Síndrome de ápex orbitario por herpes zoster ophthalmicus / Orbital apex syndrome secondary to herpes zoster ophthalmicus

El herpes zoster ophthalmicus suele cursar con manifestaciones oculares, siendo mucho más infrecuentes las complicaciones de tipo neurológico. Una mujer de 84 años con herpes zóster en el dermatomo de la primera rama trigeminal izquierda, desarrolló una queratouveítis herpética en el ojo izquierdo a pesar del tratamiento con valaciclovir oral. A los siete días, además apareció una oftalmoplejia progresiva y total izquierda que requirió ingreso hospitalario y tratamiento intravenoso con aciclovir y corticoides. La neuroimagen fue sugestiva de un síndrome de ápex orbitario. La evolución de la oftalmoplejia fue favorable con resolución completa a los cinco meses de seguimiento, dejando una disminución de la agudeza visual por la afectación del nervio óptico. (AU)

Herpes zoster ophthalmicus usually presents with ocular manifestations, but neurological complications are much more infrequent. An 84-year-old woman with herpes zoster of the left first trigeminal branch developed herpetic keratouveitis in her left eye despite treatment with oral valaciclovir. Seven days later, a progressive and total left ophthalmoplegia appeared, requiring hospital admission and intravenous treatment with acyclovir and corticosteroids. The neuroimaging was suggestive of an orbital apex syndrome. The evolution of the ophthalmoplegia was favourable, with complete resolution at 5 months, but with decreased visual acuity due to the optic nerve involvement. (AU)

Cranial nerve involvement in COVID-19.

INTRODUCTION: The COVID-19 disease emerged in Wuhan province of China in November 2019 and spread across the world in a short time, resulting in a pandemic. The first case in Turkey was detected on March 11, 2020. The aim of the current study was to reveal the effects of COVID-19 on cranial nerves by monitoring people infected with the disease based on repeated examinations and surveys. MATERIAL AND METHOD: The data of 356 patients with a positive COVID-19 polymerase chain reaction (PCR) test who received treatment between June 2020 and August 2020 in our hospital were prospectively evaluated after the study was approved by the relevant ethics committee. RESULTS: Of the 356 patients included in the study, 47 under the age of 18 years were excluded due to their unreliable examination and anamnesis findings. In addition, seven patients that died while in hospital were excluded from the study due to the lack of examination and survey records during their hospitalization. The data of the remaining 302 patients were statistically analyzed. Symptoms of cranial nerve involvement were observed in 135 patients. CONCLUSION: The COVID-19 disease caused by the SARS-CoV2 virus commonly results in cranial nerve symptoms. The fact that these findings are more common and severe in COVID-19 than previous SARS and MERS outbreaks suggests that it has a more neurotrophic and more aggressive neuroinvasion. While the negative effects of the virus on sensory functions resulting from cranial nerve involvement are evident, motor functions are rarely affected.

Magnetic resonance imaging features of COVID-19-related cranial nerve lesions.

The complete features of the neurological complications of coronavirus disease 2019 (COVID-19) still need to be elucidated, including associated cranial nerve involvement. In the present study we describe cranial nerve lesions seen in magnetic resonance imaging (MRI) of six cases of confirmed COVID-19, involving the olfactory bulb, optic nerve, abducens nerve, and facial nerve. Cranial nerve involvement was associated with COVID-19, but whether by direct viral invasion or autoimmunity needs to be clarified. The development of neurological symptoms after initial respiratory symptoms and the absence of the virus in the cerebrospinal fluid (CSF) suggest the possibility of autoimmunity.

Polyneuritis cranialis and vasculopathy caused by varicella zoster virus without rash.

The neurological disorders caused by Varicella Zoster Virus (VZV) in the absence of skin rash are a challenge to the clinician. The presentation varies from acute to subacute to chronic. Reactivation of VZV usually produces zoster (shingles), meningitis or meningoencephalitis, cerebellitis, isolated or multiple cranial nerve palsies (polyneuritis cranialis), myelitis, and vasculopathy. In our case, we report a 41-year-old female presented with right oculomotor, vestibulocochlear and facial neuropathies occurred 1 year before admission and making the diagnosis. There were no skin or mucosa lesions. Magnetic Resonance Imaging revealed multiple subcortical infractions in the right temporal and occipital lobes which consist with silent vasculopathy. The diagnosis was confirmed by the existence of anti-VZV IgG in cerebrospinal fluid (CSF).

