A retrospective review and multi-specialty, evidence-based guideline for the management of necrotising otitis externa.

BACKGROUND: Necrotising otitis externa is a progressive infection of the external auditory canal which extends to affect the temporal bone and adjacent structures. Progression of the disease process can result in serious sequelae, including cranial nerve palsies and death. There is currently no formal published treatment guideline. OBJECTIVE: This study aimed to integrate current evidence and data from our own retrospective case series in order to develop a guideline to optimise necrotising otitis externa patient management. METHODS: A retrospective review of necrotising otitis externa cases within NHS Lothian, Scotland, between 2013 and 2018, was performed, along with a PubMed review. RESULTS: Prevalent presenting signs, symptoms and patient demographic data were established. Furthermore, features of cases associated with adverse outcomes were defined. A key feature of the guideline is defining at-risk patients with initial intensive treatment. Investigations and outcomes are assessed and treatment adjusted appropriately. CONCLUSION: This multi-departmental approach has facilitated the development of a succinct, systematic guideline for the management of necrotising otitis externa. Initial patient outcomes appear promising.

Cranial nerve outcomes after primary stereotactic radiosurgery for symptomatic skull base meningiomas.

OBJECTIVE: To evaluate cranial nerve (CN) outcomes after primary stereotactic radiosurgery (SRS) for petroclival, cavernous sinus, and cerebellopontine angle meningiomas. METHODS: From our prospectively maintained database of 2022 meningioma patients who underwent Leksell stereotactic radiosurgery (SRS) during a 30-year interval, we found 98 patients with petroclival, 242 with cavernous sinus, and 55 patients with cerebellopontine angle meningiomas. Primary radiosurgery was performed in 245 patients. Patients included in this report had at least one CN deficit at the time of initial presentation and a minimum of 12 month follow up. Median age at the time of SRS was 58 years. Median follow up was 58 months (range 12-300 months), Median tumor volume treated with SRS was 5.9 cm3 (range 0.5-37.5 cm3), and median margin dose was 13 Gy (range 9-20Gy). RESULTS: Tumor control was achieved in 229 patients (93.5%) at a median follow up of 58 months. Progression free survival rate (PFS) after SRS was 98.7% at 1 year, 96.4% at 3 years, 93.7% at 5 years, and 86.4% at 10 years Overall, 114 of the 245 patients (46.5%) reported improvement of CN function. Patients with CP angle meningiomas demonstrated lower rates of CN improvement compared to petroclival and cavernous sinus meningioma patients. Deterioration of CN function after SRS developed in 24 patients (10%). The rate of deterioration was 2.8% at 1 year, 5.2% at 3 years, and 8% at 10 years. CONCLUSION: Primary SRS provides effective tumor control and favorable rate of improvement of preexisting CN deficit.

Five-year survival and median survival time of nasopharyngeal carcinoma in Hospital Universiti Sains Malaysia.

BACKGROUND: Nasopharyngeal carcinoma (NPC) is the fourth most common cancer in Malaysia. The objective of this study was to determine the five-year survival rate and median survival time of NPC patients in Hospital Universiti Sains Malaysia (USM). METHODS: One hundred and thirty four NPC cases confirmed by histopathology in Hospital USM between 1st January 1998 and 31st December 2007 that fulfilled the inclusion and exclusion criteria were retrospectively reviewed. Survival time of NPC patients were estimated by Kaplan-Meier survival analysis. Log-rank tests were performed to compare survival of cases among presenting symptoms, WHO type, TNM classification and treatment modalities. RESULTS: The overall five-year survival rate of NPC patients was 38.0% (95% confidence interval (CI): 29.1, 46.9). The overall median survival time of NPC patients was 31.30 months (95%CI: 23.76, 38.84). The significant factors that altered the survival rate and time were age (p=0.041), cranial nerve involvement (p=0.012), stage (p=0.002), metastases (p=0.008) and treatment (p<0.001). CONCLUSION: The median survival of NPC patients is significantly longer for age≤50 years, no cranial nerve involvement, and early stage and is dependent on treatment modalities.

