Oculosympathetic Palsy Research Articles

Fourth Nerve Paresis plus Crossed Horner Syndrome in Acute Leukemia

A 29-year-old patient with diplopia showed a unilateral fourth nerve palsy combined with a Horner syndrome on the opposite side. Magnetic resonance images disclosed a focal hemorrhage in the left dorsal midbrain, affecting the nucleus of the fourth nerve and adjacent oculosympathetic fibers. Further evaluations including a bone marrow biopsy confirmed acute lymphoblastic leukemia. The combination of ocular motor and oculosympathetic palsy has a distinct localizing value. Fourth nerve palsy ipsilateral to Horner syndrome locates lesion in the cavernous sinus, while contralateral fourth nerve palsy locates the lesion in the midbrain.

Window to the circulatory system: Ocular manifestations of cardiovascular diseases.

The changes in the cardiovascular system are associated with ocular manifestations, often as a consequence of pathological alteration in the ocular vasculature. The ease of visualization of these retinal changes makes the eye a window to the cardiovascular system. Certain congenital cardiac defects lead to changes in the retinal vascularity due to increased tortuosity and dilatation. In adults, the arterial dissection of internal carotid and vertebral arteries present as amaurosis fugax with or without oculo-sympathetic palsy. The patients with untreated infective endocarditis present with Roth spots, retinitis, embolic retinopathy, or sub-retinal abscesses. Hypoperfusive, hypertensive, or "mixed" retinopathy is a hallmark sign in patients of untreated infective endocarditis. Giant cell arteritis can present with ischemic ocular symptoms that may precipitate in irreversible vision loss. Systemic vascular manifestations such as coronary artery disease may manifest in a wide range of symptoms from amaurosis fugax to vision loss depending upon the size and location of retinal emboli. Rare cardio-oncological pathologies such as myxomas result in vision loss secondary to central retinal artery occlusion. A high clinical suspicion in patients with history of cardiovascular diseases can help in early diagnosis and management of impending, adverse cardiovascular and cerebrovascular events. In this review, we comprehensively discuss the spectrum of cardiac and vascular diseases with ocular manifestations as well as highlight the typical ocular presentations associated with these pathologies.

Diagnostic and management challenges of patients with recurrent tolosa hunt syndrome

Tolosa Hunt Syndrome (THS) includes painful ophthalmoplegia with periorbital or hemicranial pain, ipsilateral ocular motor nerve palsies, oculosympathetic paralysis, and sensory loss in the distribution of the ophthalmic and occasionally the maxillary division of the trigeminal nerve. The pathology is usualy localised to the region of the cavernous sinus/superior orbital fissure and may be usually due to trauma, neoplasm, aneurysm, and inflammation. Rarely patients may present with a relapsing and remitting course. Comprehensive patient evaluation is essential in establishing the correct diagnosis. Management is usually with systemic corticosteroid therapy and sometimes immunosuppression (IS) may be warranted. In this abstract we aim to present cases with recurrent THS and further discuss management strategies and diagnostic challenges. Case series: We would like to report 3 patients (2 females, ages between 36 and 45) with recurrent THS. The relapse ocurred within the first year after the initial presentation and patients were all treated with high dose steroids and IS (azaotioprine). Patients have been relapse free with IS and slow corticosteroid tapering. THS is a diagnosis of exclusion and careful diagnostic approach is warranted.

Ocular manifestations of internal carotid artery dissection.

Internal carotid artery dissection (ICAD) results from disruption of the intima of the arterial wall, and can lead to intrusion of blood into the arterial wall and form an intramural hematoma. The hematoma can compress the true lumen of the vessel, causing functional stenosis or occlusion. The classic triad signs of ICAD include pain in the ipsilateral neck, head and orbital regions; a (partial) Horner syndrome; and cerebral or retinal ischemia. However, not all ICAD patients present with this classic signs. In some cases, ocular manifestations are the initial (and sometimes the only) findings. We summarize the ocular manifestations associated with ICAD in 3 categories: visual symptoms, oculosympathetic palsy, and ocular motor nerve palsy.