Evaluation of the Incidence of Other Cranial Neuropathies in Patients With Postviral Olfactory Loss.

Importance: Postviral olfactory loss is a common cause of olfactory impairment, affecting both quality of life as well as overall patient mortality. It is currently unclear why some patients are able to recover fully after a loss while others experience permanent deficit. There is a lack of research on the possible association between postviral olfactory loss and other cranial neuropathies. Objective: To evaluate the incidence of other cranial nerve deficits in patients with postviral olfactory loss and determine if there is an association with neurologic injury in this group. This study also sought to determine if other known risk factors were associated with postviral olfactory loss. Design, Setting, and Participants: A case-control study was conducted at a tertiary care rhinology clinic from January 2015 to January 2018 to review the incidence of cranial neuropathies in 2 groups of patients, those with postviral olfactory loss and those with chronic rhinosinusitis without olfactory loss used as a control group. Exposures: The Stanford Translational Research Integrated Database Environment (STRIDE) system was used for patient identification and data extraction. Patients with a history of olfactory loss or chronic rhinosinusitis as well as incidence of cranial neuropathies were identified by using International Statistical Classification of Diseases and Related Health Problems, Tenth Revision (ICD-10) codes. Main Outcomes and Measures: This study reviewed incidence of postviral or idiopathic cranial neuropathies in both patient groups, while also evaluating for any difference in demographic characteristics, comorbidities, or other patient-related factors. Results: There were 91 patients in the postviral olfactory loss group and 100 patients in the control group, which were age and sex matched as closely as possible. Of the 91 patients with postviral olfactory loss, mean (SD) age was 56.8 (15.3), and 58 (64%) were women; for the control group, the mean (SD) age was 57.5 (15.6) years, and 63 (63%) were women. Racial breakdown was similar across cases and controls, with white individuals making up 59% to 65%; Asian individuals, 20% to 24%; black individuals, approximately 3%; Hispanic individuals, approximately 1%; and the remaining patients being of other race/ethnicity. The incidence of other cranial neuropathies in the postviral olfactory loss group was 11% compared with 2% within the control group (odds ratio, 6.1; 95% CI, 1.3-28.4). The study also found 2 cases of multiple cranial neuropathies within a single patient within the olfactory group. Family history of neurologic disease was associated with more than 2-fold greater odds of cranial nerve deficit (odds ratio, 3.05; 95% CI, 0.59-15.68). Conclusions and Relevance: Postviral olfactory loss appears to be associated with a higher incidence of other cranial neuropathies. It is possible that there is an inherent vulnerability to nerve damage or decreased ability for nerve recovery in patients who experience this disease process.

Herpes simplex virus type 2 meningitis as a manifestation of Good's syndrome.

Good's syndrome is a primary immunodeficiency phenocopy characterized for thymoma and immunodeficiency. The most frequent clinical presentation is recurrent or opportunistic infections, hematological alterations, and chronic diarrhea. We treated a 66-year-old man who consulted for 5 days of headache and diplopia with right sixth cranial nerve palsy at examination. Patient reported chronic diarrhea and prolonged febrile syndrome accompanied by weight loss of 23 kg in the last year. Exhaustive evaluation revealed Herpes simplex virus (HSV) type 2 meningitis, eosinophilic colitis, and type A thymoma. Severe antibody deficiency (hypogammaglobulinemia) associated with thymoma confirmed the diagnosis of Good's syndrome.

Varicella zoster virus (VZV) oculomeningoencephalomyeloradiculitis: a previously undescribed constellation.

A 53-year-old immunocompromised woman developed acute left eye blindness and paraparesis suspected to be due to neuromyelitis optica (NMO). During treatment for NMO, right eye blindness and progressive multiple cranial neuropathies developed. Cerebrospinal fluid polymerase chain reaction (PCR) revealed Varicella zoster virus (VZV). This case emphasizes the importance of considering VZV in individuals, particularly the immunocompromised, presenting with a constellation of neurological signs and symptoms, even in the absence of rash.

