Ocular Paresis Research Articles

Angioinvasive Rhino-Orbital-Cerebral Mucormycosis in a Patient with Type 2 Diabetes Mellitus: A Complex and Lethal Cause of Stroke

Rhino-orbital-cerebral mucormycosis is a rapidly progressive and often fatal fungal infection caused by molds of the order Mucorales, particularly affecting immunocompromised individuals. This infection is notorious for its angioinvasive properties, enabling the fungi to invade blood vessels and leading to tissue necrosis. We report the clinical course of a 59-year-old Caucasian man with poorly controlled type 2 diabetes (HbA1c 16.8%) who presented with unilateral headache, left-sided facial numbness, and incomplete left ocular motor paresis. Initial presentation raised suspicion of orbital phlegmon, leading to antibiotic and later corticosteroid pulse therapy, which worsened the patient’s condition. Subsequent imaging demonstrated extensive inflammatory changes, including wall irregularities of the left intracranial internal carotid artery, accompanied by ocular protrusion and periorbital enhancement. New palatal lesions indicated mucormycosis, which was confirmed by molecular analysis of a palatal biopsy, leading to Amphotericin B treatment. Pre-surgery imaging revealed a malignant middle cerebral artery infarction, and the patient died 16 days after symptom onset and 12 days after initial presentation under palliative care due to a poor prognosis. This case of angioinvasive mucormycosis underscores the severe and often fatal course of rhino-orbital-cerebral mucormycosis in an immunocompromised individual. The rapid progression from initially vague and unspecific symptoms to extensive vascular involvement and stroke highlights the critical need for early and accurate diagnosis, as well as prompt intervention to prevent further disease progression. Additionally, this case also illustrates the potential risks associated with corticosteroid therapy in the presence of undiagnosed fungal infections, which can exacerbate the condition and lead to serious complications. Clinicians should maintain a high index of suspicion for mucormycosis in similar clinical scenarios, prioritizing adequate antifungal treatment and careful monitoring to improve patient outcomes. Early interdisciplinary collaboration is essential for the effective management of such complex cases.

Anatomical aberration of posterior part of circle of Willis with special reference to third nerve palsy

Due to the close anatomical proximity, aneurysm of posterior communicating artery may cause oculomotor nerve palsy, which is a common manifestation and it may present with pupil involving extra ocular muscle paresis. The onset may be acute due to sudden aneurysm dissection along with subarachnoid hemorrhage. This may warrant urgent lifesaving surgical intervention along with the options of interventional radiological procedures.Normally, the occipital lobe is supplied by the posterior cerebral artery (PCA). When the pre-communicating portion of the PCA is larger than the posterior communicating artery (PCA), the occipital lobe is primarily supplied by the vertebrobasilar artery. Sometimes the pre-communicating portion of the PCA may be smaller than the PCoA in which the occipital lobe is primarily supplied by the internal carotid artery.Understanding the posterior circulation anomalies of Circle of Willis. This study involved 50 fetal brains belonging to perinatal age group and 30 adult brains of human cadavers by dissection. The PCoA was missing in 14% of the samples. The difference between the outer diameters of the P1 portion of the PCA and the PCoA was very significant.So, a detailed anatomical understanding of Circle of Willis and manifestation of the third nerve palsy is sometimes equivocal for a clinician for better management and patient outcome.

Extensive extracranial growth of spheno-orbital meningioma: illustrative case.

Spheno-orbital meningioma (SOM) typically presents with a classic triad of symptoms (i.e., proptosis, visual impairment, and ocular paresis), resulting from intraorbital tumor invasion. The authors present a very rare case of SOM in which the chief complaint was swelling of the left temporal region, which, to the best of their knowledge, has not been reported previously. The patient presented with marked extracranial extension to the left temporal region but unremarkable intraorbital extension, even on radiological examination. Physical examination of the patient showed almost no exophthalmos or restriction of left eye movement, consistent with the radiological findings. Four separate meningioma specimens were removed by extraction (i.e., one each from the intracranial, extracranial, and intraorbital segments of the tumor and one from the skull). The World Health Organization grade was 1 and the MIB-1 index was less than 1%, indicating a diagnosis of a benign tumor. SOM may be present even in patients with only temporal swelling and few ocular-related symptoms, and detailed imaging evaluations may be required to identify the tumor.

