Take the lead.

A 23-year-old previously healthy woman presented with headache, generalized seizures, ataxia, encephalopathy, abdominal pain, nausea, and vomiting culminating in a 40-pound weight loss. A contrasted magnetic resonance imaging scan of the brain showed T2/FLAIR hyperintensities in the sulci of the occipital and parietal lobes, a punctate focus of restricted diffusion along the inferior aspect of the left caudate head and an empty sella. A lumbar puncture showed an opening pressure of 55 cm H2O, and kidney, ureter, and bladder X ray showed a radiopaque particle in the colon. Serum lead level was 85 mcg/dL (<3.5). Blood smear showed foreign bodies identified as lead particles in the blood with basophilic stippling of RBCs. She was treated with chelation therapy and bowel irrigation and eventually recovered. Further investigation indicated that she was being slowly poisoned by her husband, a chiropractor who had access to lead.

Skin in the game.

A 23-year-old man with a history of migraine and focal seizures, developed painless visual loss in the left eye associated with optic disk edema. There was no recent history of cat exposure except for a cat that lives outside. Initial laboratory studies were negative. magnetic resonance imaging brain and orbits without and with fat suppression and intravenous contrast was normal. The patient admitted to routinely skinning, processing and consuming deer while not wearing gloves, and he also frequently had cuts on his hands while doing so. The serum Toxoplasma gondii IgG antibody returned at >400 (0-7.1 IU/mL) with the IgM at 10.4 (0-7.9 AU/mL).

Pott luck.

A 10-year-old boy presented with headache, fever, left-sided ptosis, and right-sided forehead soft tissue swelling. There was no recent history of trauma or infection. The patient had a large, fluctuant mass on the right side of his forehead, upgaze restriction, left-sided ptosis, and bilateral optic disk edema. Magnetic resonance imaging of the brain showed a frontal bone extradural fluid collection superficial to the superior sagittal sinus in keeping with an epidural abscess. There were multiple venous thromboses and thickening and enhancement of the dura, compatible with meningitis. There was right sphenoid sinusitis. This patient had Potts puffy tumor, a rare diagnosis associated with a forehead swelling from frontal bone osteomyelitis and subperiosteal abscess. It is seen in the pediatric population in association with sinusitis or trauma. Antibiotics, anticoagulation, and acetazolamide were initiated, and the epidural abscess was evacuated. The symptoms and signs resolved with treatment.

A Case of Progressive Supranuclear Palsy-like Syndrome: Aetiology Revealed.

In 2015, we published an enigmatic case in Neuro-Ophthalmology regarding an 18-year-old woman who underwent an initially uneventful ascending aortic aneurysm repair. After 48 h, she developed a progressive supranuclear palsy-like syndrome with no findings on magnetic resonance imaging (MRI), MRI tractography, or functional MRI which correlated with this syndrome. After following our patient over the ensuing years, it became clear that the likely aetiology of these neurologic deficits was the profound deep hypothermia associated with her aortic aneurysm repair.

The best course of action.

An 11-year-old girl noted gradual visual loss in the right eye for 1 year with subfoveal yellow deposits in both eyes. Optical coherence tomography, electro-oculogram and electroretinogram was in-keeping with Best Disease. This disorder is discussed.

Intraparenchymal Schwannoma: A Rare Entity.

A 60-year-old woman noted headaches, transient visual obscurations and pulsatile tinnitus. She had Frisén grade 4 papilloedema. Brain magnetic resonance imaging showed a large subfrontal cystic mass with ring enhancement. Tumour pathology was in-keeping with an intraparenchymal schwannoma. This uniquely rare tumour is discussed.

Diagnostic Evaluation of Visual Snow.

