Duration Of Neurological Symptoms Research Articles

Phenotypic expression of swallowing function in Niemann–Pick disease type C1

BackgroundNiemann–Pick disease type C1 (NPC1) is a rare autosomal recessive disease characterized by endolysosomal accumulation of unesterified cholesterol with progressive deterioration in swallowing, often leading to premature death. Although documented, the natural history of NPC1 swallowing dysfunction has yet to be delineated systematically. This manuscript aims to provide a comprehensive characterization of the phenotypic spectrum and progression of swallowing dysfunction in NPC1.MethodologyThe National Institutes of Health (NIH) NPC1 natural history study (NCT00344331) enrolled 120 patients, who underwent comprehensive interpretative swallow assessments for swallowing safety, dietary modifications, and aspiration risk. Longitudinal statistical modeling accounted for all outcomes with NPC1 disease covariates (first symptom onset, age at neurological symptom onset, seizure history, duration of neurological symptoms) as well as miglustat use (a glucosylceramide synthase inhibitor) and NIH study duration (NIHSD; the length of time an individual participated in the NIH study). Probabilities for disease progression and time to swallowing decline were conducted for the entire cohort.ResultsTime to swallowing decline with American Speech-Language-Hearing Association National Outcome Measure (ASHA-NOMS) and the NIH-adapted Penetration Aspiration Scale (NIH-PAS) were identified: frac{7.7}{100} person-years and frac{9.8}{100} person-years, respectively. NIHSD and seizure history consistently and significantly were associated with decline (ORNIHSD = 1.34–2.10, 95% CI 1.04–3.4, p = 0.001–0.026; ORSeizure = 3.26–18.22, 1.03–167.79; p = 0.001–0.046), while miglustat use revealed protection (ORMiglustat = 0.01–0.43, 0.007–0.98; p = 0.001–0.044). The probability of decline with NPC1 neurological severity scale and annual severity increment scale were established with the aforementioned covariates, varying amongst subgroups.ConclusionThis study represents the most extensive collection of prospective, instrumental swallowing assessments in NPC1 to date with an interpretive analysis providing an improved understanding of NPC1 disease progression with swallowing function—serving as a foundation for clinical management and future NPC1 therapeutics.

Clinical characteristics and therapeutic outcomes of mediastinal neuroblastoma with intraspinal extension: a retrospective study.

BackgroundMediastinal neuroblastoma (NB) can invade the spinal canal and result in spinal cord compression. Some patients go on to develop severe spinal deformities after decompression of the spinal cord. The optimal therapeutic strategy for mediastinal NB with intraspinal extension is still unclear. Our study is to assess the therapeutic strategies for such patients.MethodsA total of 77 patients suffered mediastinal tumors with intraspinal extension between March 2015 and Aug 2019 were enrolled in the study. According to the primary therapy, NB were classified into 4 groups: chemotherapy, video-assisted thoracoscopic surgery (VATS)/thoracotomy, neurosurgical decompression, and a combined thoracic-neurosurgical approach. Clinical features, including patient demographics, neurologic recovery and survival rate, were assessed.ResultsAmong the 77 patients suffered mediastinal tumors with intraspinal extension, neurological symptoms were present in 44 patients. Neurological deficits improved in 76.5% of patients who underwent neurosurgical intervention and 50% of the other patients (P=0.094). Compression manifestations of ≤4 weeks duration showed an improved outcome compared to a longer compression time, with complete recovery of neurological function in 60% of patients versus 28.6% for patients with a longer symptom duration (P=0.04). NB constituted 49.4% of the 77 patients. An overall survival rate of 90.0%±9.5% was achieved for patients in the combined thoracic-neurosurgical group, 59.5%±15.0% in the thoracotomy group, 40.0%±29.7% in laminectomy group, and 37.0%±20.2% in the chemotherapy group. Complete regression of the tumor was demonstrated in 80% of combined group, which was greater than that of patients in the other groups (P=0.001).ConclusionsNeurological recovery was correlated with the type of initial treatment and the duration of neurological symptoms. Mediastinal NB with intraspinal extension can be effectively managed with a combined neurosurgical and thoracic surgical approach.