Functional and clinical outcomes after superior eyelid transorbital endoscopic approach for spheno-orbital meningiomas: illustrative case and literature review.

The pattern of growth of spheno-orbital meningiomas accounts for the main presenting symptoms, such as proptosis, eye motility deficit, visual impairment, diplopia. As these are benign tumors, the postoperative patient's quality of life is an important factor to consider during the preoperative planning.A detailed literature review of superior eyelid transorbital endoscopic approach for spheno-orbital meningiomas, including our own case, was made. A Medline search up to March 2022 in PubMed online electronic database was made using the following key phrases: "superior eyelid endoscopic transorbital approach spheno-orbital meningiomas," "superior eyelid endoscopic transorbital approach," "spheno-orbital meningiomas endoscopic approach." The inclusion criteria were surgical series, reviews, and case reports in English language, as well as papers written in other languages, but including the abstract in English. Cadaveric studies, multiportal combined approaches for SOM, were excluded. The literature review has disclosed five studies for a total of 65 patients, whose demographic, clinical, pathological, surgical, complications, and outcome data were analyzed.Functional and esthetic outcome data after superior eyelid transorbital approach are the following: improvement of proptosis (100%), of visual deficits (66.66%) and of ocular paresis (75%), with only 11 complications (4 trigeminal dysesthesia, 2 CSF leak, 2 wound complications, 1 upper eyelid necrosis, 1 hemorrhage of surgical field, 1 keratitis) reported, but at the expense of extent of resection (gross total resection 33.39%).Based on the outcome data, the superior eyelid transorbital endoscopic approach results in a suitable operative technique for selected spheno-orbital meningiomas.

Vestibular syndromes after COVID-19 vaccination: A prospective cohort study.

Background and purposeDizziness and vertigo are common symptoms after COVID‐19‐vaccination. We aimed to prospectively evaluate objective central or peripheral vestibular function in patients with dizziness, vertigo, and postural symptoms that started or worsened after COVID‐19‐vaccination.MethodsOf 4137 patients who presented between January 2021 and April 2022 at the German Center for Vertigo and Balance Disorders, Ludwig Maximilian University of Munich, we identified 72 patients (mean age = 47 years) with enduring vestibular symptoms following COVID‐19 vaccination. All underwent medical history‐taking, and neurological and neuro‐otological workup with bithermal caloric test, video head‐impulse test, orthoptics, and audiometry. Diagnoses were based on international criteria. The distribution of diagnoses was compared to a cohort of 39,964 patients seen before the COVID‐19 pandemic.ResultsSymptom onset was within the first 4 weeks postvaccination. The most prevalent diagnoses were somatoform vestibular disorders (34.7%), vestibular migraine (19.4%), and overlap syndromes of both (18.1%). These disorders were significantly overrepresented compared to the prepandemic control cohort. Thirty‐six percent of patients with somatoform complaints reported a positive history of depressive or anxiety disorders. Nine patients presented with benign paroxysmal positional vertigo, three with acute unilateral vestibulopathy, and seven with different entities (vestibular paroxysmia, Ménière disease, polyneuropathy, ocular muscular paresis). Causally related central vestibular deficits were lacking. Novel peripheral vestibular deficits were found in four patients.ConclusionsNewly induced persistent vestibular deficits following COVID‐19 vaccination were rare. The predominant causes of prolonged vestibular complaints were somatoform vestibular disorders and vestibular migraine, possibly triggered or aggravated by stress‐related circumstances due to the COVID‐19 pandemic or vaccination. An increase of other central or peripheral vestibular syndromes after COVID‐19 vaccination was not observed.

Recurrent Painful Ophthalmoplegic Neuropathy in an Adult Patient: A Case Report With Literature Review

Recurrent painful ophthalmoplegic neuropathy (RPON), previously known as ophthalmoplegic migraine, is a rare disease that predominantly affects children. Recurrent episodes of ocular cranial nerve paresis with ipsilateral headache characterize this disorder. Diagnosis is mainly clinical with imaging being used as an adjunct. The pathophysiology of the disease is unknown. We present here a case of RPON in a 50-year-old female presenting with multiple episodes of headache and diplopia with associated transient thickening and enhancement of the ipsilateral oculomotor nerve on magnetic resonance imaging (MRI).