Internal Carotid Artery Dissection With Lidocaine Nerve Block During Dental Procedure: A Case Report

SESSION TITLE: Nervous System Disorders in the ICU 1 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Internal carotid artery dissection (ICAD) represents an important cause of cerebrovascular accidents in young and middle-aged patients. In this case report,we aim to describe this rare but potentially life threatening cause of ICA dissection and its unusual presentation. CASE PRESENTATION: 58year old female presented with severe occipital headache and right sided neck pain associated with nausea and lethargy from one week.Denied any head or neck trauma.On examination patient had constricted pupil and ptosis affecting right eye with a blood pressure of 206/98 mmhg and heart rate 59/min. Symptoms started immediately after receiving 2% lidocaine injection for pulpotomy of upper right second bicuspid tooth which was followed by incision and drainage completed later for the dental abscess. Computed tomography angiography revealed right ICA dissection at level of C1-C2 associated with infiltrative changes within carotid space and retropharyngeal space without abscess. Intravenous heparin was started and blood pressure was lowered over 25% every day. Heparin was continued for three days and later switched to xarelto. During the course of hospitalization miosis and ptosis improved and patient discharged home on xarelto. DISCUSSION: ICAD was first recognized in 1947,when Dratz published dissection as a complication from direct injection to the carotid artery(1)Typical signs and symptoms includes pain on one side of the head,face or neck accompanied by a partial Horner's syndrome (oculosympathetic palsy- consists of miosis and ptosis)Facial anhidrosis is absent as the facial sweat glands are innervated by the sympathetic plexus surrounding the external carotid artery. A similar case has been reported in literature by Francesco De Santis et al (2) Understanding the pathogenesis we can state that although carotid artery is anatomically relatively distant from the anesthetic block target areas but there is always a possibility of a needle insertion more posteriorly and medially.Further,cervical hyperextension during a dental procedure can compress the cervical internal carotid artery against upper cervical vertebra and facilitate the needle reaching submandibular capsule.Periodontal infections associated with secretion of inflammatory cytokines,free radicals and proteases which further lead to vessel wall weakening and increased susceptibility for rupture. CONCLUSIONS: ICAD developing after dental work is a relatively rare scenario and we hope that this case report can help intensivists better recognize this uncommon cause of this life threatening disease. Reference #1: Dratz HMW,Traumatic Dissecting Aneurysm of Left Internal Carotid, Anterior Cerebral and Middle Cerebral Arteries.Journal of Neuropathology & Experimental Neurology. 1947;6(3):286-291. Reference #2: Francesco De Santiset al:Internal Carotid Artery Dissection After Inferior Alveolar Nerve Block for Third Molar Dental Care Presented as Hypoglossal Nerve Palsy:Vascular and Endovascular Surgery 46(7) DISCLOSURE: The following authors have nothing to disclose: Naureen Narula, Faraz Siddiqui, Michel Chalhoub No Product/Research Disclosure Information

Autres névralgies : névralgie occipitale, syndrome de la bouche brûlante, neuropathie ophtalmoplégique récurrente douloureuse, syndrome de Tolosa-Hunt

Occipital neuralgia is defined as an electricshock- type or stabbing pain in the area of the occipital nerves. There are several types of treatment available: medical (anticonvulsant or tricyclic agents), transitional (infiltrations) or surgical (neurolysis or stimulation). Burning mouth syndrome is a chronic and spontaneous burning-type pain to the mouth, which may affect the tongue and/or other oral mucosae, with no identifiable injuries. Recurrent ophthalmoplegic cranial neuropathy is a rare neurological syndrome characterised by the onset of recurrent headaches and ophthalmoplegia, affecting the third and/or sixth cranial nerves. Tolosa-Hunt syndrome occurs as a periorbital or hemicranial pain associated with ipsilateral paresis of the oculomotor nerves, oculosympathetic paralysis and a loss of sensitivity in the distribution of the trigeminal nerve branches.

