Cranial Polyneuropathy Research Articles

Ramsay Hunt Syndrome: An Unusual Variant After Dental Infection

Ramsay Hunt Syndrome: An Unusual Variant After Dental Infection

Intravenous immunoglobulin for cranial polyneuropathy associated with Campylobacter jejuni infection

This study reports the efficacy of IV immunoglobulin in a patient with cranial polyneuropathy resulting from Campylobacter jejuni infection who had high titers of serum IgG antibodies against gangliosides GD1a and GT1b in the acute phase. Treatment with IV immunoglobulin (400 mg/kg/day × 5 days) led to rapid partial resolution of his neurologic manifestations, but complete recovery was not obtained until 6 months later. The present case suggests that IV immunoglobulin therapy prevents further progression of the disease but that it may not shorten the clinical course of cranial polyneuropathy in some cases associated with Campylobacter jejuni infection.

Hereditary and acquired amyloid neuropathies.

Amyloid neuropathies occur in a context of hereditary (FAP) or acquired amyloidosis. They present usually as severe and progressive polyneuropathy and carry a poor prognosis. Most FAP are associated with endoneurial deposits of variant transthyretin (TTR) with substitution of one aminoacid and are secondary to a point mutation of the TTR gene. Portugal is the main endemic area of TTR-FAP, secondary to point mutation of exon 2. However, around the world, 50 other TTR gene mutations have been recently reported, each one in few families. Genetic studies are useful for diagnosis of FAP in patients with a positive family history and for identification of the cause of seemingly sporadic cases. TTR gene analysis is also useful for genetic counselling including antenatal diagnosis in variants with early onset. Gel-solin-FAP are the second variety and present as a benign cranial and sensory polyneuropathy and affect essentially Finnish patients. Acquired amyloid neuropathy concerns only immunoglobulin light chain amyloidosis (AL) and are frequently associated with renal manifestations and monoclonal protein in serum or urine. Specific treatment of amyloid polyneuropathy varies with the variety of amyloidosis including liver transplantation in TTR-FAP, at the onset of the disease or chemotherapy for immunoglobulin light chain amyloidosis.

Three patients with ophthalmoplegia associated with Campylobacter jejuni

Cranial polyneuropathy is idiopathic in most patients. Idiopathic cranial polyneuropathy is an acute postinfectious syndrome, along with Guillain-Barré syndrome and Miller Fisher syndrome, in which the common preceding pathogen is Campylobacter jejuni. Serum anti-GQ1b antibodies are elevated in Miller Fisher syndrome and Guillain-Barré syndrome with ophthalmoplegia. Three patients with idiopathic cranial polyneuropathy with predominant ocular involvement are presented. C. jejuni isolated from stool specimens belonged to Penner serotypes O:4, O:23, and O:33. Serum anti-GQ1b antibodies were elevated in all patients but demonstrated rapid reduction concomitant with clinical recovery. All patients recovered completely. Because both preceding C. jejuni infection and elevated anti-GQ1b antibodies decreasing with time were seen in all patients, the pathogenesis of idiopathic cranial polyneuropathy with ophthalmoplegia may be similar to that of Miller Fisher syndrome.

Herpes zoster oticus treated with acyclovir and prednisolone: clinical manifestations and analysis of prognostic factors.

Herpes zoster oticus is a cranial polyneuropathy with facial nerve involvement as its main feature. The prognosis of the facial palsy is usually poor. Thirty patients with herpes zoster oticus suffering from facial palsy were admitted for parenteral acyclovir and oral prednisolone. Multiple regression analysis of improvement of facial palsy showed three significant covariates: age, multiple nerve palsies, and the initial grading of the palsy. The recovery of the facial palsy treated with acyclovir and prednisolone was good, and possibility of a good outcome was greater when the initial grade of the palsy was higher. Multiple nerve palsies and age had negative effects on the improvement.