A Perplexing Case of Pituitary Apoplexy Masquerading as Meningoencephalitis

Pituitary apoplexy (PA) is extremely rare in children and adolescents. It is a life-threatening condition usually results from sudden hemorrhage or infarction induced swelling in a pituitary adenoma. The clinical manifestations of PA include severe headaches, impaired consciousness, fever, visual disturbance, and variable ocular paresis. Therefore, the presence of meningeal irritation may lead to misdiagnosis as a case of meningoencephalitis or spontaneous subarachnoid hemorrhage, and delay in the proper management of the disease. We report a case of 17-year-old pubertal boy who developed sudden severe headache, vomiting, slurring of speech and abnormal behaviour followed by impaired sensorium with fever. The patient who was initially diagnosed with meningoencephalitis (ME) based on clinical presentation and cerebrospinal fluid (CSF) analysis, which was consistent with bacterial meningitis. MRI of brain was performed, confirming a pituitary macroadenoma with hemorrhage and ischemic changes in both basal ganglia and pons. A complete analysis of the pituitary hormones revealed decreased cortisol and thyroid hormone level and hyperprolactinemia and he was subsequently started on placement corticosteroid and L-thyroxine therapy and cabergoline. After 14/ days of antimicrobial therapy with ceftriaxone and ampicillin, the patient improved and was discharged on hormone replacement therapy and surgical advised. Hereby, we report our case with a review of literatures. Bangladesh J Medicine July 2022; 33(1) : 85-88

Atypical neurological manifestations in Wernicke’s encephalopathy due to hyperemesis gravidarum

ABSTRACT Objectives: Hyperemesis gravidarum is known to induce nutritional, water and electrolyte deficiencies which can be fatal if not treated urgently. Thiamine deficiency may lead to a constellation of neurological symptoms that include Wernicke encephalopathy. Moreover, Wernicke encephalopathy is typically manifested as ocular paresis, ataxia and confusion. Methods: Retrospective review of 6 women who developed neurological abnormalities following hyperemesis gravidarum and were treated with varying dosage of parenteral thiamine. Results: Five women developed atypical neurological symptoms, namely, slurred speech, visual loss, seizure and aggressive behaviour while one woman developed typical clinical triad of Wernicke encephalopathy after hyperemesis gravidarum. Magnetic Resonance Imaging (MRI) scans revealed abnormalities suggestive of Wernicke encephalopathy in three women only. All women improved after parenteral thiamine administration during hospital stay and had a complete neurological recovery during 2 months follow up. Discussion: Wernicke encephalopathy may not be necessarily associated with the typical neurological triad and may not have noticeable hyperintensity signal in dorsomedial thalami, mammillary bodies, hippocampus and periaqueductal region during magnetic resonance imaging. Atypical neurological signs and symptoms following hyperemesis gravidarum would invariably respond immediately to appropriate dosage of parenteral thiamine. A lower loading dosage of thiamine (100 mg thrice daily) appeared adequate for management in women with normal MRI scans.

Acute Onset Variable and Progressive Trochlear Nerve Palsy and Ophthalmoparesis Secondary to Bilateral Carotid Cavernous Fistula

ABSTRACT Purpose: To describe a patient who presented with right unilateral white-eyed posteriorly-draining carotid-cavernous fistulas (CCF) causing an isolated, acute-onset trochlear nerve palsy with atypical progression to a contralateral left red-eyed anteriorly-draining CCF associated with orbital congestion. Observation: A 74-year-old female presented with an acute onset incomitant right hypertropia consistent with the clinical diagnosis of a superior oblique palsy. Initial workup including MRI was normal. During a several-week course, she progressed to a comitant deviation. Six-weeks later she developed an episode of severe headache followed by worsening of her diplopia, reverse left hypertropia and left orbital congestive signs. CTA revealed mild opacification of the left cavernous sinus without a concomitantly present superior ophthalmic vein dilatation. A diagnostic angiogram was obtained which revealed a posterior-draining indirect CCF of the right internal carotid artery (ICA) and an anterior-draining indirect CCF of the left ICA. Transvenous Coil embolization resulted in immediate resolution of diplopia, orbital congestion, and headaches. Conclusion: Although it is atypical for a CCF – or other vascular pathology – to present with an isolated fourth nerve palsy, this case illustrates that vascular imaging should be considered in cases of acquired ocular motor paresis associated with headaches and cases with atypical progression despite normal initial cranial imaging.