Spontaneous Arterial Dissection—Risk Factors And Clinical Features: Study Of 70 Cases And Review Of Literature

Dissection refers to a serious condition that begin as a tear or rupture in the inner layers of large vessel wall. Tear allows the blood to surges through the layers, leads to intramural hematoma or an aneurysmal dilatation, either of which can be a source of thromboembolism. Ischemic symptoms in surrounding structures are more often secondary to embolism. Dissections are labeled spontaneous in the absence of major blunt or penetrating trauma. The incidence rates for spontaneous carotid artery dissection have been reported to be 2.5 to 3 per 100,000 population. The average annual incidence for vertebral artery dissection were calculated to be 1 to 1.5 per 100,000. Typical clinical features seen with dissection include unilateral headache, ipsilateral Homer's syndrome, oculosympathetic palsy, prominent neck and facial pain, retinal ischemia, and symptoms of focal brain ischemia due to the site of dissection. The reason of dissection occurrence is still unknown but numerous factors e.g. connective tissue disorders, hyperhomocystinemia, fibromuscular dysplasia, recent infection, etc. may underlie spontaneous or minor trauma dissections. Patients with Spontaneous dissection are more susceptible to developing to stroke and its morbidity and mortality. In this retrospective study, we evaluate the clinical features, risk factors, treatment and outcome of spontaneous dissection in 70 patients (31 female and 39 male), referred to Namazi hospital between September 2012 and September 2016. The diagnosis of arterial dissection was established by qualified physicians according to radiological and clinical features. Patients with traumatic dissection and a history of major or minor head and neck injury were excluded. Subjects were observed for multiple risk factors such as age dependent risk factors, family history of venous thrombosis, history of trauma, coagulopathies, infectious diseases, systemic disorders, arterio-venous malformations and autoimmune diseases. We also investigated for any history of OCP administration in female patients. The collected data was evaluated using SPSS 16. Chi-square test were performed for the comparison of qualitative variables. And p<0.05 was considered as the level of significance in two-tailed tests. Statistical analysis of our data found a significant correlation between hypertension and occurrence of spontaneous arterial dissection. The findings confirm that hypertension, OCP administration and smoking can increase the risk of CVST significantly.

Congenital horner syndrome with heterochromia iridis associated with ipsilateral internal carotid artery hypoplasia.

BackgroundHorner syndrome (HS), also known as Claude-Bernard-Horner syndrome or oculosympathetic palsy, comprises ipsilateral ptosis, miosis, and facial anhidrosis.Case ReportWe report herein the case of a 67-year-old man who presented with congenital HS associated with ipsilateral hypoplasia of the internal carotid artery (ICA), as revealed by heterochromia iridis and confirmed by computed tomography (CT).ConclusionsCT evaluation of the skull base is essential to establish this diagnosis and distinguish aplasia from agenesis/hypoplasia (by the absence or hypoplasia of the carotid canal) or from acquired ICA obstruction as demonstrated by angiographic CT.

Cervical arterial dissections and association with cervical manipulative therapy: a statement for healthcare professionals from the american heart association/american stroke association.