Jugular foramen syndrome caused by varicella zoster virus infection in a patient with ipsilateral hypoplasia of the jugular foramen

We report a patient with acute cranial polyneuropathy with unilateral involvement of the ninth, tenth, and eleventh cranial nerves. Although this patient lacked a typical cutaneous herpetic manifestation, elevated levels of IgM and IgG antibodies to varicella zoster virus (VZV) in both the serum and cerebrospinal fluid confirmed the clinical diagnosis of VZV infection and zoster sine herpete. Coexisting hypoplasia of the ipsilateral jugular foramen was detected using three-dimensional, surface-rendering displays reconstructed from the cranial helical CT scan. The patient recovered almost completely following treatment with an anti-inflammatory corticosteroid. Anatomical narrowing of the jugular foramen in this patient may have contributed to entrapment of the affected nerves at their passage through the foramen.

Cranial Polyneuropathies in Multiple Sclerosis: Case Report and Literature Review

Although cranial neuropathies are common disorders in multiple sclerosis patients, multiple cranial nerve involvement is an unusual occurrence. Correlation of clinical symptoms with magnetic resonance imaging evidence of demyelinating central nervous system lesions can confirm the diagnosis. The authors report on the case of a 43-year-old woman who initially was thought to have suffered a brainstem infarct but, in fact, had developed multiple cranial nerve functional deficits. Treatment of multiple sclerosis remains primarily supportive in nature, with corticosteroids used for acute exacerbations and chronic progression.

Facial canal decompression leads to recovery of combined facial nerve paresis and trigeminal sensory neuropathy: Case report

BACKGROUND Trigeminal sensory neuropathy is often associated with facial idiopathic nerve paralysis (Bell’s palsy). Although a cranial nerve viral polyneuropathy has been proposed as the usual cause, in many instances the etiology remains unclear. This case report of recovery of both trigeminal and facial neuropathy after surgical decompression of the facial nerve suggests an anatomic link. METHODS A case of a 39-year-old woman presenting with recurrent unilateral facial paralysis is summarized. Her fifth episode, which did not spontaneously recover, was associated with retroorbital and maxillary pain as well as sensory loss in the trigeminal distribution. RESULTS A middle cranial fossa approach for decompression of the lateral internal auditory canal, labyrinthine segment of the facial nerve and the geniculate ganglion was performed. The patient’s pain and numbness resolved immediately postoperatively, and the facial paralysis improved markedly. CONCLUSION This result implicates a trigeminal-facial reflex as hypothesized by others. It suggests that decompression of the facial nerve can lead to improvement in motor and sensory function as well as relief of pain in some patients with combined trigeminal and facial nerve dysfunction.

Ramsay-Hunt 증후군 환자의 다발성 뇌신경병변

Background and Objectives: Ramsay-Hunt syndrome is characterized by otalgia, vesicular eruption on the pinna, retroauricular area or face and facial palsy. It is caused by Herpes zoster infection of the facial nerve in patients of low immune state. Sometimes other cranial nerves can be involved in this syndrome but there is not much information about these cases because of its rare incidence. The purpose of this report is to study the characteristics of cranial polyneuropathy in Ramsay Hunt syndrome and the clinical differences between the groups of Ramsay-Hunt syndrome with and without polyneuropathy. Materials and Methods: There were 215 cases of facial nerve palsy for 14 years in Yongdong Severance Hospital including 37 cases of Ramsay-Hunt syndrome which were reviewed retrospectively among them. The duration of their clinical follow up was more than 6 months. Results: Cochleovestibular nerve was involved most frequently in Ramsay Hunt syndrome and it was also noted that the symtoms and signs of trigeminal, abducens, glossopharyngeal, vagus and accessory nerves. There was female predominance and higher mean age in the cranial polyneuropathy group. It had worse recovery rate than typical Ramsay-Hunt syndrome but there was no statistical significance. Conclusion: It is suggested that cranial polyneuropathy in Ramsay-Hunt syndrome has different clinical findings from typical Ramsay Hunt syndrome and it is required that evaluation of more cases, study of the pathogenesis, and setting of the diagnostic criteria.

両側同時性顔面神経麻ひ例

We encountered a patient with bilateral simultaneous facial nerve palsy who was suspected to have idiopathic cranial polyneuropathy, but no change in the cerebrospinal fluid or evidence of infectious disease was detected. The patient displayed symptoms of palsy, but they were related only to the facial nerve. Consequently, he was diagnosed with Bell's palsy.There are many different potential diagnoses in cases of facial palsy. This report discusses various forms of Guillain-Barré syndrome and Lyme disease which tends to be overlooked as a cause of facial nerve palsy.