Occurrence of erythema migrans in children with Lyme neuroborreliosis and the association with clinical characteristics and outcome \u2013 a prospective cohort study

BackgroundErythema migrans (EM) is the most common manifestation of Lyme borreliosis (LB), caused by the spirochete Borrelia burgdorferi sensu lato. The infection can disseminate into the nervous system and cause Lyme neuroborreliosis (LNB), the second most frequent LB manifestation in children. The aim of this prospective cohort study is to describe the occurrence of EM among children with LNB and to evaluate possible differences in clinical characteristics or outcome between LNB patients with and without EM.MethodChildren being evaluated for LNB in southeast Sweden during the period 2010–2014 underwent a clinical examination, laboratory testing and filled out a questionnaire regarding duration and nature of symptoms, EM and the child’s health. Children were classified according to European guidelines for LNB. Clinical recovery was evaluated at a 2-month follow-up.ResultsThe occurrence of EM among children with LNB was 37 out of 103 (36%). Gender, age, observed tick bite, clinical features, duration of neurological symptoms or clinical outcome did not differ significantly between LNB patients with or without EM. However, facial nerve palsy was significantly more common among children with EM in the head and neck area.ConclusionEM occurred in 36% of children with LNB and the location on the head and neck was more common among children with facial nerve palsy. EM was not associated with other specific clinical characteristics or outcome. Thus, the occurrence of EM in children with LNB cannot be useful as a prognostic factor for clinical outcome. This aspect has not previously been highlighted but seems to be relevant for the paediatrician in a clinical setting.

Course and Outcome of Early European Lyme Neuroborreliosis (Bannwarth Syndrome): Clinical and Laboratory Findings.

Information on the course and outcome of early European Lyme neuroborreliosis is limited. The study comprised 77 patients (38 males, 39 females; median age, 58 years) diagnosed with painful meningoradiculitis (Bannwarth syndrome) who were followed up for 1 year at a single center. Duration of neurological symptoms before diagnosis was 30 (interquartile range, 14-50) days. The most frequent symptoms/signs were radicular pain (100%), sleep disturbances (75.3%), erythema migrans (59.7%), headache (46.8%), fatigue (44.2%), malaise (39%), paresthesias (32.5%), peripheral facial palsy (PFP) (36.4%), meningeal signs (19.5%), and pareses (7.8%). Cerebrospinal fluid (CSF) analysis revealed lymphocytic/monocytic pleocytosis, elevated protein concentration, and intrathecal synthesis of borrelial immunoglobulin M and immunoglobulin G antibody in 100%, 81.1%, 63%, and 88.7% of patients, respectively. Borreliae (predominantly Borrelia garinii) were isolated from CSF, skin, and blood in 15.6%, 40.6%, and 2.7% of patients, respectively. The outcome after 14-day treatment with ceftriaxone was favorable in 87.8% of patients. Control CSF examination at 3 months showed decreased leukocyte counts in all patients; however, 23.3% still had pleocytosis (>10 × 10(6) cells/L). A model based on pretreatment data and the findings at the end of 14-day antibiotic treatment accurately predicted which patients would have an unfavorable outcome 6 or 12 months after treatment. Our patients had fewer pretreatment neurological complications (PFP, pareses) than reported for Bannwarth syndrome decades ago, probably as the result of earlier recognition and prompt antibiotic treatment. Unfavorable outcome was rare and was predicted by the continued presence of symptoms 14 days after commencement of treatment.

Spinal cord concussion: studying the potential risks of repetitive injury.