Cervical artery dissections (CDs) are among the most common causes of stroke in young and middle-aged adults. The aim of this scientific statement is to review the current state of evidence on the diagnosis and management of CDs and their statistical association with cervical manipulative therapy (CMT). In some forms of CMT, a high or low amplitude thrust is applied to the cervical spine by a healthcare professional. Members of the writing group were appointed by the American Heart Association Stroke Council's Scientific Statements Oversight Committee and the American Heart Association's Manuscript Oversight Committee. Members were assigned topics relevant to their areas of expertise and reviewed appropriate literature, references to published clinical and epidemiology studies, morbidity and mortality reports, clinical and public health guidelines, authoritative statements, personal files, and expert opinion to summarize existing evidence and to indicate gaps in current knowledge. Patients with CD may present with unilateral headaches, posterior cervical pain, or cerebral or retinal ischemia (transient ischemic or strokes) attributable mainly to artery-artery embolism, CD cranial nerve palsies, oculosympathetic palsy, or pulsatile tinnitus. Diagnosis of CD depends on a thorough history, physical examination, and targeted ancillary investigations. Although the role of trivial trauma is debatable, mechanical forces can lead to intimal injuries of the vertebral arteries and internal carotid arteries and result in CD. Disability levels vary among CD patients with many having good outcomes, but serious neurological sequelae can occur. No evidence-based guidelines are currently available to endorse best management strategies for CDs. Antiplatelet and anticoagulant treatments are both used for prevention of local thrombus and secondary embolism. Case-control and other articles have suggested an epidemiologic association between CD, particularly vertebral artery dissection, and CMT. It is unclear whether this is due to lack of recognition of preexisting CD in these patients or due to trauma caused by CMT. Ultrasonography, computed tomographic angiography, and magnetic resonance imaging with magnetic resonance angiography are useful in the diagnosis of CD. Follow-up neuroimaging is preferentially done with noninvasive modalities, but we suggest that no single test should be seen as the gold standard. CD is an important cause of ischemic stroke in young and middle-aged patients. CD is most prevalent in the upper cervical spine and can involve the internal carotid artery or vertebral artery. Although current biomechanical evidence is insufficient to establish the claim that CMT causes CD, clinical reports suggest that mechanical forces play a role in a considerable number of CDs and most population controlled studies have found an association between CMT and VAD stroke in young patients. Although the incidence of CMT-associated CD in patients who have previously received CMT is not well established, and probably low, practitioners should strongly consider the possibility of CD as a presenting symptom, and patients should be informed of the statistical association between CD and CMT prior to undergoing manipulation of the cervical spine.

Tolosa-Hunt Syndrome: A Case Report and Review of the Literature

Tolosa-Hunt Syndrome is a painful ophthalmoplegia which is characterized by periorbital or hemicranial pain, with ipsilateral ocular motor nerve palsies, oculosympathetic paralysis, sensory loss in the distribution of the ophthalmic and occasionally the maxillary division of the trigeminal nerve. Various combinations of these cranial nerve palsies may occur, localising the pathological process to the region of the cavernous sinus/superior orbital fissure. We report the case of a patient presented with severe pain in the right side of face which was periorbital with ipsilateral 3rd,4th, 6th cranial nerve palsies along with ophthalmic and maxillary division of trigeminal nerve involvement. MRI of orbit showed hypo-intense lesion in right cavernous sinus extending to right superior orbital fissure (suggestive of granulomatous infiltration). After taking oral steroid her pain was relieved quickly and cranial nerve palsies reversed within one week. Azathioprin was added and she was completely cured of within next three months.
 Bangladesh Journal of Neuroscience 2013; Vol. 29 (2) : 130-133