Gamma Knife surgery for the management of glomus tumors: a multicenter study.

OBJECT: Glomus tumors are rare skull base neoplasms that frequently involve critical cerebrovascular structures and lower cranial nerves. Complete resection is often difficult and may increase cranial nerve deficits. Stereotactic radiosurgery has gained an increasing role in the management of glomus tumors. The authors of this study examine the outcomes after radiosurgery in a large, multicenter patient population. METHODS: Under the auspices of the North American Gamma Knife Consortium, 8 Gamma Knife surgery centers that treat glomus tumors combined their outcome data retrospectively. One hundred thirty-four patient procedures were included in the study (134 procedures in 132 patients, with each procedure being analyzed separately). Prior resection was performed in 51 patients, and prior fractionated external beam radiotherapy was performed in 6 patients. The patients' median age at the time of radiosurgery was 59 years. Forty percent had pulsatile tinnitus at the time of radiosurgery. The median dose to the tumor margin was 15 Gy. The median duration of follow-up was 50.5 months (range 5-220 months). RESULTS: Overall tumor control was achieved in 93% of patients at last follow-up; actuarial tumor control was 88% at 5 years postradiosurgery. Absence of trigeminal nerve dysfunction at the time of radiosurgery (p = 0.001) and higher number of isocenters (p = 0.005) were statistically associated with tumor progression-free tumor survival. Patients demonstrating new or progressive cranial nerve deficits were also likely to demonstrate tumor progression (p = 0.002). Pulsatile tinnitus improved in 49% of patients who reported it at presentation. New or progressive cranial nerve deficits were noted in 15% of patients; improvement in preexisting cranial nerve deficits was observed in 11% of patients. No patient died as a result of tumor progression. CONCLUSIONS: Gamma Knife surgery was a well-tolerated management strategy that provided a high rate of long-term glomus tumor control. Symptomatic tinnitus improved in almost one-half of the patients. Overall neurological status and cranial nerve function were preserved or improved in the vast majority of patients after radiosurgery.

Incidence, predictors and prognostic value of cranial nerve involvement in patients with tuberculous meningitis: a retrospective evaluation.

BACKGROUND: Cranial nerve involvement is commonly observed in patients with tuberculous meningitis. The present study evaluated the incidence, predictors and prognostic significance of cranial nerve involvement in tuberculous meningitis. MATERIALS AND METHOD: One hundred-fifty-eight adult patients with tuberculous meningitis were retrospectively evaluated and followed up for 9 months. A detailed clinical evaluation and cranial magnetic resonance imaging were done in every patient. RESULT: At inclusion, 60 (38%) patients had cranial neuropathy. Sixteen patients were having involvement of two or more cranial nerves. Abducent nerve was the most frequently (32.3%) affected cranial nerve. Predictors of cranial nerve involvement were age >25 years, history of vomiting, altered sensorium, hemiparesis, diplopia, papilledema, signs of meningeal irritation, severe functional disability, cerebrospinal fluid protein >2.5 g/L and cerebrospinal fluid cell count >100/mm(3). The presence of optochiasmatic arachnoiditis and hydrocephalus was also a significant predictor of cranial neuropathy. Presence of cranial neuropathy was significantly associated with poor outcome. CONCLUSION: Cranial nerve involvement occurred in more than one third of patients with tuberculous meningitis. The presence of cranial neuropathy was associated with poor outcome.

Outcomes of malignant external otitis: survival vs mortality.