What is spinal concussion? Spinal cord concussion is a variant of mild spinal cord injury, clinically designated as transient paraplegia or neurapraxia, and characterized by variable degrees of sensory impairment and motor weakness that typically resolve within 24–72 hours without permanent deficits (Del Bigio and Johnson, 1989; Zwimpfer and Bernstein, 1990; Torg et al., 1997). Accordingly, a grading system was developed based on the duration of symptoms, ranging from Grade I ( 24 hours) (Zwimpfer and Bernstein, 1990; Torg et al., 1997). Spinal cord concussion is predominantly a sport-related injury occurring in a wide variety of contact sports in adult and pediatric athletes including wrestling, hockey, gymnastics, and diving, but most commonly in American football, although spinal concussions can also occur after minor car accidents as “whiplash injuries” and falls (Zwimpfer and Bernstein, 1990). In contact sports, the cervical spine is particularly susceptible to injury because of axial loading forces to the head with the neck in flexion or extension. In these circumstances, injury may occur due to disc herniation, buckling of the ligamentum flavum or the posterior longitudinal ligament, or by compression of the spinal cord between vertebral bodies. There appears to be a mechanistic difference in the injury between adult and pediatric age groups. In the adult, a stenotic spinal canal or a diminished spinal canal to vertebral body diameter predispose patients to cervical concussion at the level of stenosis after hyperextension, hyperflexion, or axial loading. The pediatric spine, in contrast, has increased mobility, predisposing the spinal cord to contact with bony elements even in absence of focal stenosis (Clark et al., 2011). Guidelines regarding return to play have been developed based upon the duration of neurological symptoms, neurapraxia, and MRI analysis, but they are based on a limited number of small-scale retrospective studies (Tempel et al., 2015). Given the lack of randomized trials, medical clearance of athletes for resumption of activities is a highly controversial topic without consensus opinion (Cantu, 1998; Morganti, 2003; Mayers, 2008; Harmon et al., 2013). Furthermore, many individuals may experience multiple episodes of spinal cord concussion with recurrence of symptoms (Zwimpfer and Bernstein, 1990; Torreman et al., 1996; Clark et al., 2011). It has therefore been important to develop animal models of spinal cord concussion to elucidate the histological and functional deficits of single and repeated injuries. This perspective presents our recent efforts in developing such a model and the consequences of repetitive injury (Jin et al., 2014, 2015).

Predictors of meningioma consistency: A study in 243 consecutive cases

Meningioma is a common neoplasm primarily arising in the central nervous system. Its consistency is considered to be one of the critical prognostic factors for determining surgical resectability. The present study endeavored to investigate predictive factors associated with the tumor consistency. Two hundred and forty-three consecutive participants who underwent resective surgery of meningioma were examined. The authors designed an objective grading system for meningioma consistency and utilized it for assessing consistency among all cases. We focused on the relationship between preoperative tumor characteristics on neuroimaging studies and the consistency. The tumor attributes on T2-weighted image (T2WI) and fluid attenuated inversion recovery (FLAIR) image were significantly correlated with the tumor consistency (p = 0.004 and 0.045, respectively). The hypointense tumors on both MRI sequences tended to be hard, whereas the tumors showing hypersignal intensity were associated with soft consistency. There was no correlation between the consistency and age, gender, duration of neurologic symptoms, tumor location, size, calcification, cystic portion, en plague appearance, tumor-brain contact interface expressed by cerebrospinal fluid (CSF) cleft, perilesional vasogenic edema, bony status, features on T1-weighted image (T1WI) and pattern of contrast enhancement. In multiple logistic regression analysis, the tumor characteristics on T2WI and FLAIR image were independent factors significantly correlated with the tumor consistency (p = 0.005 and 0.041, respectively). The tumor consistency was also correlated with operative radicalness as evaluated by the Simpson criteria. Signal intensity on T2WI and FLAIR image can be used for insinuating meningioma consistency. Presurgical prediction of the consistency is highly valuable in operative planning, particularly in arduous cases.

Spinal cord compression in children with Wilms’ tumour

To document the clinical, imaging and histopathological features of five children with paraplegia due to Wilms' tumour (WT), highlighting therapeutic options and patient outcome in a developing country. Patients with WT and paraplegia seen at the Department of Paediatric Surgery since 1984 form the study cohort. Patient demographics, duration of neurological symptoms, stage of primary tumour, therapeutic intervention and outcome were recorded. Histology of the primary tumours and paraspinal or epidural biopsies were reviewed. Five patients with WT and paraplegia were identified. Imaging showed epidural masses with paraspinal disease, cord displacement and compression. Four patients have died. Of the two patients with neurological recovery, one relapsed 4 months later. Histology revealed triphasic WT with one case showing anaplasia. Paraspinal or epidural biopsies confirmed WT with post-treatment changes. Three biopsies showed lymphovascular, perineurial and intraneural tumour invasion and one showed epidural venous invasion. Although rare, WT-associated spinal disease may cause permanent neurological deficit, adding considerably to the burden of disease. In developing countries where patients present late, the prognosis is poor, however surgery may provide immediate relief of compression symptoms and biopsy material. The treatment of choice will depend on the facilities available and the clinical circumstances.