English

Tolosa-Hunt syndrome (THS) is a painful ophthalmoplegia caused by nonspecific inflammation of the cavernous sinus or superior orbital fissure. The syndrome consists of periorbital or hemicranial pain, combined with ipsilateral ocular motor nerve palsies, oculosympathetic paralysis, and sensory loss in the distribution of the ophthalmic and occasionally the maxillary division of the trigeminal nerve. Although they have relapsing and remitting course, they respond promptly to systemic corticosteroid therapy. The diagnostic eponym Tolosa-Hunt syndrome has been applied to these patients and it is this entity which forms the basis of this review. CASE REPORT: A 42 year old man presented with painful diminution of vision since one month and diplopia in the left eye since 7 days. Tingling sensation was noted over left half of upper face. On clinical examination –V/A was 6/12 (B/E) improving to 6/6 with pinhole, IOP and fundus was normal both eyes, extra ocular movement was completely restricted in left eye with painful movements. Left III, IV and VI Cranial nerve palsies were noted with decreased sensation over the area supplied by V Cranial Nerve. The patient was sent for an MR examination. MR imaging was performed on a 1.5 Tesla MR scanner (GE). 10ml Gadolinium was injected in the left antecubital vein to obtain post contrast T1WI.Study revealed moderately enhancing soft tissue in the region of left cavernous sinus and orbital apex. It was isointense to muscle on T1WI and hypo intense to fat on T2WI. MANAGEMENT: On the basis of history and imaging findings, a diagnosis of THS was made and the patient was started on corticosteroid therapy: Injection Methyl-Prednisolone (1gm.I.V for 3 days).Dramatic improvement in diplopia and pain was noted within 48 hours of starting IV steroid therapy. Oral Prednisolone 40mg daily was started after 3 days and continued for 6 weeks. At the end of therapy the patient's ophthalmoplegia also recovered. DISCUSSION: Tolosa–Hunt syndrome (THS) is a rare disorder characterized by severe and unilateral headaches with extra ocular palsies, usually involving the third, fourth, fifth, and sixth cranial nerves, and pain around the sides and back of the eye, along with weakness and paralysis (ophthalmoplegia) of certain eye muscles1. The syndrome of painful ophthalmoplegia consists of periorbital or hemicranial pain, combined with ipsilateral ocular motor nerve palsies, oculosympathetic paralysis, and sensory loss in the distribution of the ophthalmic and occasionally the maxillary division of the

Spontaneous dissection of the internal carotid artery: description of a case with lower cranial nerve palsy

Spontaneous dissection of the extracranial internal carotid artery is a well recognized cause of headache and juvenile stroke; lower cranial nerve palsy as a complication of dissection is rare. We report the case of a female patient with bilateral dissecting aneurysm of the internal carotid artery, associated with unilateral cranial nerve XII palsy and oculosympathetic palsy. Neuroradiological findings, in particular those obtained by Magnetic Resonance imaging, allow the identification of the dissecting pathology and the correlation of the aneurysmal formation with nerve palsy.

Carotid artery dissection

Carotid artery dissections are potentially disabling, probably underdiagnosed, and mainly affect young-aged and middle-aged people. We present three consecutive cases illustrating different clinical presentations and thereby emphasizing the diagnostic challenge of carotid artery dissections for the emergency physician. Neck and facial pain, headache, unilateral pulsatile tinnitus, partial Horner's syndrome (or oculosympathetic palsy), amaurosis fugax, retinal infarction, and anterior circulation brain ischemia may all occur in isolation or in various combinations. Medical imaging plays a pivotal role in making the right diagnosis. Clinical vigilance is of utmost importance as early diagnosis and timely treatment favor long-term prognosis and even prevent ischemic complications. We review the literature and discuss the pathophysiology, etiology, clinical presentation, diagnosis, imaging techniques, treatment, and prognosis of carotid dissections.

Painful oculosympathetic palsy as the sole presentation of spontaneous dissection of the distal internal carotid artery

Spontaneous dissection of the carotid artery is an important differential diagnosis to consider in cases of early onset stroke. Its natural history is variable, ranging from a benign course to permanent disability or death. Dissection usually occurs in the cervical portion of the artery, rendering it amenable to surgical therapy. These issues are compounded if there is intrapetral extension, with associated mortality rates up to 75%. The authors describe such a case of spontaneous dissection and discuss imaging and therapy issues that result.

RAEDER PARATRIGEMINAL SYNDROME IN A PATIENT WITH A MASS LESION IN THE MAXILLARY SINUS

SUMMARY Raeder paratrigeminal syndrome is a rare syndrome, characterized by severe unilateral facial pain and headache in the distribution of the ophthalmic division of the trigeminal nerve in combination with ipsilateral oculosympathetic palsy or Horner syndrome. We describe a case of a 65-year-old male patient with a large tumor in the right maxillary sinus who presented with the rare Raeder syndrome.