CONCLUSIONS: In patients with malignant external otitis (MEO), mortality was not related to age, sex, degree of glucose tolerance, duration of diabetes mellitus (DM), microorganism, comorbid disease, or involvement of a single cranial nerve. Severe complications such as skull base osteomyelitis, intracranial extension, and involvement of multiple cranial nerves were correlated with mortality. OBJECTIVES: To analyze the factors that affect the survival of patients with MEO in today's era of advanced antibiotics. PATIENTS AND METHODS: Patients with a diagnosis of MEO from 1993 to 2005 were collected. RESULTS: A total 26 patients with a mean age of 63.7+/-10.2 years were included. All had a history of DM. Pseudomonas aeruginosa (26.9%), Klebsiella pneumoniae (19.2%), and fungus (15.4%) were most frequently found in pus cultures. Cranial nerves were involved in 11 patients: the facial nerve was most frequently (38.46%) involved. Complications such as osteomyelitis (11 patients) and intracranial involvement (3 patients) were noted in this series. Mastoidectomy was performed in 12 patients. A total of five patients died.

Association between minor and major surgical complications after carotid endarterectomy: results of the New York Carotid Artery Surgery study.

OBJECTIVE: Most studies on outcomes of carotid endarterectomy (CEA) have focused on the major complications of death and stroke. Less is known about minor but more common surgical complications such as hematoma, cranial nerve palsy, and wound infection. This study used data from a large, population-based cohort study to describe the incidence of minor surgical complications after CEA and examine associations between minor and major complications. METHODS: The New York Carotid Artery Surgery (NYCAS) study examined all Medicare beneficiaries who underwent CEA from January 1998 to June 1999 in NY State. Detailed clinical information on preoperative characteristics and complications < or =30 days of surgery was abstracted from hospital charts. Associations between minor (cranial nerve palsies, hematoma, and wound infection) and major complications (death/stroke) were examined with chi(2) tests and multivariate logistic regression. RESULTS: The NYCAS study had data on 9308 CEAs performed by 482 surgeons in 167 hospitals. Overall, 10% of patients had a minor surgical complication (cranial nerve (CN) palsy, 5.5%; hematoma, 5.0%; and wound infection, 0.2%). Cardiac complications occurred in 3.9% (myocardial 1.1%, unstable angina 0.9%, pulmonary edema 2.1%, and ventricular tachycardia 0.8%). In both unadjusted and adjusted analyses, the occurrence of any minor surgical complication, CN palsy alone, or hematoma alone was associated with 3 to 4-fold greater odds of perioperative stroke or combined risk of death and nonfatal stroke (P < 0.0001). Patients with cardiac complications had 4 to 5-fold increased odds of stroke or combined risk of death and stroke. CONCLUSION: Minor surgical complications are common after CEA and are associated with much higher risk of death and stroke. Patient factors, process factors, and direct causality are involved in this relationship, but future work will be needed to better understand their relative contributions.

Nasopharyngeal carcinoma with cranial nerve palsy: the importance of MRI for radiotherapy.