Surgical Intervention for Two Patients Suffering from Pituitary Apoplexy Associated with Covid-19

Background: Pituitary apoplexy (PA), which is mainly associated with pituitary adenomas, is a very rare disease. PA imposes high risk of hemorrhages and infarction, which in most cases; emergent surgical intervention would be needed. Moreover, SARS-CoV-2 is a newly emergent viral infection, which has shown several neurological manifestations, including intracranial hemorrhages. Yet, a definitive correlation between PA and COVID-19 still needs further investigations. Observations: We report two cases of COVID-19 male patients, who presented with PA manifestations, mainly headache associated with ocular paresis and diplopia. Both patients underwent transsphenoidal resection of the sellar tumor, and both showed significant clinical improvement. Conclusion: Pituitary apoplexy has been linked to COVID- 19 as a post-infection complication, with surgery being a successful treatment.

A Case of Transient Visual Disturbance and Ocular Paresis after Endoscopic Sinus Surgery

Endoscopic sinus surgery (ESS) is widely used as standard surgical treatment for chronic rhinosinusitis. Orbital complications of varying degrees occurred during ESS have been widely reported. If the orbital symptoms occurred immediately after surgery, ocular damage associated with surgery is suspected if the patient’s preoperative ocular function was patent. If immediate action is not taken, permanent visual loss might develop, so it is very important to diagnose orbital complications and take appropriate action. In our case, there was no definite intraorbital hemorrhage when sudden visual loss was noted. The symptoms were fully recovered without further treatment and it is clinically suspected to be caused by transient ocular muscle toxicity of local anesthetics.

Left Planum Sphenoidale Transitional Meningioma -Simpson Grade-1

Planum sphenoidale meningiomas are rare slow growing tumours that impair cognition and lead to behavioural changes. Other common presentations of this condition include persistent headache followed by vision diminution. Dismissing cognitive impairment and behavioural changes as part of dementia, depression or aging might lead to loss of opportunity to treatment. The common symptoms manifested in sphenoid (spheno-orbital) wing meningioma is bulging of the eyes which may be painless, visual impairment and ocular paresis. Headache is also a common symptom, in addition to ptosis. Variants of the clinical syndrome include the following: Eye swelling and hearing loss, optic atropy, Kennedy syndrome and mental changes. Patient had severe headache for which he went for the eye checkup, where the loss of vision in the left eye was confirmed, through the field test. The MRI confirmed the tumour presence. Though the diagnosis was informed it took one year to convince the patient for the surgery. Hence, the author wishes to emphasise the role of tumour support systems. Physician’s serve as patients’ first point of entry into the health care system and as the continuing focal point for all necessary health care services along with their team they play a vital role in convincing patients from postdiagnosis to surgery and recovery.

Acute Monocular Blindness Due to Orbital Compartment Syndrome Following Pterional Craniotomy

We present a case of orbital compartment syndrome (OCS) leading to monocular irreversible blindness following a pterional craniotomy for clipping of an anterior communicating artery aneurysm. OCS is an uncommon but vision-threatening entity requiring urgent decompression to reduce the risk of permanent visual loss. Iatrogenic orbital roof defects are a common finding following pterional craniotomies. However, complications related to these defects are rarely reported. A 65-year-old female who underwent an anterior communicating artery clipping via a pterional approach 4 days before developed proptosis, ocular movement paresis, and irreversible visual impairment following an orthopedic surgery. Computed tomography images revealed an intraorbital cerebrospinal fluid (CSF) collection, which was evacuated via an acute recraniotomy. The next day, proptosis and intraorbital CSF collection on computed tomography images reoccurred and an oral and maxillofacial surgeon evacuated the collection via a blepharoplasty incision and blunt dissection. In addition, the patient was treated with acetazolamide and an external lumbar CSF drainage during 12 days. Hereafter, the CSF collection did not reoccur. Unfortunately, monocular blindness was persistent. We hypothesize the CSF collection occurred due to the combination of a postoperative orbital roof defect and a temporarily increased intracranial pressure during the orthopedic surgery. We plead for more awareness of this severe complication after pterional surgeries and emphasize the importance of 1) strict ophthalmologic examination after pterional craniotomies in case of intracranial pressure increasing events, 2) immediate consultation of an oral and maxillofacial surgeon, and 3) consideration of CSF-draining interventions since symptoms are severely invalidating and irreversible within a couple of hours.

