Multiple Nerve Involvement Research Articles

Nonsystemic vasculitic neuropathy: insights from a clinical cohort.

Nonsystemic vasculitic neuropathy (NSVN) is an uncommon disorder. Few series with small numbers of patients have been reported. The prognosis and treatment of patients presenting with NSVN remain uninvestigated. The authors sought to address these issues by assembling a large retrospective cohort with extended follow-up. All nerve biopsies performed over 20 years were reviewed; cases with definite, probable, or possible vasculitis were segregated for clinical correlation. Patients satisfying clinical criteria for NSVN at presentation were selected. Clinicopathologic, treatment, and outcome measures were analyzed in patients followed for > or = 6 months. A total of 48 patients (30 women, 18 men) with a median of 63 months of follow-up were identified. Most patients (85%) had extensive, overlapping involvement of multiple nerves. Only one had a symmetric polyneuropathy. Most neuropathies (96%) were painful. In 96%, nerve damage was distally accentuated, but most had concurrent proximal weakness. Diagnostic sensitivity was 58% for superficial peroneal nerve/peroneus brevis muscle biopsy and 47% for sural nerve biopsy. Combination corticosteroid/cytotoxic therapy was more effective than corticosteroid monotherapy in inducing remission and improving disability, with trends toward reduced relapses and chronic pain. Treatment with cyclophosphamide for >6 months decreased the relapse rate, which was 46% for all patients. Disease/treatment-related mortality was 10%. Six percent developed cutaneous involvement. Although chronic pain persisted in 60% of survivors, 80% had good outcomes. NSVN nearly always presents as an asymmetric, distally accentuated, painful, sensorimotor polyneuropathy. Risks for systemic spread and death are small, and, aside from pain, neurologic prognosis is unexpectedly good. Although this was not a randomized controlled trial, combination therapy produced the best outcome in this cohort.

Peripheral Nerve Injury Associated With Shoulder Trauma: A Retrospective Study and Review of the Literature

To determine the incidence of peripheral nerve injury in patients referred to an electrodiagnostic laboratory with a history of shoulder trauma. The characteristics of those presenting with the triad of shoulder dislocation, peripheral nerve injury, and rotator cuff tear were further examined. We conducted a retrospective chart review of all patients referred to our electrodiagnostic laboratory over a 17-month period. Requisitions, clinical histories, physical findings, electrophysiological results, and radiologic investigations were reviewed. Inclusion for analysis was a clinical history of shoulder trauma and electrophysiological evidence of nerve injury. Of 1844 patients studied, 48 had nerve injury associated with trauma to the shoulder. Twenty presented with brachial plexopathies; 17 isolated mononeuropathies (axillary nerve was most common, 47%); and 11 multiple nerve involvement. Of the multiple nerves involved, the axillary was most commonly affected (45% axillary and musculocutaneous; 36% axillary and suprascapular). Fifteen (31%) patients had a history of shoulder dislocation, whereas 5 (10.4%) demonstrated the triad of dislocation, nerve injury, and rotator cuff tear. Peripheral nerve injury is an important consideration in patients with shoulder trauma. For patients presenting with nerve injury post-shoulder dislocation, it is important to consider a potential concomitant rotator cuff tear as an ongoing source of pain or weakness. Similarly, a patient with a rotator cuff tear following dislocation may have an associated peripheral nerve lesion.

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.

Reversal of cranial nerve dysfunction with radiation therapy in adults with lymphoma and leukemia

Twenty patients with leukemic (8) or lymphomatous (12) meningitis and cranial nerve palsies were treated with cranial irradiation at Memorial Sloan-Kettering Cancer Center from June 1987 through December 1988. The diagnosis of malignant meningitis was confirmed by malignant or `suspicious' cerebrospinal fluid cytology in 13 20 patients. The 6th and 7th cranial nerves were most commonly involved. Multiple nerve involvement was present in 11 20 patients, for a total of 42 nerves affected. Treatment consisted of whole brain irradiation to a median dose of 2400 cGy (range: 1000–3000) at 200–300 cGy per fraction. Median survival was 7 months. Forty of 42 nerves improved, for an actuarial response rate of 95% at 3 months. The complete response rate was 44% and was better for patients with lymphoma than for those with leukemia (72% vs 19%, p = 0.04). Delaying initiation of irradiation more than 3 days after the onset of symptoms did not affect the ultimate response rate, but slightly increased the median time to response (9 days vs 14 days, p = 0.1). Radiation therapy is an effective means of reversing cranial nerve dysfunction in leukemia and lymphoma. Higher doses may be needed to increase the rate of complete response.

