Etiology and therapy of delayed facial paralysis after middle ear surgery.

PURPOSE: This review focuses on the etiology, incidence and therapy of delayed paralysis of the facial nerve (DFP) after different types of middle ear surgery. METHODS: Retrospective review of studies published in English from 1970 until 2019 reporting DFP after tympanoplasty, tympanomastoid surgery, stapedotomy and stapedectomy. The search used the databases of PubMed, Scopus and Cochrane Library. Studies reporting from adult patients and DFP onset after 48 h after surgery were included. Studies dealing with iatrogenic or preexisting facial palsy and case reports were excluded. The initial literature search resulted in 52 studies. The relevance of the publications was verified using title, abstract and full-text analysis. Data were analyzed with descriptive statistics using median, simple sum and statistical significance. RESULTS: Ten studies having 12,161 patients could be included in this review. The incidence of DFP after the middle ear surgeries varies between 0.2 and 1.9%. The surgical stress of the middle ear surgeries is the main trigger for the development of DFP and leads to a virus reactivation and/or neuronal edema. Patients with a dehiscence of the facial canal have a significantly higher probability for a DFP. The recommended therapy of DFP based on the data of the therapy of Bell's palsy, consists of the administration of a steroid. For patients having a case history of previous viral infections, an antiviral prophylaxis is recommended. CONCLUSION: Overall, DFP has a very good prognosis, with mostly complete healing with appropriate therapy. Viral reactivation is the most favored genesis of DFP. Immunization or antiviral prophylaxis is recommended to those patients being at risk for a viral reactivation.

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.

rhesus cytomegalovirus (macacine herpesvirus 3)-associated facial neuritis in simian immunodeficiency virus-infected rhesus macaques (Macaca mulatta).

Peripheral neuropathies are common sequelae to human immunodeficiency virus (HIV) infection in humans and are due to a variety of mechanisms, including direct antiretroviral toxicity, HIV-mediated damage, immune-mediated disorders, and opportunistic viral infections. Rhesus macaques (Macaca mulatta) infected with simian immunodeficiency virus (SIV) remain the most consistent animal model for unraveling the pathogenesis of lentiviral-associated disease and its associated opportunistic infections. Rhesus cytomegalovirus (RhCMV) is the most common opportunistic viral infection in rhesus macaques infected with SIV and causes multiorgan pathology; however, its role in peripheral nerve pathology has not been explored. We have identified 115 coinfected cases with SIV and RhCMV, of which 10 cases of RhCMV-associated facial neuritis were found (8.7% prevalence). Histologic lesions were consistent in all cases and ranged from partial to complete obliteration of the nerves of the tongue, lacrimal gland, and other facial tissues with a mixed inflammatory population of neutrophils and macrophages, of which the latter commonly contained intranuclear inclusion bodies. Luxol fast blue staining and myelin basic protein immunohistochemistry confirmed the progressive myelin loss in the peripheral nerves. Bielschowsky silver stain revealed progressive loss of axons directly related to the severity of inflammation. Double immunohistochemistry with spectral imaging analysis revealed RhCMV-infected macrophages directly associated with the neuritis, and there was no evidence to support RhCMV infection of Schwann cells. These results suggest that peripheral nerve damage is a bystander effect secondary to inflammation rather than a direct infection of Schwann cells and warrants further investigations into the pathogenesis of RhCMV-induced peripheral neuropathy.

Successful response of non-recovering Ramsay Hunt syndrome to intravenous high dose methylprednisolone.

Ramsay Hunt syndrome (RHS) is a frequent cause of facial palsy. It is a consequence of the infection of geniculate ganglion by herpes zoster or herpes simplex virus. In the lack of randomized controlled trials, RHS is empirically treated by a combination therapy of antiviral agents and steroids given orally. However, RHS has, per se, a poorer prognosis than idiopathic facial palsy (Bell's palsy). We describe a case series of two patients with RHS unsuccessfully treated with antiviral drugs and oral corticosteroids, showing an almost complete recovery after late administration of intravenous (i.v.) high dose methylprednisolone. Both patients had all recognized negative prognostic factors including age of onset, a high grade facial weakness, absence of R1 and R2 response at blink reflex test, and in the first case, the involvement of greater superficial petrosal nerve. We propose that i.v. high dose methylprednisolone should be considered, even as a late treatment option, in patients with RHS non recovering after standard antiviral and oral steroid therapy as well as presenting clinical features suggestive of a poor prognosis.

Ramsay Hunt syndrome with unilateral polyneuropathy involving cranial nerves V, VII, VIII, and XII in a diabetic patient.

