Herpes zoster in neuro-ophthalmology: a practical approach.

Herpes Zoster (HZ) or shingles is the reactivation of the Varicella Zoster Virus (VZV), usually along a single sensory nerve, but can affect both sensory and motor cranial nerves. Major risk factors for HZ include immunosuppressed status and age older than 60 years. In the United States, the lifetime risk of HZ is approximately 30%. Worldwide, the median incidence of HZ is 4-4.5 per 1000 person-years across the Americas, Eurasia, and Australia. HZ ophthalmicus, occurring in 10-20% of patients, is an ophthalmic emergency characterized by VZV reactivation along the V1 branch of the trigeminal nerve. Approximately half of this patient subgroup will go on to develop ocular manifestations, requiring prompt diagnosis and management. While anterior segment complications are more common, neuro-ophthalmic manifestations are rarer and can also occur outside the context of overt HZ ophthalmicus. Neuro-ophthalmic manifestations include optic neuropathy, acute retinal necrosis or progressive outer retinal necrosis, cranial neuropathy (isolated or multiple), orbitopathy, and CNS manifestations. Although typically a clinical diagnosis, diagnosis may be aided by neuroimaging and laboratory (e.g., PCR and serology) studies. Early antiviral therapy is indicated as soon as a presumptive diagnosis of VZV is made and the role of corticosteroids remains debated. Generally, there is wide variation of prognosis with neuro-ophthalmic involvement. Vaccine-mediated prevention is recommended. In this review, we summarize neuro-ophthalmic manifestations of VZV.

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.

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.

Late Ebola virus relapse causing meningoencephalitis: a case report.

BACKGROUND: There are thousands of survivors of the 2014 Ebola outbreak in west Africa. Ebola virus can persist in survivors for months in immune-privileged sites; however, viral relapse causing life-threatening and potentially transmissible disease has not been described. We report a case of late relapse in a patient who had been treated for severe Ebola virus disease with high viral load (peak cycle threshold value 13.2). METHODS: A 39-year-old female nurse from Scotland, who had assisted the humanitarian effort in Sierra Leone, had received intensive supportive treatment and experimental antiviral therapies, and had been discharged with undetectable Ebola virus RNA in peripheral blood. The patient was readmitted to hospital 9 months after discharge with symptoms of acute meningitis, and was found to have Ebola virus in cerebrospinal fluid (CSF). She was treated with supportive therapy and experimental antiviral drug GS-5734 (Gilead Sciences, San Francisco, Foster City, CA, USA). We monitored Ebola virus RNA in CSF and plasma, and sequenced the viral genome using an unbiased metagenomic approach. FINDINGS: On admission, reverse transcriptase PCR identified Ebola virus RNA at a higher level in CSF (cycle threshold value 23.7) than plasma (31.3); infectious virus was only recovered from CSF. The patient developed progressive meningoencephalitis with cranial neuropathies and radiculopathy. Clinical recovery was associated with addition of high-dose corticosteroids during GS-5734 treatment. CSF Ebola virus RNA slowly declined and was undetectable following 14 days of treatment with GS-5734. Sequencing of plasma and CSF viral genome revealed only two non-coding changes compared with the original infecting virus. INTERPRETATION: Our report shows that previously unanticipated, late, severe relapses of Ebola virus can occur, in this case in the CNS. This finding fundamentally redefines what is known about the natural history of Ebola virus infection. Vigilance should be maintained in the thousands of Ebola survivors for cases of relapsed infection. The potential for these cases to initiate new transmission chains is a serious public health concern. FUNDING: Royal Free London NHS Foundation Trust.

Herpes Zoster of the Third Division of the Trigeminal Nerve. A Clinical Pathologic Conference.

Herpes zoster of the trigeminal nerve is a disease that often challenges dentists and dental specialists trying to make the proper diagnosis, as many ulcerative and vesiculobullous diseases of the mouth have a similar clinical appearance. We report a clinical case in which a 27-year-old patient sought care for this vesicular lesion. Included are the differential diagnosis and treatment modalities that we used to diagnose the disease. A clinical pathologic conference is provided to highlight the appropriate courses of action in the management of herpes zoster.

Neurological manifestations of dengue: a cross sectional study.