Thrombosis of venous outflows of the cavernous sinus: possible aetiology of the cortical venous reflux in case of indirect carotid-cavernous fistulas.

The presence of a cortical venous reflux (CVR) in a carotid-cavernous fistula (CCF) is well described and is considered to be a criterion for urgent treatment. This reflux is often associated with direct/traumatic CCF and the high-flow of the fistula alone explains the reflux. For indirect CCF, the pathophysiology of a CVR is unclear. All patients treated endovascularly for an indirect CCF with a cortical venous reflux between 2003 and 2015 were included. We retrospectively analysed data focusing on whether venous outflows of the cavernous sinus would opacify or not with the local injection of contrast, in order to locate those that could explain the venous reflux. Twenty consecutive patients (male/female ratio, 2/3) were included in this series with a mean age of 63 years. All patients presented ocular signs and no patients showed any neurological sign secondary to the CVR. We distinguished four patterns of CVR: in the superficial middle cerebral vein (75%), in the uncal vein (15%), in the superior petrosal vein (5%) and in the inferior petrosal vein (5%). Seventy percent of the cases presented a lack of opacification in more than three venous outflows of the CS involved. Each patient received an endovascular therapy by venous approach with a success rate of 76.9% per embolisation session. Two patients (10%) presented a permanent ocular paresis and two others a transient deficit. CVR is directly correlated with the thrombosis of multiple venous outflows of the CS. The "non-opacification" of at least three of the CS venous outflows is necessary for the development of CVR. Such thrombosis may be explained by the combination of haemodynamic and inflammatory changes of the venous wall.

Pituitary apoplexy masquerading as meningitis

Pituitary apoplexy is an uncommon condition and medical emergency that classically presents with abrupt onset of severe headache, nausea, fever, impaired level of consciousness, visual disturbances and a variable degree of ocular paresis. The constellation of signs, symptoms and cerebrospinal fluid findings in keeping with meningitis is considered a rare presentation of pituitary apoplexy and often leads to a misdiagnosis of infectious meningoencephalitis. We present the case of a 52 year old male admitted with a diabetic ketoacidosis who subsequently developed a severe headache with features of meningism. Haematological tests revealed an elevated white cell count and C-reactive protein, and lumbar puncture demonstrated a marked neutrophilic pleocytosis and elevated protein. Ceftriaxone was commenced for the presumptive diagnosis of bacterial meningitis, but resulted in no improvement in the patient’s condition and so imaging studies were performed which revealed the presence of an incidental pituitary ma...

Microepidemia familiar de botulismo alimentario en la Comunidad de Madrid

Botulism is a syndrome caused by the toxin of the bacillus Clostridium botulinum. The toxin acts by blocking the presynaptic cholinergic endings of the neuromuscular junction and of the parasympathetic nervous system, and gives rise to a flaccid paralysis and parasympathetic failure. The most common way to catch the disease is by ingestion of the preformed toxin present in badly sterilised home-made preserves, although other mechanisms are also possible. Its incidence in Spain today is very low. We report the case of three members of a family living together who presented a clinical picture of food-borne botulism. The initial clinical symptoms showed a predilection for ocular paresis and for dysautonomic symptoms of little specificity, and the familial aggregation was the fundamental evidence that suggested the diagnosis. Later, the patients' state got worse and two of them presented involvement of the respiratory function and required a lengthy stay in the intensive care unit. After a period of convalescence the three patients recovered without any sequelae. Botulinum toxin was detected by bioassay in some food samples, which allowed the diagnosis to be categorised as confirmed. The familial microepidemic reported here is a case of predominantly ocular and dysautonomic involvement. Likewise, it illustrates several aspects that are typical of the disease: the suspected diagnosis in cohabiting patients who visit at the same time for a similar clinical picture, the characteristic complications of the process and its treatment, the laboratory diagnosis and its natural history towards resolution.

