Hypertrophic cranial pachymeningitis and orbital apex syndrome secondary to infection of the eye: illustrative case

BACKGROUND Hypertrophic cranial pachymeningitis is a rare inflammatory disorder characterized by thickening of the dura mater and multiple cranial neuropathies. Although an infectious etiology may be present, often no specific cause is discovered. OBSERVATIONS The authors described a 71-year-old man with progressive right eye vision loss, ptosis, and complete ophthalmoplegia with imaging findings suggestive of hypertrophic cranial pachymeningitis. Extensive studies, including cerebrospinal fluid studies, showed negative results. Blood serum, cell-free evaluation, and paraffin-embedded dural tissue testing had positive results for Pseudomonas aeruginosa, which allowed treatment tailored to the organism and a salutary clinical outcome. LESSONS The constellation of neurological and radiological findings may make a diagnosis difficult in an inflammatory setting. The most precise methodology for establishing a diagnosis involves sampling the dura and testing it for infectious pathology. However, if results are inconclusive, further cell-free serum sampling with next-generation sequencing is a viable option for identifying pathogens with infectious concerns. This case highlighted the importance of multimodality studies for identifying a targetable pathogen.

Rhino-orbital mucormycosis during steroid therapy in COVID-19 patients: A case report

Purpose: To report two cases of COVID-19 under treatment with a corticosteroid; in one case rhino-orbitocerebral mucormycosis and in another one rhino-orbital mucormycosis developed. Case presentation: A 40-year old woman and a 54-year old man with severe COVID-19 underwent corticosteroid therapy for immune-related lung injuries. The first case presented with a bilateral visual loss and complete ophthalmoplegia of the right eye. The second case presented with vision loss, proptosis, orbital inflammation, and complete ophthalmoplegia on the left side. Histopathologic, nasal endoscopic examinations, and radiologic findings confirmed mucormycosis in both patients. The patients denied orbital exenteration and were managed with systemic amphotericin B and daily endoscopic sinus debridement and irrigation with diluted amphotericin B. Because of the intracranial space involvement, the first case died. The second case was successfully managed surgically and medically. Conclusion: Rhino-orbital/cerebral mucormycosis may be developed in COVID-19 patients under treatment with corticosteroid, and requires prompt diagnosis and management.

A. Péckin and M. Rollin. Ophthalmoplegia and arteriosclerosis. Revue Neurol. 1903, pp. 256

The authors describe a case where a patient suffering from syphilis and tabes had complete ophthalmoplegia and atrophy n during his lifetime. optici on the left eye, incomplete ptosis and paralysis of the upper part of the right eye.

Complete bilateral ophthalmoplegia in malignant intracranial hypertension in a child

Purpose: To describe a case of fulminant idiopathic intracranial hypertension (IIH) in a child with “malignant” presentation. Case report: A 16-year-old, previously healthy, girl presented with bilateral visual loss and bilateral global limitation of eye movements in the absence of headache. Extensive laboratory evaluation for infectious, inflammatory, autoimmune, and neoplastic conditions was negative. Magnetic resonance imaging (MRI) of the brain and lumbar puncture findings were consistent with a diagnosis of IIH. Extraocular motility improved in the next few days as well as optic disc edema but visual acuity remained poor. Conclusion: The authors believe that the acute, severe, and fulminant (“malignant”) presentation with markedly elevated intracranial pressure may produce the unique presentation of severe vision loss and bilateral complete ophthalmoplegia. Interestingly, there was no headache. To our knowledge this is the first such case to be reported in the English language ophthalmic literature.

Immunoglobulin G4-Related Ophthalmic Disease: A Case Report

The authors reported a rare case with immunoglobulin (Ig) G4-related ophthalmic disease presented with chronic progressive bilateral complete ophthalmoplegia and blindness from orbital apex syndrome. MRI brain and orbit demonstrated ill-defined infiltrative lesions at bilateral orbital apexes, bilateral optic canals, and bilateral Meckel’s caves, causing optic nerve compression and possibly optic neuropathy with generalized leptomeningeal enhancement at dura, cavernous sinus, and parotid gland. Lumbar puncture revealed few small lymphocytes, rare monocytes, very rare neutrophils with degenerative cells in background, and negative for malignancy. Serology titers for IgG subclass 4 (IgG4) had resulted in 5.959 grams per deciliter (g/dL). A dural biopsy revealed aggregate histiocytes with chronic inflammation and focal foreign body type giant cells. Motility improvement was achieved in the patient after systemic corticosteroids treatment. IgG4 serology should be considered for workup when patients present with chronic idiopathic orbital inflammation. Keywords: IgG4-related ophthalmic disease, Ophthalmoplegia, Orbital apex syndrome

Miller Fisher Syndrome presenting as unilateral abducens nerve palsy: A case report

Background : Miller Fisher Syndrome usually presents with complete ophthalmoplegia, areflexia and ataxia. We present an unusual case which presented with unilateral abducens nerve palsy. Case presentation : A 51 year old female patient presented with a history of difficulty in walking and double vision for 1 week which started 2 weeks following an episode of acute gastroenteritis. She didn’t have any bladder bowel incontinence or difficulty in breathing. On examination there was left side abducens nerve palsy and bilateral significant dysmetria. Upper limb and lower limb power was 4/5 with global areflexia. There was no fatigability or sensory deficit. Higher cortical functions were intact with Glasgow Coma Scale of 15/15. Brain stem cerebro vascular accident, alcohol, toxin or drug mediated disease, myasthenia gravis, Bickerstaff encephalitis and Miller-Fisher syndrome were considered as possible differential diagnosis. There was no history of alcohol consumption or any exposure to drugs or toxins. Her Non contrast brain and MRI brain were normal. Nerve conduction study showed asymmetrical sensory motor and F wave abnormalities consistent with a Guillen-Barre Sydrome variant. Cerebro Spinal Fluid analysis showed albumino-cytological dissociation. These findings suggested the diagnosis of Miller-Fisher Syndrome. She was started on plasmaphareis. Her vital parameters, vital capacity and neurological deficit were closely monitored. With 5 cycles of plasmapharesis she made a complete neurological recovery and she was discharged on 16th day of admission. Conclusion: Miller Fisher Syndrome can present as unilateral abducens nerve palsy and early diagnosis and treatment leads to excellent functional outcome.