BACKGROUND: Dengue is an infectious disease caused by a virus of the flaviviridae family. It is a multi systemic illness causing considerable morbidity and mortality. A spectrum of neurological manifestations has been associated with dengue. METHODS: This was a descriptive cross sectional study including patients diagnosed with Dengue fever (DF), Dengue with warning signs and severe dengue with neurological sequale presenting to the Institute of Neurology, National Hospital of Sri Lanka from June 2011 to August 2012. All patients underwent serology testing for Dengue IgM in blood and CSF as confirmation of the diagnosis. RESULTS: Seven patients were included. 1/7 had bilateral optic neuritis (ON), 3/7 had a cerebellar syndrome (CS), 2/7 had transverse myelitis (TM) and 1/7 had cranial nerve palsy. The patient with ON had a post-infectious pattern and protracted recovery. All patients with CS had bilateral involvement. All had a self limiting course with complete recovery. Two were associated with acute infection. Both patients with TM had longitudinally extensive disease with one patient experiencing complete recovery. The patient with cranial nerve involvement had isolated 6th nerve palsy. CONCLUSIONS: Neurological manifestations of dengue are diverse. It is important to consider dengue as a cause for the above neurological presentations in hyper endemic territories for the disease.

Unusual oral complications of herpes zoster infection: report of a case and review of literature.

A case of herpes zoster infection with unusual oral complications involving the mandibular division of the trigeminal nerve is presented. The post-herpetic complications of osteonecrosis, spontaneous exfoliation of teeth, and subsequent pathologic fracture of mandible in the absence of concurrent predisposing factors in a 65-year-old man are demonstrated. Forty-one cases with osteonecrosis and spontaneous exfoliation of teeth previously presented in the literature are reviewed. This is the first report of pathologic fracture after herpes zoster infection.

Neuropathic orofacial pain.

Neuropathic orofacial pain is a general term employed to describe a number of clinical syndromes, which may be spontaneous or triggered by local trauma or systemic disorders. Symptomatically these painful syndromes may be episodic or continuous and are often difficult to distinguish from dental pathology. In the present article, we review the diagnosis, pathophysiology and therapeutic approaches to trigeminal and glossopharyngeal neuralgias, orofacial pain associated with herpetic infection, persistent idiopathic facial pain (previously termed atypical facial pain), post-traumatic orofacial neuropathy and neuritis.

[A case of brainstem encephalitis following multiple cranial neuropathy in a hepatocellular carcinoma patient--association with cytomegalovirus and varicella-zoster virus infection].

A 72-year-old male with liver cirrhosis and hepatocellular carcinoma experienced general fatigue. Four days later he was admitted to our hospital because of dizziness, dysbasia and left facial palsy (day 1). On day 6, a neurological examination revealed left trigeminal neuralgia, left medial longitudinal fasciculus (MLF) syndrome, skew deviation, hypacusia, tongue deviation and left limb ataxia. Magnetic resonance imaging of the brain including diffusion-weighted imaging showed previous lacunar infarctions at the left thalamus and pons. The immunological investigation for viral infection in his serum samples showed high titers of IgM antibody against cytomegalovirus (CMV). Cerebrospinal fluid (CSF) investigation revealed mononuclear pleocytosis, elevated protein levels and high titers of IgG antibody against the varicella-zoster virus (VZV). Anti-CMV antibody measurement and CMV-DNA detection by the polymerase chain reaction in CSF revealed that the central nervous system (CNS) was not infected by CMV. We diagnosed this case as brainstem encephalitis following multiple cranial neuropathy associated with CMV and VZV infections. The neurological symptoms gradually improved with aciclovir and prednisolone therapy. The titers of antibody for CMV in his serum samples normalized 4 months later after onset. Although there was no evidence of CMV infection in the CNS was obtained, parainfection or autoimmune mediated responses followed by viral infections might have led to brainstem encephalitis with multiple cranial nerve involvements in our patient.

Herpes zoster associated with tooth resorption and periapical lesions.

A 72-year-old woman presented with multiple periapical lesions and resorption of teeth in a single quadrant 17 years after an attack of herpes zoster (shingles) of the maxillary division of the trigeminal nerve. It is possible that cases of tooth resorption that were previously classified as idiopathic may have a viral aetiology and we suggest that these patients should be asked about a previous attack of shingles.

Delayed cranial neuropathy after neurosurgery caused by herpes simplex virus reactivation: report of three cases.