Wilson’s disease: two treatment modalities. Correlations to pretreatment and posttreatment brain MRI

Brain magnetic resonance imaging (MRI) studies on Wilson's disease (WD) show lack of correlations between neurological and neuroimaging features. Long-term follow-up reports with sequential brain MRI in patients with neurological WD comparing different modalities of treatment are scarce. Eighteen patients with neurological WD underwent pretreatment and posttreatment brain MRI scans to evaluate the range of abnormalities and the evolution along these different periods. All patients underwent at least two MRI scans at different intervals, up to 11 years after the beginning of treatment. MRI findings were correlated with clinical picture, clinical severity, duration of neurological symptoms, and treatment with two different drugs. Patients were divided into two groups according to treatment: D: -penicillamine (D-P), zinc (Zn), and Zn after the onset of severe intolerance to D-P. MRI scans before treatment showed, in all patients, hypersignal intensity lesions on T2- and proton-density-weighted images bilaterally and symmetrically at basal nuclei, thalamus, brain stem, cerebellum, brain cortex, and brain white matter. The most common neurological symptoms were: dysarthria, parkinsonism, dystonia, tremor, psychiatric disturbances, dysphagia, risus sardonicus, ataxia, chorea, and athetosis. From the neurological point of view, there was no difference on the evolution between the group treated exclusively with D-P and the one treated with Zn. Analysis of MRI scans with longer intervals after the beginning of treatment depicted a trend for neuroimaging worsening, without neurological correspondence, among patients treated with Zn. Neuroimaging pattern of evolution was more favorable for the group that received exclusively D-P.

Transient Ischemic Attack: A Dangerous Harbinger and an Opportunity to Intervene

Transient ischemic attack (TIA) and ischemic stroke are both characterized by sudden onset of neurological symptoms due to focal cerebral ischemia, but they are distinguished by the duration of neurological symptoms, with TIA traditionally defined by resolution of symptoms within 24 hours and stroke reserved for symptoms of longer duration. Because TIA and ischemic stroke share etiologies, it is not surprising that the recommended evaluations and secondary prophylaxis are identical. However, recent studies suggest that optimal management of TIA and stroke may differ more than previously recognized. The short-term risk of ischemic stroke after TIA is very high, which may be because rapid recovery from neurological symptoms is indicative of reversal of ischemia and tissue still at risk. Regardless of whether residual symptoms or infarction are present, rapid recovery appears to predict a greater short-term risk of subsequent ischemic stroke and is more likely to reflect a distinct, unstable pathophysiology. Therefore, it may be more useful to characterize acute ischemic cerebrovascular syndromes on the basis of the extent of rapid recovery (i.e., on the inferred reversal of ischemia) than on the completeness of recovery at 24 hours. Patients with substantial rapid recovery may be those for whom acute intervention is most warranted.

８０歳以上の超高齢者における脊椎脊髄手術の効果と問題点

The object of this study was to investigate surgical results and problems associated with spinal surgery in patients older than 80 years of age. Since April, 2003, we have performed 25 operations in 20 elderly patients (7 men and 13 women with ages ranging from 80-89 years) for lumbar lesions (14 cases) and cervical lesions (6 cases). Data obtained from chart review included age, sex, duration of neurological symptoms, associated illnesses, type of anesthesia, time of operation, blood loss during operation, and complications attributable to surgery. Neurological deficits before and after surgery were assessed using the Japanese Orthopedic Association (JOA) score for lumbar lesions, and the Neurosurgical Cervical Spine Scale (NCSS) for cervical lesions. Four patients had histories of heart disease and one had a history of lung disease. Twelve patients with lumbar lesions underwent surgery under lumbar anesthesia, and 2 patients general anesthesia. Mean operation time was 98 minutes, and mean blood loss during surgery was 54 grams in patient with lumbar lesions, and 260 minutes, 138 grams with cervical lesions. Two patients with both cervical and lumbar lesions underwent cervical and lumbar operations simultaneously. In patients with lumbar lesions, the preoperative mean JOA score was 12, and postoperative 19.3, and the mean recovery rate was 43.3%. In patients with cervical lesions, the preoperative mean NCSS was 5.8 and postoperative 8.0, and the mean recovery rate was 29.3%. There were no deaths and three patients had postoperative complications including transient foot drop in one, hypotension in one, and instrumentation failure in the other. Our data suggest that spinal surgery is a reasonable alternative with satisfactory results in patients older than 80 years who are in good general health.