Varicella zoster presenting as cranial polyneuropathy.

Cranial polyneuropathy is commonly caused by Lyme disease. We discuss the case of a man who presented with cranial nerve deficits causing dysphagia, dysphonia and facial weakness. This diagnostic dilemma stemmed from a workup that ruled out Lyme and vascular causes leading to an expanded search for infectious explanations, which revealed varicella zoster in the cerebrospinal fluid. On review, this phenomenon is rarely reported, but has been observed with a number of herpes family viruses. In emergency department settings, clinical suspicion should be raised for VZV infection even in the absence of rash in patients that present with multiple cranial nerve palsies.

Ischemic Stroke due to Virologically-Confirmed Varicella Zoster Virus Vasculopathy: A Case Series.

BACKGROUND: Limited data are available regarding the characteristics and prognosis of patients with stroke due to varicella zoster virus (VZV) vasculopathy. METHODS: We studied 4 patients (2 men and 2 women; age, 38-63 years) from a single center who developed acute ischemic stroke due to VZV vasculopathy. The virological diagnosis was confirmed by detecting VZV DNA and/or the IgG antibody to VZV in the cerebrospinal fluid. RESULTS: Three patients were taking immunosuppressive agents, including prednisolone and/or methotrexate, at baseline. Each patient had a characteristic skin rash prior to stroke, with the interval from rash to stroke onset ranging from 13 to 122 days. Two patients experienced antecedent cranial nerve palsies; one had the third, seventh, ninth, and 10th nerve palsies and the other had the fourth nerve palsy before stroke. Cerebral infarctions were located in the anterior circulation lesion (n = 1), in the posterior circulation lesion (n = 2), and in both lesions (n = 1). Intracranial arterial stenosis was only identified in one patient on magnetic resonance angiography. A high plasma d-dimer level was detected in 1 patient, whereas high ß-thromboglobulin and platelet factor 4 levels were detected in 2 patients. As a result of combined therapies with acyclovir, steroid, and antithrombotic agents, neurological symptoms markedly improved in 3 patients, whereas 1 patient was left with moderate hemiplegia. CONCLUSIONS: Cranial nerve palsies may be prodromal symptoms of VZV-associated stroke. Increased levels of thrombotic markers may support the use of antithrombotic agents, although the benefit of combined treatment should be determined through larger studies.

Treatment of Ramsay-Hunt's syndrome with multiple cranial nerve involvement and severe dysphagia: A case report.

RATIONALE: Ramsay-Hunt's syndrome (RHS) is a disorder characterized by facial paralysis, herpetic eruptions on the auricle, and otic pain due to the reactivation of latent varicella zoster virus in the geniculate ganglion. A few cases of multiple cranial nerve invasion including the vestibulocochlear nerve, glossopharyngeal nerve and vagus nerve have been reported. However, there has been no report about RHS with delayed onset multiple cranial nerve involvement causing severe aspiration, and a clinical course that improved after more than one year of dysphagia rehabilitation and percutaneous endoscopic gastrostomy (PEG). Here, we report on a 67-year old male with delayed onset swallowing difficulty after 16 days of RHS development. PATIENT CONCERN: Severe aspiration during swallowing. DIAGNOSIS: Severe dysphagia caused by RHS with multiple cranial nerve involvement. INTERVENTION: Application of percutaneous endoscopic gastrostomy (PEG) and rehabilitation therapy of dysphagia. OUTCOMES: After 13 months from symptom onset, his PAS improved from 7 to 2 in follow-up video-fluoroscopic swallowing study (VFSS). Then, he was re-admitted, and the PEG tube was removed and oral feeding was started. LESSONS: This case gives us the lesson that optimal doses of acyclovir and corticosteroids are important to prevent progression of multiple cranial involvement in RHS, and swallowing difficulty in RHS patients with multiple cranial nerve involvement can be improved through long-term rehabilitation even if there is no improvement for more than one year.