Introduction: To determine which patients with visual snow (VS) and VS syndrome (VSS) require standard ophthalmologic testing including automated visual field and which patients require further testing such as macular spectral domain optical coherence tomography (SD-OCT), electrophysiology, and neuroimaging. Materials and Methods: We retrospectively reviewed 52 consecutive patients at three institutions with VS and VSS including the University of Alabama, Callahan Eye Hospital, the University of Missouri-Kansas City School of Medicine, and the Little Rock Eye Clinic from the years 2015 to 2021. We collected historical information, examination findings, ophthalmic testing, electrophysiology, and neuroimaging. Results: Of the 52 patients with VS and VSS, eight of the 52 cases met the clinical criteria for VSS. The ages ranged from 7 to 79 years, with a mean age of 25 years (SD = 14.0). There were 22 males and 30 females. Color vision was tested in 51 cases and was normal in 47 cases (92%). A funduscopic exam was performed in all 52 cases and was normal in 46 cases (88%). The macular SD-OCT was normal in all of the 19 cases that it was performed (100%). A Humphrey visual field was performed in 50 cases and was normal in 43 (86%). A visually evoked potential (VEP) was normal in 18 of the 19 cases where it was obtained (95%). The full-field electroretinography (ffERG) was obtained in 28 cases and was normal in 25 (89%). The multifocal electroretinography (mfERG) was normal in 11 of 12 cases (92%). Only four patients accounted for all of the abnormal electrophysiological tests. In the 37 cases that had an MRI, 29 were normal (78%). Only one patient revealed a lesion in the visual pathway (right optic nerve enhancement in an optic neuritis patient). Conclusions: Patients with VS and VSS, if typical in presentation and with normal testing, do not require a workup beyond a thorough history, neuro-ophthalmologic examination, and automated perimetry. If this testing is abnormal, then ancillary testing is required.

Chronic recurrent multifocal osteomyelitis, central retinal artery occlusion and optic neuropathy: A new association.

PURPOSE: To report a patient with chronic recurrent multifocal osteomyelitis (CRMO) complicated by optic neuropathy and central retinal artery occlusion (CRAO). OBSERVATIONS: CRMO is a noninfectious, inflammatory bone disorder. It is thought to be an autoimmune condition related to an imbalance of pro- and anti-inflammatory cytokines. Retinal vasculitis has been reported in a patient with CRMO but not CRAO or optic neuropathy. CONCLUSIONS: We expanded the list of ophthalmic involvement of CRMO to include CRAO and optic neuropathy.

Disorders of the Fourth Cranial Nerve.

ABSTRACT: This review of disorders of the fourth cranial nerve includes discussion on anatomy, examination techniques, congenital and acquired etiologies, differential diagnosis, and management options. The findings of the superior oblique muscle on orbital MRI in patients with fourth nerve palsy have had a major impact on our understanding of this cranial neuropathy. In addition, briefly reviewed are rare disorders of the fourth nerve: superior oblique myokymia, Brown syndrome, and ocular neuromyotonia. It behooves the clinician to have a clear understanding of the role that the fourth cranial nerve plays in a variety of neuro-ophthalmic conditions.

Erythropsia and Chromatopsia: Case Study and Brief Review.

A 65-year-old woman presented with erythropsia (red-tinged vision) in the right eye from a subfoveal macula dehaemoglobinised intraretinal haemorrhage. Erythropsia is a type of chromatopsia, a condition in which objects appear to be abnormally coloured or tinged with colour. This manuscript provides a brief review of colour vision abnormalities including chromatopsia, and additionally we discuss dyschromatopsia and achromatopsia defined as deficiency and absence of colour vision respectively, both of which may be congenital or acquired. We theorise that the mechanism of the chromatopsia may be selective damage of ganglion cells involved in colour opponency.

The Visual Agnosias and Related Disorders.

BACKGROUND: There are many disorders of higher visual processing that result from damage to specific areas of the cerebral cortex that have a specific role in processing certain aspects (modalities) of vision. These can be grouped into those that affect the ventral, or "what?", pathway (e.g., object agnosia, cerebral achromatopsia, prosopagnosia, topographagnosia, and pure alexia), and those that affect the dorsal, or "where?", pathway (e.g., akinetopsia, simultanagnosia, and optic ataxia). EVIDENCE ACQUISITION: This article reviews pertinent literature, concentrating on recent developments in basic science research and studies of individual patients. RESULTS: An overview of the current understanding of higher cerebral visual processing is followed by a discussion of the various disorders listed above. CONCLUSIONS: There has been considerable progress in the understanding of how the extrastriate visual cortex is organized, specifically in relation to functionally specialized visual areas. This permits a better understanding of the individual visual agnosias resulting from damage to these areas.

Occult Acute Macular Neuroretinopathy.

A 19-year-old Caucasian woman developed an upper respiratory infection, took a cold formulation containing 5 mg of phenylephrine, and developed a very rare and unusual form of acute macular neuroretinopathy (AMN) that could not be detected on fundoscopic examination, visual fields, nor electrophysiological testing. Spectral-domain optical coherence tomography (SD-OCT) revealed a lesion limited to the fovea. This case illustrates the value of SD-OCT, in light of otherwise normal testing, in a variant of AMN the authors call "occult AMN".