Evidence of Cellular Immune Activation in Children With Opsoclonus-Myoclonus: Cerebrospinal Fluid Neopterin

To evaluate cellular immune activation in opsoclonus-myoclonus syndrome, we measured the inflammatory marker neopterin in the cerebrospinal fluid of 16 children with opsoclonus-myoclonus and neuroblastoma, 24 children with opsoclonus-myoclonus but no tumor, and 19 age-matched controls. The mean concentration in opsoclonus-myoclonus was 2.3-fold higher than in controls (P = .008). Neopterin was greatly elevated in four of the most neurologically severe cases, up to 8.3-fold above the highest control level. Thirteen of the 40 children with opsoclonus-myoclonus but no controls had a neopterin concentration >2 SD above the control mean (P = .005). In this high neopterin subgroup, neurologic severity was significantly greater and the duration of neurologic symptoms was less. In 16 children re-examined on immunotherapy, including adrenocorticotropic hormone (ACTH) combination therapy, treatment was associated with a significant reduction in both neopterin and neurologic severity. Neopterin did not differ significantly between the tumor and non-tumor opsoclonus-myoclonus etiologies. No abnormalities of tetrahydrobiopterin were found. Although cerebrospinal fluid neopterin lacked the sensitivity to be a biomarker of disease activity in opsoclonus-myoclonus, elevated concentrations do support a role for T-cell activation and cell-mediated immunity in its pathophysiology.

End-stage renal disease and dialysis in hereditary amyloidosis TTR V30M: presentation, survival and prognostic factors

Classical familial amyloid polyneuropathy may have a course with progressive renal impairment. We studied 62 patients (24 males, 38 females) with FAP, transthyretin variant V30M, and end-stage renal disease (ESRD) treated with hemodialysis, all referred to a single center over a period of 11 years. Clinical course, morbidity and survival after dialysis were analyzed. Patient's mean age at first dialysis was 51.5 ± 10.7 years, and mean duration of neuropathy was 10.2 ± 3.8 years. The most frequent form of presentation of FAP nephropathy was nephrotic proteinuria with renal dysfunction. In the year prior to dialysis, renal function declined rapidly, and fluid overload was the main indication to initiate treatment. The presence of decubitus ulcers, significant disability, venous catheter for definitive vascular access for long-term treatment, and permanent bladder catheter, were related to death during the first year of dialysis. The mean duration of renal replacement therapy was 21 months, with a 54.5% one year, and 38.4% two year treatment survival. However, when the duration of neurological symptoms at first dialysis exceeded 10 years, survival was significantly lower. Infections, (41% were decubitus ulcers with sepsis) were the cause of early, as well as late mortality. Early creation of vascular access for hemodialysis, surveillance of skin wounds, and intervention on neurogenic bladder are essential to improve the prognosis of ESRD in FAP.

Syndrome opsoclonus-myoclonus associé au neuroblastome non métastatique. Suivi à long terme. Étude de la Société française d'oncologie pédiatrique