Ramsay Hunt syndrome is a rare complication of the varicella zoster virus, defined as a peripheral facial palsy that typically results from involvement of the facial and auditory nerves. Ramsay Hunt syndrome can be associated with cranial nerves V, VI, IX, and X but rarely with XII. We describe an atypical case of Ramsay Hunt syndrome with multiple cranial nerve involvement of nerves V, VII, VIII, and XII. Antiviral drugs, antibiotics, insulin, and traditional Chinese drugs were administered immediately after admission. After 3 months of combination therapy, the patient had recovered satisfactorily. Herpes zoster can cause severe infections in diabetic patients and should be treated as soon after detection as possible. Ramsay Hunt syndrome should be recognized as a polycranial neuritis characterized by damage to sensory and motor nerves. In addition to facial and vestibular nerve paralysis, Ramsay Hunt syndrome may also involve cranial nerves V and XII.

Facial nerve palsy: etiology and approach to diagnosis and treatment.

Facial nerve palsy has a broad differential diagnosis and possible psychological and anatomical consequences. A thorough investigation must be performed to determine the cause of the palsy and to direct treatment. If no cause can be found, therapy with prednisone with or without an antiviral medication can be considered and begun as early as possible after onset of symptoms. Resolution and time to recovery vary with etiology, but overall prognosis is good.

Reactivation and centripetal spread of herpes simplex virus complicating acoustic neuroma resection.

BACKGROUND: Herpes simplex is a common human pathogen that has rare but severe manifestations including encephalitis. CASE DESCRIPTION: A 44-year-old man underwent uneventful resection of an acoustic neuroma. Postoperatively, he developed swinging pyrexia, vomiting, and episodic confusion. Analysis of cerebrospinal fluid showed a lymphocytosis, and polymerase chain reaction revealed herpes simplex DNA. After treatment of herpes encephalitis with acyclovir, the patient made a good recovery. CONCLUSION: Herpes encephalitis is a rare complication of neurosurgical procedures, and the most likely etiology is reactivation of latent infection from manipulation of cranial nerves.

Central nervous system involvement after herpes zoster ophthalmicus.

PURPOSE: To report central nervous system involvement after varicella zoster virus infection. METHODS: We evaluated the frequency and type of neurological complications in patients initially presenting with ophthalmic herpes zoster at an ophthalmological department in a Danish university hospital, over a 7-year period. RESULTS: Of the 110 immunocompetent patients who presented with initial ophthalmic zoster, six (5.5%) suffered from neurological complications other than post-herpetic neuralgia. Four experienced isolated cranial motor nerve palsies, one patient had meningitis with a favourable outcome and one patient had severe encephalitis with a poor clinical outcome. CONCLUSIONS: Central nervous system involvement after varicella zoster virus infection is an uncommon, but potentially life-threatening, complication. Early recognition of neurological complications prompts acute, appropriate antiviral treatment.

Severe complications of herpes zoster.

The usual presentation of herpes zoster is as a self-limiting vesicular rash, often accompanied by post-herpetic neuralgia (PHN), its most common complication. However, herpes zoster can give rise to other complications, many of which have unusual presentations and serious sequelae. The incidence and burden of many of these less common complications are poorly understood. Ocular complications of ophthalmic zoster are relatively frequent but, with early antiviral therapy, need not be sight-threatening. Delayed contralateral hemiparesis is a rare complication of ophthalmic zoster that may present as stroke, temporally remote from the zoster episode. Ramsay Hunt syndrome is caused by reactivation of varicella zoster virus (VZV) involving the facial nerve; facial paralysis, ear pain and vesicles in the ear are diagnostic. Facial paralysis in the absence of vesicles may indicate zoster sine herpete, which can be mistaken for Bell's palsy. Herpetic facial palsies may respond to combination therapy with an antiviral plus steroid, but further research is needed to determine the benefit of such treatments.

Isolated double herpes zoster paresis involving the left facial nerve and the right peroneal nerve following disseminated herpes zoster.

A 72-year-old Japanese male developed disseminated herpes zoster and could not easily walk due to right drop foot and pain. He soon developed numbness and pain on the left side of his face, and noticed difficulty closing his left eye. The left angle of his mouth dropped. The patient was diagnosed as having a double mononeuropathy (a left facial nerve paresis and a right peroneal nerve paresis) following disseminated herpes zoster. Given that the patient was elderly and had diabetes mellitus, the patient appeared to be an immunocompromised host. We also describe other rare complications of herpes zoster from the published work.