Pharmacologic testing in Horner's syndrome – a new paradigm

For more than three decades, topical cocaine has been used to confirm the diagnosis and hydroxyamphetamine to localise the causative lesion in oculosympathetic palsy or Horner's syndrome. More recently, other drugs have demonstrated the ability to point to the diagnosis or anatomical site. Apraclonidine and phenylephrine, given their similar diagnostic efficacy and increased availability, may have superseded cocaine and hydroxyamphetamine as first-line pharmacological testing agents in Horner's syndrome.

Horner’s Syndrome and Neuroblastoma: Our Family’s Odyssey with Disorders of the Sympathetic Nervous System

September 11, 2010: the anniversary of a national tragedy and a date that changed our family forever. While at my son’s soccer game, my wife, Stacey, realized something was not quite right with Paige, our 17 month old daughter. It was a scorcher of a day, and Paige began sweating and became flushed but only on the left side of her head. It was as if someone had drawn a line down the middle of her face and one side was bright red and the other pale. Stacey immediately told me that we have an issue with Paige’s sympathetic nervous system and that she was presenting with what she thought was Horner’s syndrome (1). What? You see, Stacey is in Physical Therapy School and a former medicinal chemist at Merck, and her Professor covered Horner’s syndrome the day before in Neuroanatomy: this proved to a very fortunate timing of events and one that probably saved our daughter’s life. Horner’s syndrome, also referred to as Bernard−Horner syndrome or oculosympathetic palsy, is acquired by damage to or pressure on the sympathetic nervous system (1). The clinical features of Horner’s syndrome include ptosis (drooping eyelid), anhidrosis (decreased sweating on the affected side of face), and miosis (constricted pupil) (1). On closer inspection of our daughter, both the ptosis and miosis were discernible as well. Looking back through photographs, we were able to identify that the miosis was present in photos from late June of 2010, but not before; therefore, the Horner’s syndrome was relatively recent. While not life-threatening, we scrambled to find the cause and quickly ran through the list of known causes, ruling one out after the other and quickly finding ourselves facing unthinkable culprits.

Painful ophthalmoplegia of the left eye in a 19-year-old female, with an emphasis in Tolosa-Hunt syndrome: a case report

IntroductionPainful ophthalmoplegia refers to periorbital or hemicraneal pain plus ipsilateral ocular motor nerve palsies with or without oculo-sympathetic paralysis, sensory loss in the distribution of V1 and V2 can co-occur. There are many etiologies of painful ophthalmoplegia. Tolosa-Hunt syndrome is a steroid-responsive painful ophthalmoplegia secondary to idiopatic granulomatous inflammation of the cavernous sinus or orbital apex. THS is a diagnosis of exclusion and treatment should be with high dose steroid.Case presentationWe describe the case of a 19-year-old female that was admitted to our hospital for painful ophthalmoplegia of the left eye. After the diagnostic work-up, we concluded that the patient had a benign form of Tolosa-Hunt syndrome. We initiated treatment with steroids and 72 hours later saw a response.ConclusionIn conclusion, steroid treatment is the cornerstone in the management of THS. Even though there is no standardized dose specified in the literature, this type of treatment with steroids at a dose of 1 mg/kg/day tapered slowly over 3 to 4 months has been well received.

Bilateral Carotid Artery Dissection After Dental Work

Carotid artery dissection has been reported to occur spontaneously and after many types of neck trauma. A case patient is presented who developed a right-sided oculosympathetic palsy and was found to have bilateral carotid artery dissection after she visited her dentist. To the best of our knowledge, this case represents the first report of bilateral carotid dissection following dental work.

Oculosympathetic Paratrigeminal Paralysis With Isolated V2 Involvement in Carotid Artery Dissection

Oculosympathetic Paratrigeminal Paralysis With Isolated V2 Involvement in Carotid Artery Dissection