PURPOSE: To evaluate various prognostic factors and the impact of imaging modalities on tumor control in patients with nasopharyngeal cancer (NPC) with cranial nerve (CN) palsy. MATERIAL AND METHODS: Between September 1979 and December 2000, 330 NPC patients with CN palsy received radical radiotherapy (RT) by the conventional opposing technique at Chang Gung Memorial Hospital-Linkou. Imaging methods used varied over that period, and included conventional tomography (Tm) for 47 patients, computerized tomography (CT) for 195 patients, and magnetic resonance image (MRI) for 88 patients. Upper CN (II-VI) palsy was found in 268 patients, lower CN (IX-XII) in 13, and 49 patients had both. The most commonly involved CN were V or VI or both (23%, 12%, and 16%, respectively). All patients had good performance status (World Health Organization <2). The median external RT dose was 70.2 Gy (range, 63-77.5 Gy). Brachytherapy was also given to 156 patients in addition to external RT, delivered by the remote after-loading, high-dose-rate technique. A total of 139 patients received cisplatin-based chemotherapy, in 115 received as neoadjuvant or adjuvant chemotherapy and in 24 concomitant with RT. Recovery from CN palsy occurred in 171 patients during or after radiotherapy. Patients who died without a specific cause identified were regarded as having died with persistent disease. RESULTS: The 3-year, 5-year, and 10-year overall survival was 47.1%, 34.4%, and 22.2%. The 3-year, 5-year, and 10-year disease-specific survival (DSS) rates were 50.4%, 37.8%, and 25.9%. The 5-year DSS for patients staged with MRI, CT, and Tm were 46.9%, 36.7%, and 21.9%, respectively (p = 0.016). The difference between MRI and CT was significant (p = 0.015). The 3-year and 5-year local control rates were 62% and 53%, respectively. The 5-year local control was 68.2% if excluding patients who died without a specific cause. Patients who had an MRI had a significantly better tumor control rate than those evaluated with CT or Tm, with a 15-30% improvement in local tumor control and survival. Patients with extensive CN palsy had worse survival than those with only lower CN or upper CN involvement (5-year DSS 20.4% vs. 43.2% and 40.4%, respectively; p < 0.001). Patients who recovered from CN palsy had better survival than those who did not (47% vs. 26%, p < 0.001). Brachytherapy was associated with poorer local control, whereas a total external dose of more than 70 Gy improved local tumor control and marginally improved DSS. Subgroup analysis in CT and MRI patients group, either DSS or OS was significantly associated with imaging modality, N stage, or location of or remission of CN palsy. CONCLUSION: The use of MRI was associated with improved tumor control and survival of patients with NPC causing CN palsy. Patients recovering from CN palsy had better survival. A higher radiation dose delivered by external beam may achieve better tumor control than brachytherapy.

The role of Gamma Knife radiosurgery in the management of glomus jugular tumours.

BACKGROUND: Glomus jugular tumours are usually managed by microsurgical resection and/or radiotherapy with considerable risk for treatment-related morbidity. The role of Gamma Knife Radiosurgery (GKRS) in the management of these lesions remains to be defined. METHOD: Between May 1992 and November 2000, 19 patients with glomus tumours underwent GKRS at our department. Nine patients received radiosurgery for residual or recurrent paragangliomas following microsurgical resection and in 10 cases GKRS was performed as primary treatment. The median tumour volume was 5.22 ccm (range: 0.38-33.5 ccm). Marginal doses of 12-20 Gy (median 14 Gy) were applied to enveloping isodose volume curves (Range: 30-55%, median 50%). FINDINGS: Except for an 81-year-old patient who died 9 months after radiosurgery the observation time ranged from 1.5 to 10 years (median 7.2 yrs). The total tumour control rate was 94.7% (7 cases with decreased and 11 with stable tumour size). The only patient with tumour progression (5.3%) underwent repeated radiosurgical treatment 85 months after initial GKRS. A newly diagnosed second lesion in the cavernous sinus was treated radiosurgically as well 53 months after the first Gamma Knife procedure. On clinical examination 10 patients (52.6%) presented with improved and 8 patients (42.1%) with unchanged neurological status. Deterioration in one patient (5.3%) was not related to tumour or radiosurgery. INTERPRETATION: As GKRS demonstrated to be a minimally invasive treatment alternative to microsurgery and radiotherapy with no acute or chronic toxicity it should be considered more frequently in the primary or adjuvant strategy for glomus jugular tumours.

Role of radiation therapy to the brain in leukemic patients with cranial nerve palsies in the absence of radiological findings.