Vernet syndrome resulting from varicella zoster virus infection-a very rare clinical presentation of a common viral infection.

Vernet syndrome is a unilateral palsy of glossopharyngeal, vagus, and accessory nerves. Varicella zoster virus (VZV) infection has rarely been described as a possible cause. A 76-year-old man presented with 1-week-long symptoms of dysphonia, dysphagia, and weakness of the right shoulder elevation, accompanied by a mild right temporal parietal headache with radiation to the ipsilateral ear. Physical examination showed signs compatible with a right XI, X, and XI cranial nerves involvement and also several vesicular lesions in the right ear's concha. He had a personal history of poliomyelitis and chickenpox. Laringoscopy demonstrated right vocal cord palsy. Brain MRI showed thickening and enhancement of right lower cranial nerves and an enhancing nodular lesion in the ipsilateral jugular foramen, in T1 weighted images with gadolinium. Cerebrospinal fluid (CSF) analysis disclosed a mild lymphocytic pleocytosis and absence of VZV-DNA by PCR analysis. Serum VZV IgM and IgG antibodies were positive. The patient had a noticeable clinical improvement after initiation of acyclovir and prednisolone therapy. The presentation of a VZV infection with isolated IX, X, and XI cranial nerves palsy is extremely rare. In our case, the diagnosis of Vernet syndrome as a result of VZV infection was made essentially from clinical findings and supported by analytical and imaging data.

A Rare Presentation of Cranial Polyneuropathy Without Rash Caused by Varicella Zoster Virus.

INTRODUCTION: Varicella Zoster Virus (VZV) is associated with many disorders of the central and peripheral nervous systems including neuralgia, meningitis, meningoencephalitis, cerebellitis, vasculopathy, myelopathy, Ramsay-Hunt syndrome, and polyneuritis cranialis. Cranial nerves V, VI, VII, VIII, IX, X, XI, and/or XII may be affected. The neurological disorders caused by VZV usually present with rash, but may rarely present without rash. CASE REPORT: We herein present a case of polyneuritis cranialis without rash caused by VZV affecting cranial nerves VII, VIII, IX, and X. After excluding other causes of the condition, we diagnosed VZV infection based on VZV DNA in the CSF and an elevated anti-VZV IgG level in serum. The patient responded well to antiviral therapy. CONCLUSION: VZV infection should be kept in mind during the differential diagnosis of polyneuritis cranialis; it is important to note that VZV re-activation may occur without rash.

Ramsay Hunt syndrome with cranial polyneuropathy with features of supraglottitis.

A 67-year-old woman presented with a 1-week history of left otalgia and a 1-day history of odynophagia, pain extending into the face and neck, and a productive cough. Flexible nasendoscopy showed features of supraglottitis, with swollen arytenoids and pooling of saliva in the piriform fossae. Laboratory investigations revealed a mildly raised C reactive protein. A CT scan of the neck supported the diagnosis of supraglottitis and pharyngitis, with thickening of the mucosa of the left piriform fossae and left oropharynx. Standard supraglottitis treatment was instigated, but on day 4 of the admission, a vesicular rash and features of cranial nerve involvement (V, VII, VIII, X) developed. A revised diagnosis of Ramsay Hunt syndrome with cranial polyneuropathy was made and later confirmed by varicella zoster virus PCR. After 4 weeks, facial nerve function normalised, but features of other cranial nerve palsies were persistent.

Diplopia as isolated presentation of varicella zoster central nervous system reactivation.

Here, we report a patient who developed diplopia secondary to a right cranial nerve III and IV palsy, as well as fever and headache. Cerebrospinal fluid analysis (CSF) showed high varicella-zoster virus (VZV)-DNA viral load (>300,000,000 copies/ml). VZV antibodies in CSF was ≥1:16. Diagnosis of neurological reactivation of VZV infection was made without the presence of characteristic vesicular rash. Quantitative real-time PCR for VZV and intrathecal dosage of VZV IgM and IgG should be performed in cases suspected for viral encephalitis and also in all patients with not otherwise attributable cranial nerve lesions.