Ryan D. Walsh

Outer retinal structure after closed-globe blunt ocular trauma.

Purpose: To evaluate outer retinal structural abnormalities in patients with visual deficits after closed-globe blunt ocular trauma. Methods: Nine subjects with visual complaints after closed-globe blunt ocular trauma were examined between 1 month after trauma and 6 years after trauma. Spectral domain optical coherence tomography was used to assess the outer retinal architecture, whereas adaptive optics scanning light ophthalmoscopy was used to analyze the photoreceptor mosaic integrity. Results: Visual deficits ranged from central scotomas to decreased visual acuity. Spectral domain optical coherence tomography defects included focal foveal photoreceptor lesions, variable attenuation of the interdigitation zone, and mottling of the outer segment band, with one subject having normal outer retinal structure. Adaptive optics scanning light ophthalmoscopy revealed disruption of the photoreceptor mosaic in all subjects, variably manifesting as foveal focal discontinuities, perifoveal hyporeflective cones, and paracentral regions of selective cone loss. Conclusion: We observe persistent outer retinal disruption in subjects with visual complaints after closed-globe blunt ocular trauma, albeit to a variable degree. Adaptive optics scanning light ophthalmoscopy imaging allows the assessment of photoreceptor structure at a level of detail not resolvable using spectral domain optical coherence tomography or other current clinical imaging tools. Multimodal imaging seems to be useful in revealing the cause of visual complaints in patients after closed-globe blunt ocular trauma. Future studies are needed to better understand how photoreceptor structure changes longitudinally in response to various traumas.

Acute visual loss after ipilimumab treatment for metastatic melanoma

BackgroundIpilimumab, a humanized CLTA-4 antibody is a standard therapy in the treatment of advanced melanoma. While ipilimumab provides an overall survival benefit to patients, it can be associated with immune related adverse events (IrAEs).Case presentationHere we describe a patient treated with ipilimumab who experienced known IrAEs, including hypophysitis, as well as a profound vision loss due to optic neuritis. There are rare reports of optic neuritis occurring as an adverse event associated with ipilimumab treatment. Furthermore, the patient experienced multiple complications from high dose steroids used to manage his IrAEs.ConclusionsThis case highlights the need for recognition of atypical immune mediated processes associated with newer checkpoint inhibitor therapies including ipilimumab.

Optic perineuritis as the presenting feature of Crohn disease.

Crohn disease (CD) is primarily considered an inflammatory condition of the small and large intestine although associated extraintestinal inflammation is relatively common. Ocular manifestations are generally localized to the anterior chamber and ocular surface but rarely can involve the posterior pole, orbit, and optic nerve. We report a case of an otherwise healthy 42-year-old man who was diagnosed with CD after presenting with acute vision loss from optic perineuritis.

Bálint syndrome and visual allochiria in a patient with reversible cerebral vasoconstriction syndrome.

Bálint syndrome (simultagnosia, optic ataxia, and ocular apraxia) is typically caused by pathology affecting the parietal-occipital regions bilaterally. Visual allochiria is an uncommonly reported symptom associated with parietal lobe pathology in which visual stimuli presented to one hemispace are transposed to the opposite side. We describe a patient with Bálint syndrome and visual allochiria whose initial brain MRI demonstrated acute infarction of the right parietal-occipital region. Repeat imaging 9 days later revealed bilateral parietal-occipital infarctions consistent with the observed clinical syndrome. Reversible cerebral vasoconstriction syndrome is introduced as a novel cerebrovascular etiology of Bálint syndrome.

Primary adenoid cystic carcinoma of the orbital apex

Adenoid cystic carcinoma is an uncommon malignant tumor of epithelial origin typically arising from salivary glands. Orbital involvement may occur via direct or perineural spread from a lacrimal gland or sinonasal source. Primary orbital adenoid cystic carcinoma without involvement of the lacrimal gland is rare. The authors report a 53-year-old woman who was examined for insidious monocular vision loss and was found to have a primary adenoid cystic carcinoma of the orbital apex and cavernous sinus. Systemic workup for a primary source, including ipsilateral lacrimal gland biopsy, was negative. One must maintain a high index of suspicion for adenoid cystic carcinoma when evaluating orbital tumors.

Complex transient epileptic amnesia

Transient epileptic amnesia is a rare but probably underrecognized form of temporal lobe epilepsy, which typically manifests as episodic isolated memory loss. Consequently, transient epileptic amnesia may be readily misdiagnosed as a nonepileptic memory dysfunction in older individuals. When appropriately recognized, it has been described as a treatment-responsive syndrome amenable to antiepileptic drugs. We describe a patient with drug-resistant transient epileptic amnesia treated with unilateral temporal lobectomy. Prolonged postictal slowing in the mesial temporal structures was evident on invasive electroencephalography 5 hours after the occurrence of a brief focal seizure. These findings support the theory of a Todd phenomenon as the underlying pathophysiological mechanism in transient epileptic amnesia.

Congenital mydriasis associated with megacystis microcolon intestinal hypoperistalsis syndrome.

We report a case of congenital mydriasis in a neonate with megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS). Pilocarpine testing and gastrointestinal pathology in our patient suggest that the mydriasis is due to an underlying smooth muscle myopathy of the iris sphincter muscle. These findings may have important implications regarding the pathogenesis of MMIHS.

STEPPING MOVEMENTS INDUCED BY ACUTE SPINAL CORD INJURY

Caudal to the forebrain, the generation of basic locomotion is thought to involve a network of neurons in the brainstem and spinal cord. This network consists of the mesencephalic locomotor region (MLR), reticulospinal neurons in the pontomedullary region, and spinal cord locomotor central pattern generators (CPGs).1 In humans, spinal CPGs are thought to be sufficient for generating stepping movement when activated by appropriate sensory or descending inputs.2 However, the anatomic location of descending pathways connecting brainstem and spinal CPGs that initiate stepping movement has not been defined in humans. Here we describe a remarkable patient who developed transient, bilateral, and uncontrollable stepping movements following unintentional puncture injury at the C2 spinal cord level. ### Case report. A 35-year-old woman with unexplained, unremitting headache underwent a diagnostic cervical spinal tap after an unsuccessful lumbar tap. The cervical tap was complicated by cord puncture, immediately after which the patient developed uncontrollable bilateral stepping movements while supine. The stepping movements involved rhythmic, bilateral hip and knee flexion and extension, and occurred paroxysmally about four to five cycles per minute. Simultaneously the patient described an ill-defined urge to move her legs, but stated that this was not in response to pain or discomfort. Neurologic examination identified …