The indirect immunofluorescence test was positive for Rickettsia conorii. Although retinal lesions have been described in Rickettsia conorii infection, this is the first reported case of ischemic optic neuropathy. This infection should be considered in a patient with nonarteritic anterior ischemic optic neuropathy with high fever or skin rash who inhabits or travels from an endemic area.     

Anterior ischemic optic neuropathy associated with viagra.

A 42-year-old male presented with acute onset of an inferior visual field defect OD after sildenafil citrate use. Examination revealed a right relative afferent pupillary defect and a swollen disc with a 0.1 cup-to-disc ratio and a prominent disc hemorrhage. Anterior ischemic optic neuropathy (AION) is associated with acute episodes of hypotension in patients with structurally crowded discs. Sildenafil citrate may cause episodes of hypotension and was temporally related to the onset of symptoms in this patient. Because patients are often reluctant to volunteer their history of sildenafil citrate use, the physician may need to ask specifically about use of this medication. Physicians should counsel patients with crowded optic discs and a history of nonarteritic anterior ischemic optic neuropathy in one eye that use of sildenafil citrate might increase their risk of ischemic optic neuropathy in the fellow eye.     

Progressive visual loss following rupture of an intracranial dermoid cyst

A 51-year-old man with several months of headache and progressive visual decline was found to have bilateral optic disc pallor with significant impairment of visual acuity. Despite a thorough ophthalmologic evaluation, the cause of visual loss could not be elucidated. MRI of the brain revealed a lesion in the left anterior Sylvian fissure as well as disseminated foci of subarachnoid fat consistent with a diagnosis of a ruptured dermoid cyst. The decision for open surgical resection was chosen to minimize the risk of cyst re-rupture and further visual or neurologic decline. The diagnosis of dermoid cyst was confirmed at the time of surgery. Vasospasm-induced ischemia of the optic nerves, optic chiasm or bilateral optic tracts secondary to the inflammatory reaction following cyst rupture is the most likely mechanism of visual loss in this patient. To the authors’ knowledge, this report represents the first reported case of visual loss secondary to rupture of an intracranial dermoid cyst not related to mass effect of the tumor on the optic apparatus, visual pathways or visual cortex.     

Chiasmal high signal on magnetic resonance imaging in the atrophic phase of leber hereditary optic neuropathy.

A 30-year-old man with Leber hereditary optic neuropathy mutation 11778 displayed no magnetic resonance imaging abnormalities during the acute phase of visual loss in the right eye. Three months later, during the acute phase of visual loss in the left eye, magnetic resonance imaging showed T2 hyperintense signal changes on the right half of the optic chiasm. Six months later, magnetic resonance imaging revealed T2 hyperintense signal changes on both sides of the optic chiasm. This is the first reported case of optic chiasmal involvement on magnetic resonance imaging in the atrophic phase of Leber hereditary optic neuropathy.     

Acquired pendular nystagmus in multiple sclerosis: clinical observations and the role of optic neuropathy.

Thirty seven patients with pendular nystagmus due to multiple sclerosis were reviewed. Most developed nystagmus later in a progressive phase of the disease. All had cerebellar signs on examination and evidence of optic neuropathy. MRI in eight patients showed cerebellar or brainstem lesions in seven; the most consistent finding was a lesion in the dorsal pontine tegmentum. Dissociated nystagmus was seen in 18 patients: in these the signs of optic neuropathy were often asymmetric and the severity correlated closely with the side with larger oscillations. This suggests that dissociations in acquired pendular nystagmus may be due to asymmetries in optic neuropathy rather than asymmetries in cerebellar or brainstem disease.     

Mitochondrial DNA nucleotide changes C14482G and C14482A in the ND6 gene are pathogenic for Leber's hereditary optic neuropathy

A novel mitochondrial DNA nucleotide transversion, C14482A (M64I), different from the previously reported C14482G (M64I), was found to cause Leber's hereditary optic neuropathy with visual recovery in an Italian family. These equivalent changes are the fifth pathogenic mutation for pure Leber's hereditary optic neuropathy. This confirms that the ND6 gene of complex I is a mutational hot spot and suggests that different amino acid substitutions at residue 64, as induced by C14482G or C14482A (M64I) and the common T14484C (M64V) mutations, are associated with visual recovery.     

Retinal and intracranial arteriovenous malformations: Wyburn-Mason syndrome.

A 7-year-old girl who failed a vision screening examination was found to have a retinal arteriovenous malformation (AVM) in her right eye along with a small angioma of her right lower lip. Magnetic resonance imaging and cerebral angiography revealed a large AVM involving the thalamus and basal ganglia as well as a small AVM involving the right ophthalmic artery. The combination of retinal and intracranial AVMs is known as Wyburn-Mason syndrome. The retinal AVMs of this syndrome do not grow or bleed. Visual loss is usually the result of optic neuropathy from associated orbitocranial AVMs. The natural course of the intracranial AVMs of this syndrome is poorly documented. However, they tend to be extensive and inaccessible and therefore difficult to treat.     

A case of posterior ischemic optic neuropathy in a posterior-draining dural cavernous sinus fistula.

A 79-year-old woman presented with sudden unilateral visual loss after an ocular motor disturbance and pulsatile tinnitus. Neuro-ophthalmologic examination showed a presumed right posterior ischemic optic neuropathy (PION), oculosympathetic, and third, sensory fifth, and sixth cranial nerve pareses. Selective angiography of the right internal and external carotid arteries confirmed a posterior-draining dural carotid cavernous sinus fistula (CCF) fed by the right meningohypophyseal trunk and right middle meningeal artery. Angiography also showed an ophthalmic-middle meningeal arterial anastomosis. We postulate that the PION was caused by an arterial steal, because blood was drawn into the fistula and away from the intraorbital optic nerve.     

Magnetic resonance imaging of luxury perfusion of the optic nerve head in anterior ischemic optic neuropathy

ABSTRACT:: A 49-year-old woman with painless reduction in visual acuity in her left eye was found to have nonarteritic anterior ischemic optic neuropathy (NAION). Fluorescein angiography revealed optic disc capillary leakage consistent with "luxury perfusion." Contrast-enhanced FLAIR magnetic resonance imaging (MRI) showed marked enhancement of the left optic disc. Resolution of the optic disc edema and the MRI abnormalities followed a similar time course. This report appears unique in documenting the MRI findings of luxury perfusion in NAION.