Frequency and prognosis of delayed facial palsy after microvascular decompression for hemifacial spasm.

BACKGROUND: Microvascular decompression (MVD) for hemifacial spasm (HFS) provides a long-term cure rate. Delayed facial palsy (DFP) is not an unusual complication, but it has only been sporadically described in the literature. The purpose of this report is to evaluate the incidence of delayed facial palsy after MVD and its clinical course and final results. METHODS: From January, 1998 to April, 2004, 410 patients underwent microvascular decompression for hemifacial spasm at our Institute. During this time, 21 patients (5.4%) developed delayed facial weakness; eighteen of them were given steroid medication and they were followed up in the out-patient clinic. FINDINGS: Twenty-one patients developed DFP after microvascular decompression an incidence of 5.4%. There were seventeen women (81.0%) among the 21 patients with DFP who were included in this study. In twenty of them, the symptoms of HFS improved completely after the operation, but the spasm remained with one of them. The onset of palsy occurred between postoperative day 7 and 23 (average: 12.1 days). The palsy was at least Grade II or worse on the House-Brackmann (HB) scale. The time to recovery averaged 5.7 weeks (range: 25 days-17 weeks); 20 patients improved to complete recovery and 1 patient remained with minimal weakness, as Grade II on the HB scale, at the follow-up examination. CONCLUSION: Our findings demonstrated that the incidence of DFP was not so low as has been reported the literature, and it did not have any striking predisposing factors. Even though the degree of facial palsy was variable, almost all patients exhibited a complete recovery without any further special treatment. The etiology of DFP and its association with herpes infection should be further clarified.

[Animal model of facial neuritis induced by herpes simplex virus].

OBJECTIVE: To study the role of herpes simplex virus type 1 ( HSV-1 ) in facial paralysis by developing an experimental animal model of viral facial paralysis. METHODS: Both sides of posterior auricular branch of facial nerve were anatomies and incised in 66 mice. The HSV-1 was inoculated into right ear branch and fetal bovine serum was inoculated into left ear branch as control. The symmetry of mouse face was observed and scored. The temporal bones were serially sectioned and stained with hematoxylin and eosin. The extratemporal facial nerves were stained with osmium tetroxide. HSV-1 DNA in bilateral facial nerve, brain stem, trigeminal ganglion and spinal cord was detected by the polymerase chain reaction. RESULTS: Twenty-eight (42. 42%) mice developed right facial paralysis between 2 and 5 days after inoculation. Continuing 3-6 days, the facial paralysis recovered spontaneously. Thirty-eight mice had no signs of facial paralysis. Compared with the left, nerve swelling, inflammatory cell infiltration were manifested in right temporal facial nerve of paralyzed mice. The ratio of the cross-sectional area of the facial nerve to the facial canal ( FN/FC ) was significantly higher than that on the control side (P < 0.01). Demyelinated nerve fibers were seen in the right extratemporal facial nerve. Not only in paralyzed mice, but also in non-paralyzed mice, HSV DNA was detected in some nerve tissues. CONCLUSIONS: Inoculating HSV-1 into posterior auricular branch of facial nerve can produce an acute and transient facial paralysis in mice. The possible pathophysiologic mechanism of the facial paralysis is viral invasion and transportation from distal branch to main trunk. Then the viral facial neuritis causes facial paralysis.

The pharyngeal-cervical-brachial form of Guillain-Barré syndrome in childhood.

Variant forms of the Guillain-Barré syndrome are characterized by their localized or regional involvement of the peripheral and autonomic nerves. As there is no single clinical or serologic marker for Guillain-Barré syndrome, diagnosis of this condition is based upon consistent clinical, laboratory, and neurophysiologic findings, with exclusion of other conditions mimicking this disorder. Recognition of atypical cases enables anticipatory monitoring for disease complications and identifies therapeutic options for affected children. A regional variant with predominant facial, neck, and arm weakness without sensory loss has been reported in adults but only rarely described in childhood. This study reports clinical and neurophysiologic findings in two children with the pharyngeal-cervical-brachial form of Guillain-Barré syndrome. These are the youngest cases of this uncommon disorder reported to date.

Ramsay Hunt syndrome presenting as a cranial polyneuropathy.

Ramsay Hunt syndrome is herpes zoster of the facial nerve, frequently associated with VIII cranial nerve involvement, but on rare occasions V, VI, IX, and X cranial nerves are affected as well. We present a case of a Ramsay Hunt syndrome with involvement of V, VII, and VIII cranial nerves.