The value of brain radiotherapy for leukemic patients with cranial nerve palsies in the absence of radiological evidence of leukemic infiltration is not well defined. This retrospective study was undertaken to evaluate the effectiveness of brain irradiation in reversing the cranial nerve palsies in leukemic patients with no radiological evidence of intracranial leukemic infiltration. Records of leukemic patients who received brain radiotherapy between June 1980 and December 1993 were reviewed. Criteria for inclusion were 1) no evidence of intracranial leukemic infiltration by computed axial tomography (CT) or magnetic resonance imaging scan (MRI), 2) no evidence of leukemic infiltration on ophthalmologic examination, and 3) no previous radiotherapy to the brain. Actuarial survival rates were calculated using the Kaplan-Meier method. Pearson's chi-squared test was used to compare responses. Twenty-eight patients met these criteria. The median age was 38 years (range 3-75 years): Seventeen patients had acute lymphoblastic leukemia, nine had acute myelogenous leukemia, and two had chronic myelogenous leukemia. Four patients had initial presentation with leukemia, and 24 presented with relapse. Twenty-six patients had cerebrospinal fluid cytology that was positive for leukemic cells. Fifteen patients had involvement of more than one cranial nerve, and nine had bilateral involvement. The most commonly involved nerves were the facial (n = 18), oculomotor (n = 9), and abducens nerves (n = 8). Twenty-six patients received whole-brain radiotherapy. Two received radiation to the base of the skull only. The median radiation dose was 24 Gy (range 16-30 Gy) at 2-3 Gy per fraction. Every patient had either concomitant intrathecal (n = 6) or systemic (n = 5) chemotherapy or both (n = 17) with radiation. Fourteen patients had complete reversal of the cranial nerve deficit, eight had partial recovery, and four had no response or progression of the disease. The response was unknown in two patients. Factors associated with complete response were unilateral versus bilateral involvement (72% vs. 13%, P = 0.005) and single versus multiple nerve involvement (75% vs. 36%, P = 0.045). In conclusion, radiation therapy to whole brain was effective in reversing cranial nerve deficits from leukemia, although the leukemic infiltration may not be visualized by CT or MRI. No dose-response relationship was observed in the range we examined.

Outcome of sixth nerve palsy or paresis in young children.

This study evaluated the ophthalmological outcome following sixth nerve palsy or paresis in 64 children 7 years of age and younger. The outcomes considered were vision, residual strabismus and the need for strabismus surgery. Etiologies included tumor, hydrocephalus, trauma, infection, malformation, and idiopathic and miscellaneous causes. Strabismus surgery was performed on 24% of the patients, with residual strabismus present in 66% of the patients. Neurologists and ophthalmologists should monitor visual acuity in these young children at frequent intervals be prepared to institute amblyopia therapy early in the course of the ocular misalignment if permanent visual disability is to be avoided.

Cranial neuropathy in systemic malignancy in a VA population.

We studied 30 men with cranial neuropathy (CN) and systemic malignancy to determine the etiology and prognosis of CN. The most common malignancy was prostate (33%), followed by lung (27%). The etiology of CN was metastatic in 25 (83%) and nonmetastatic in 5 (17%). Only 6 patients improved: 3 metastatic and 3 nonmetastatic. Although prognosis for recovery was better in the nonmetastatic group, there was no significant difference in survival between the metastatic and the nonmetastatic groups.

Amyotrophic lateral sclerosis. Its natural history.

ALS is the most common of the various MNDs, which also include the clinical entities of PBP, PMA, and PLS. Mean age of onset of ALS is 57 years, and there is a sex predilection for men in a ratio of 1.5:1. Area of first symptom is in the lower extremity is 36 per cent of cases, in the upper extremity in 32 per cent, and 25 per cent of patients have a bulbar onset. Motor cranial nerves in the lower brain stem from cranial nerve nuclei or corticobulbar tract degeneration are affected. This results in symptoms of speech and swallowing difficulty and emotional lability in up to 60 per cent of cases. One hundred per cent of cases have motor weakness, over 90 per cent have muscle atrophy and fasciculations, and 47 per cent have spasticity. There seems to be a pattern of progression of ALS signs and symptoms based on area of onset with LLE involvement tending to follow RLE weakness, LUE weakness following RUE onset, and RUE involvement following next in patients whose onset is bulbar. Significant numbers of ALS patients had sparing of involvement of parts of the body for follow-up times approaching 3 years. Although the majority of patients experience a deterioration that is significantly linear, seven of 28 patients or 25 per cent achieved a plateau lasting a minimum of 9 months. Survival in our series was 4.08 years for all forms of MND.(ABSTRACT TRUNCATED AT 250 WORDS)