Recurrent pituitary apoplexy due to two successive neoplasms presenting with ocular paresis and epistaxis.

SummaryA case of recurrent pituitary apoplexy is described in a 72-year-old man who initially presented with haemorrhage in a non-functioning pituitary adenoma. Five years later, he re-presented with a severe pituitary haemorrhage in an enlarging sellar mass invading both cavernous sinuses causing epistaxis and bilateral ocular paresis. Subsequent histology was consistent with a sellar malignant spindle and round cell neoplasm. Multiple pituitary tumours have previously been reported to coexist in the same individual, but to our knowledge this is the only case where two pathologically distinct pituitary neoplasms have sequentially arisen in a single patient. This case is also notable with respect to the progressive ocular paresis, including bilateral abducens nerve palsies, and the presentation with epistaxis.Learning points Ocular paresis in pituitary apoplexy can result from tumour infiltration of nerves, or by indirect compression via increased intrasellar pressure.Epistaxis is a very rare presentation of a pituitary lesion.Epistaxis more commonly occurs following trans-sphenoidal surgery, and can be delayed.

Partial Third Nerve Palsy and Ocular Neuromyotonia From Displacement of Posterior Communicating Artery Detected by High-Resolution MRI

Ocular neuromyotonia is an unusual condition in which sustained, undesired contraction of one or more extraocular muscles occurs after normal muscle activation. Although most commonly reported after paraseller cranial irradiation for tumor, chronic nonaneurysmal vascular compression of the third nerve can produce partial ocular motor nerve paresis and ocular neuromyotonia. A 75-year-old woman presented with intermittent left-gaze-evoked binocular diplopia. She had an incomplete right third nerve palsy but became symptomatically diplopic and esotropic upon sustained left gaze. High-resolution brain magnetic resonance imaging showed displacement of the right posterior communicating artery and contact with the right third nerve. Gaze-evoked diplopia resolved with carbamazepine, but a partial third nerve paresis remained.

Pituitary apoplexy mimicking meningitis.

Pituitary apoplexy is a rare but life-threatening disorder. Clinical presentation of this condition includes severe headaches, impaired consciousness, fever, visual disturbance, and variable ocular paresis. The clinical presentation of meningeal irritation is very rare. Nonetheless, if present and associated with fever, pituitary apoplexy may be misdiagnosed as a meningitis. We experienced a case of pituitary apoplexy masquerading as a meningitis. A 42-year-old man presented with meningitis associated symptoms and initial imaging studies did not show evidence of intra-lesional hemorrhage in the pituitary mass. However, a follow-up imaging after neurological deterioration revealed pituitary apoplexy. Hereby, we report our case with a review of literatures.

Pituitary apoplexy resembling acute meningitis without visual defect and ophthalmoplegia

Pituitary apoplexy is a rare but life-threatening disorder. The clinical presentation includes severe headache, impaired consciousness, fever, visual disturbances, variable ocular paresis and hypoadrenalism. It usually results from sudden hemorrhage or infarction-induced swelling in a pituitary adenoma. Signs of meningeal irritation are very rare. The presentation of headache, fever, pleocytosis and meningism might lead to a misdiagnosis of septic meningitis. We described a 71-year-old man who suffered from acute headache, fever, chills, dizziness and photophobia. Stiffness in the neck was notable and the cerebrospinal fluid study revealed neutrophilic pleocytosis. Empiric antibiotic therapy was administered for suspected septic meningitis but the symptoms persisted. Further brain imaging study showed pituitary adenoma with recent hemorrhage and the endocrine survey revealed a low cortisol level. Corticosteroid was added for pituitary apoplexy, and the patient recovered without surgical management. When a patient presents with fever, headache and meningeal irritation, it is important to include pituitary apoplexy in the differential diagnosis of infectious meningitis. Early treatment of pituitary apoplexy allows for aggressive endocrine management or neurosurgical decompression when required.