A novel monoclonal antibody which reacts with a high molecular weight neuronal cytoplasmic protein and myelin basic protein (MBP) in a patient with macroglobulinemia

We report on the case of a 70-year-old man with primary macroglobulinemia who showed cranial polyneuropathy and extensive radiculoneuropathy. His serum contained an IgM λ monoclonal antibody which reacted with both a high molecular weight protein in grey matter and purified myelin basic protein (MBP) on immunoblotting. In an immunohistochemical study, strong immunoreactivity was detected only in the cytoplasm of neurons and weak immunoreactivity was detected in myelin. These findings suggest that this antibody may be related to the pathogenesis of neuronal damage in patients with macroglobulinemia.

Cranial polyneuropathy—ramsay hunt's syndrome: Case report and discussion

Ramsay Hunt's syndrome is an infectious cranial polyneuropathy caused by varicella zoster, the herpetic virus that also causes chickenpox and shingles. Its symptoms include facial paralysis, ear pain, and an auricular rash. Oral lesions are also present in most cases. This syndrome can affect any cranial nerve and usually affects multiple nerves, causing central, cervical, and peripheral effects. This article reports the case of a 35-year-old white female who was treated by the oral surgery service of a large urban hospital, after first reporting to the emergency clinic. Her reported symptoms of unilateral left-side facial paralysis, auricular pain, and trigeminal hyperesthesia were confirmed by clinical examination. An initial short low-dose steroid regimen was unsuccessful. A second daily dosage of 50 mg of prednisone was successful in 21 days. No permanent sequelae were evident or reported after treatment.

Cranial polyneuropathy with elevated serum antiganglioside antibody

We report a 6-year-old girl with cranial polyneuropathy with elevated serum levels of antiganglioside antibodies. She manifested herpetic vesicles around the right upper eyelid and mouth without antecedent infection. She developed facial asymmetry and double vision 5 days after the first apperance of the vesicles. Neurological examination on admission disclosed palsies of the bilateral sixth and twelfth cranial nerves and right third and seventh cranial nerves, but limb muscle weakness, ataxia, and areflexia were not observed. Cerebrospinal fluid examination and MRI of the brain showed no abnormalities. Serum antibodies to gangliosides GQ1b and GT1b, but not GM1, and those to Campylobacter jejuni were significantly increased on admission and on the hospital day 14. These observations suggest that the present case is a variant form of Miller Fisher syndrome or Bickerstaff's brainstem encephalitis subsequent to asymptomatic C. jejuni infection. We treated her with intravenous administration of high-dose methylprednisolone and acyclovir, but almost no effect was observed. All cranial nerve palsies, however, had resolved completely approximately 4 months later. This may be the first pediatric case in which cranial polyneuropathy and antiganglioside antibodies were associated.

Clinical significance of diffuse dural enhancement detected by magnetic resonance imaging.

This study was performed to determine the clinical significance of diffuse dural enhancement (DDE) detected by magnetic resonance (MR) imaging and to typify enhancing patterns related to inflammatory or metastatic causes. The authors retrospectively evaluated the clinical, imaging, and laboratory characteristics of 20 consecutive patients with DDE. Those with DDE and an underlying neoplastic disease (13 patients) were compared to 11 consecutive patients with cytological evidence of neoplastic leptomeningeal metastasis evaluated by MR imaging. The DDE was often associated with an underlying malignancy (13 (65%) of 20 patients) but it coexisted with leptomeningeal metastasis in only one patient. Skull metastases were evident in 10 (77%) of 13 patients and cranial nerve palsies in six (46%) of 13. Other causes of DDE were related to cerebrospinal fluid (CSF) leak or shunting (five (25%) of 20), with or without symptoms of intracranial hypotension, and to dural sinus thrombosis and pachymeningitis. Dural biopsies obtained in two patients with DDE showed a narrow rim of granulation-like tissue adherent to the dural surface facing the inner skull table. Magnetic resonance subtraction, diffusion, and perfusion studies revealed unique characteristics in patients with metastatic causes as compared to those with DDE secondary to CSF leak. None of the patients with proven leptomeningeal metastasis had DDE, but four of them presented with focal dural enhancement and two displayed apparent leptomeningeal enhancement. The findings indicate that DDE is not a radiographic hallmark of leptomeningeal metastasis in spite of the similarities in clinical manifestations (for example, headache and cranial polyneuropathy). Nonetheless, DDE is most frequently associated with metastatic malignancies and particularly with skull metastases and CSF leak. Special MR techniques can discern the underlying cause and elucidate the disparity in the pathophysiological mechanisms leading to DDE.