Cranial nerve involvement with carcinoma of prostate

Eleven patients with known prostate cancer presented with cranial nerve deficits and skull metastases during the course of their disease. All were treated with radiation therapy to the base of skull. Of the 11 patients, 10 (91 %) responded to therapy. Four of the patients in the responder group had complete resolution of the cranial nerve deficits. This response lasted until their death. One patient had a complete response but later relapsed and is still alive. He is the only survivor of the 11 patients. Partial responses were achieved in 5 patients. These patients had either improvement but not resolution of the cranial nerve deficit or, in cases of multiple nerve involvement, there was response of some of the involved nerves. Four of the partial responders retained the response until their death. One patient achieved partial response but later relapsed with additional nerve deficits. The development of this problem represented a grave prognostic factor as 10 patients died within a median of five months (range 1–16 months) after presentation. One patient is alive nineteen months later but in poor condition with disease progression. Only 1 patient (9 %) did not achieve any response to therapy. His treatment was not completed due to deterioration of his general health. The seventh cranial nerve was the most frequently involved either alone or in combination with other nerves. The most commonly used treatment schedule was 3,000 rad in 10 treatments (7 cases). We conclude that effective palliation is achieved though short survival is possible.

Pain and dysphagia in patients with squamous carcinomas of the head and neck: the role of perineural spread.

Clinical and pathological features of perineural spread have been investigated in patients with squamous carcinomas at several sites in the head and neck. In 100 surgical cases, the clinical and pathological findings were congruent in 76%. Combined clinical and histological evidence of perineural invasion was recorded in 33% and the overall incidence of nerve involvement detected morphologically was 44%. Perineural infiltration was demonstrated histologically in 51% of major excisions from the buccal cavity and in 34% of resections from the oropharynx, hypopharynx and cervical oesophagus. The neurological findings were dominated by hypoaesthesia, dysaesthesia and referred pain - mainly in the territories of cranial nerves V and IX. Multiple and/or sequential nerve involvement was occasionally seen. No correlation was established between nerve invasion and metastasis to regional lymph nodes. Long-distance infiltration of nerve trunks, and multiple involvement, are grave prognostic features.In 17 terminal patients submitted to autopsy, 65% had combined clinical and pathological evidence of perineural spread and the overall incidence of nerve involvement detected morphologically was 88%. Sensory changes again predominated. Multiple nerve involvement was observed in 35%. An apparently new `dysphagia syndrome' is described in 4 patients with oropharyngeal carcinomas in whom gross mechanical obstruction was simulated by a combination of perineural spread of tumour into the ipsilateral vagal trunk, sometimes accompanied by segmental infarction, variable invasion of the sympathetic chain, and `splinting' of the pharynx by local fibrosis and tumour in the soft tissues of the neck. Short-term palliation was achieved in these patients with high-dose steroids.

Paralytic Strabismus in Children: Etiologic Incidence and Management of the Third, Fourth, and Sixth Nerve Palsies

The results of a retrospective study of the etiologic incidence of all cases of third, fourth, and sixth nerve palsies in patients at Wills Eye Hospital and St Christopher's Hospital are reported. A total of 121 patients ranging in age from birth to sixteen were studied. Thirty-two cases of third nerve palsy, eighteen cases of fourth nerve palsy, and sixty-two cases of sixth nerve palsy were identified in this series. Nine additional cases illustrated combinations of multiple nerve involvement. With few exceptions, acquired third nerve palsy in children is an ominous sign whereas the congenital form is generally indicative of developmental anomaly or birth trauma. Acquired sixth nerve palsy in a child is always a matter of concern since it may represent the first sign of a brain-stem glioma. Active force studies may be carried out periodically to assess the speed of recovery and indicate when it is time to initiate a more aggressive role in the surgical management. The surgical results in children with paralytic strabismus that have been carefully evaluated offer a favorable prognosis.