BACKGROUND: Delayed cranial neuropathy is an uncommon complication of neurosurgical interventions of which the exact etiology is uncertain. Several authors have hypothesized that reactivation of herpesviruses may play a role. CASE DESCRIPTIONS: The first patient underwent microvascular decompression of the left facial nerve because of hemifacial spasm. Nine days postoperatively, he developed severe facial weakness on the ipsilateral side. The polymerase chain reaction for herpes simplex virus (HSV) was positive in the cerebrospinal fluid (CSF). Treatment with intravenous acyclovir was initiated, after which a rapid and marked improvement was observed. The second patient developed left-sided facial numbness 20 days after microvascular decompression of the left facial nerve. The polymerase chain reaction for HSV was positive in the CSF. Treatment with intravenous acyclovir resulted in full recovery. The third patient underwent a suboccipital craniectomy with excision of a meningioma located at the left petrosal apex. Three months postoperatively, she developed multiple cranial neuropathies (involving cranial nerves V, VI, VIII, and XII). This was accompanied by serologic evidence of HSV reactivation and a positive polymerase chain reaction for HSV in the CSF. The patient was successfully treated with intravenous acyclovir. CONCLUSIONS: The 3 reported cases provide evidence that delayed postoperative cranial neuropathy can be caused by HSV reactivation and can involve multiple cranial nerves. An increased awareness of this treatable postoperative complication is warranted.

Isolated cranial neuropathy associated with anti-glycolipid antibodies.

We describe seven patients with isolated cranial neuropathy in whom serum anti-glycolipid antibodies were detected. Trigeminal sensory neuropathy was found in four patients, who had exhibited symptoms for 2 months to 4 years. The other three patients showed facial nerve palsy with or without ophthalmoparesis. Temporal profile analysis of anti-glycolipid antibodies revealed that titers of anti-glycolipid IgM antibodies against GM2 and LM1 gradually decreased in patients having chronic trigeminal sensory neuropathy. In patients with acute trigeminal sensory neuropathy, elevation of anti-LM1 antibody titers continued over 12 months although anti-GalNAc-GD1a antibody disappeared. On the other hand, titers of anti-glycolipid antibodies rapidly decreased in patients with acute facial nerve palsy with or without ophthalmoparesis. We conclude that anti-glycolipid antibodies may play an important role in the development of isolated cranial neuropathy in some patients.

[Unusual manifestation of zoster sine herpete as unilateral caudal cranial nerve syndrome]. / Ungewöhnliche Manifestation eines Zoster sine herpete als unilaterales kaudales Hirnnervensyndrom.

Multiple lower cranial nerve palsies are a rare complication following varicella zoster virus (VZV) reactivation, especially if typical herpetic eruptions are lacking. We report a case of a 45-year-old, immunocompetent male with unilateral involvement of the cranial nerves VIII, IX, X, and XI without skin lesions. Cerebrospinal fluid (CSF) studies revealed mononuclear pleocytosis with intrathecal antibody synthesis against VZV, while polymerase chain reaction (PCR) did not detect VZV or HSV (herpes simplex virus). The patient almost completely recovered after aciclovir administration. VZV reactivation without rash (zoster sine herpete) may lead to multiple cranial nerve palsies. PCR is a useful tool to detect VZV-DNA in CSF, but negative results do not exclude a reactivation. In case of multiple cranial nerve palsies of unknown etiology with mononuclear pleocytosis in CSF tumors of the skull base, meningitis tuberculosis, and meningeosis have to be excluded, and antiviral therapy should be discussed.

Mental nerve neuropathy as a result of primary herpes simplex virus infection in the oral cavity. A case report.

We describe a 25-year-old woman who had mental nerve neuropathy. The symptom was attributed to herpes simplex virus infection, which appeared as herpetic gingivostomatitis 4 days after the extraction of the lower third molar. This case suggests that herpes simplex virus can infect the inferior alveolar nerve through an extraction wound and can induce mental nerve neuropathy.

Enterovirus associated neurological disease in an HIV-1 infected man.

We report a case of relapsing multifocal neurological disease associated with CNS echovirus 6 infection in an HIV-1-infected individual with no evidence of immunoglobulin deficiency. The illness was initially characterized by optic and cranial neuropathies and myelopathy; concurrent granulomatous hepatitis suggested disseminated viral infection. Treatment with combination nucleoside analogues led to partial remission, but a demyelinating polyneuropathy subsequently developed. There was improvement and sustained remission in the polyneuropathy following treatment with intravenous immunoglobulin. Neurotropic enterovirus infection may be involved in the pathogenesis of certain HIV-associated neurological syndromes.