多彩な全身症状を呈した自己免疫性感音難聴

We report a case of ASNHL (autoimmune sensorineural hearing loss) with systemic symptoms. The patient was a 64-year old female who consulted us with complaints of bilateral hearing loss and left tinnitus. Later, she had cranial polyneuropathy (L: V VII VIII, R: VIII XII), diabetes insipidus, headache and fever. Her blood sedimentation rate, CRP and γ-globulin were increased. Antinuclear antibodies and antihypophysis antibodies were found in her serum. Her general condition and hearing level were improved by steroid and cyclophosphamide therapy. Autoimmune vasculitis was suspected, but histopathological studies of granulation tissue around the left stapes and left superficial temporal artery were not specific. Steroid therapy is being continued.

Varicella Zoster--Associated Neurologic Disease Without Skin Lesions

We have investigated a group of cases with inflammatory neurologic disease and serologic evidence of varicella zoster virus activity without cutaneous vesicles. In agreement with experience, a case of cranial polyneuropathy with facial palsy and cases of aseptic meningitis were found. However, cases of acute polyneuropathy, myelitis, and encephalitis were also identified. Thus, it seems that the spectrum of neurologic disease associated with varicella zoster virus in the absence of skin lesions is considerably broader than previously recognized.

Candida meningitis: Clinical and radiographic diagnosis

Two patients with Candida meningitis are presented to emphasize cranial polyneuropathy and ventricular isolation with septation. These clinical and radiographic criteria can be used for early diagnosis and treatment.

Attenuated cranial polyneuropathy

International Journal of Clinical PracticeVolume 40, Issue 10 p. 441-442 Case Report Attenuated cranial polyneuropathy J Singh MD, J Singh MD All- India Institute of Medical Sciences, New Delhi, IndiaSearch for more papers by this authorV Menon MS, V Menon MS All- India Institute of Medical Sciences, New Delhi, IndiaSearch for more papers by this author J Singh MD, J Singh MD All- India Institute of Medical Sciences, New Delhi, IndiaSearch for more papers by this authorV Menon MS, V Menon MS All- India Institute of Medical Sciences, New Delhi, IndiaSearch for more papers by this author First published: 01 October 1986 https://doi.org/10.1111/j.1742-1241.1986.tb08027.xRead the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article. Volume40, Issue10October 1986Pages 441-442 RelatedInformation

Sprouting inducing activity of human sera: low capacity in sera from patients with cranial polyneuropathy.

Cranial idiopathic acute peripheral facial nerve paralysis (Bell palsy) has been used as a model disease in the present study mainly to investigate trophic capacity of serum components. The ability to induce sprouting from chick embryo dorsal root ganglia was used as the bioassay for trophic activity. Sera from normal patients induce neuritic outgrowth from dorsal root ganglia of chick embryo in the absence of any exogenous factor. Sprouting, to a very limited extent or none, has been induced by 80% of the sera derived from the Bell palsy patients. Further analysis of the biochemical nature of the reduced activity has revealed that in sera of Bell palsy patients the substances that are fractionated within the Ig fraction are responsible for the reduced capacity to induce sprouting without loss of supportive molecules.

Ramsay hunt syndrome: A cranial polyneuropathy

Two patients with Ramsay Hunt syndrome with unusual manifestations are described. The first patient had a cranial polyneuropathy with herpes zoster uveitis and anhidrosis of the face, and the second patient had erysipelas of the nose and auricle followed by facial nerve paresis and mild cochleo-vestibular symptoms. On the basis of the histologic, viral and immunologic findings, it is suggested that this syndrome and Bell's palsy are different clinical manifestations of the same disease. Thus, Hunt's classification and the idiopathy of bell's palsy are probably no longer tenable.