Opsoclonus-myoclonus is a rare syndrome characterized by multidirectional chaotic eye movements, myoclonus and ataxia. In children, it could be a paraneoplastic syndrome in association with neuroblastoma, usually with a high survival rate, but having a high frequency of neurologic and psychologic sequelae. The aim of this study was to describe oncologic outcome (prospectively) and neurologic outcome (retrospectively) in children with non-metastatic neuroblastoma, and to determine its best treatment. Data were collected on 21 children diagnosed with localized neuroblastoma and opsoclonus-myoclonus between 1990-1999 from the French Society of Pediatric Oncology institutions. Median age at diagnosis was 18 months. Location of the tumor was abdominal in 14 cases, thoracic in three cases, pelvic in three cases, and cervical in the last case. There was a majority of small tumors with a maximal diameter < 5 cm in 13 cases. Only four tumors were initially considered as unresectable tumors and received first-line chemotherapy. Complete macroscopic resection was performed in 20 cases (four after primary chemotherapy). Nine children received chemotherapy. Twenty children remained in first complete remission, and one relapsed and died (the unique NMYC amplified case). Treatment for opsoclonus-myoclonus varied widely. Only one child received no medical treatment for opsoclonus-myoclonus, because of complete resolution of neurologic symptoms after exclusive surgery. The following agents were used: corticosteroids in 18 cases, intravenously immune globulin in five cases, and antiepileptic drugs in seven cases. Ten patients experienced relapses of opsoclonus-myoclonus symptoms, mainly related to the decrease of steroid therapy (5/10). Ten of 16 assessable children had persistent neurologic deficits including speech delay or cognitive deficits (8/16), ataxia (6/16), motor delay (2/16), and behavioral problems (2/16). There is no correlation between neurologic outcome, and either age at diagnosis or duration of neurologic symptoms, or type of treatment of the tumor, particularly chemotherapy. Persistent neurologic deficits are characteristic for children with neuroblastoma and opsoclonus-myoclonus. Neurologic outcome seems unrelated to the treatment of neuroblastoma, which should exclusively be conducted according to oncological criteria. The treatment of opsoclonus-myoclonus should be standardized, mainly based on high-dose hydrocortisone, with a very low decreasing dosage, associated to intravenously immune globulin in severe cases. A biological immunologic work-up of the disease and cautious neurologic and psychologic standardized follow-up should be performed.

Spinal Angiolipoma

Spinal extradural angiolipomas are distinct, benign, and rare lesions composed of mature lipocytes admixed with abnormal blood vessels. They account for 0.14% of all spinal axis tumors. The case described here was a 72-year-old patient presenting with a history of paraparesis, hypoesthesia under the T2 level, hyperreflexia, and urinary overflow incontinence that appeared within 7 days after the administration of a coronary vasodilator drug regimen. The spinal magnetic resonance scan showed a lipomatous mass with signal void lesions, suggesting a vascular component of the tumor. The patient improved rapidly after surgical resection of the epidural tumor and decompression of the cord. According to the present literature, the duration of neurological symptoms ranges from 1 to 180 months (mean 28 months). But this patient's neurological deterioration took place 4 days before hospitalization. We believe that this can be explained by the increased tumor blood volume caused by vasodilator drugs, which in turn exerted a pulsatile compressive effect on the cord.

Astroglial and neuronal proteins in cerebrospinal fluid as markers of CNS involvement in Lyme neuroborreliosis.

Is Lyme neuroborreliosis, even in its early phase, a parenchymatous disorder in the central nervous system (CNS), and not merely a meningitic process? We quantified cerebrospinal fluid (CSF) levels of four nerve and glial cell marker proteins in Lyme neuroborreliosis patients with pretreatment durations of 7-240 days. All 23 patients had meningoradiculitis, and six had objective signs of encephalopathy. Glial fibrillary acidic protein (GFAp) pretreatment levels in CSF, and the light subunit of neurofilament protein (NFL) levels were related to clinical outcome and declined significantly after treatment (P < 0.001 and P < 0.01, respectively). NFL was detectable in 11 out of 22 patients, and pre- and post-treatment NFL levels were associated with the duration of neurological symptoms within 100 days prior to treatment. Neuron-specific enolase (NSE) concentrations also decreased after therapy (P < 0.001), while CSF levels of glial S-100 protein remained unchanged. The pretreatment duration of disease was related to postinfectious sequelae. GFAp, NSE and NFL levels in CSF are unspecific indicators of astroglial and neuronal involvement in CNS disease. The findings in the present study are in agreement with the hypothesis that early and late stages of Lyme neuroborreliosis damage the CNS parenchyma.

Brain calcification in patients with cerebral lupus.

Cerebral lupus (CL) is a common cause of morbidity and mortality in patients with SLE. The brain CTs of 27 consecutive adult patients with SLE and various neurological presentations were reviewed. The median age and duration of neurological symptoms at the time of the brain CT were 30 years (range = 14-51 years) and six days (range = 1 day-22 years), respectively. Eleven patients (41%) had normal CTs. The abnormalities in the remaining patients could be divided into six categories: (a) cerebral atrophy alone (two patients); (b) calcification alone (three patients); (c) infarct(s) alone (five patients); (d) cerebral atrophy and calcification (three patients); (e) cerebral atrophy and infarct(s) (one patient) and (f) cerebral atrophy, calcification and infarct(s) (two patients). Altogether eight patients (30%) (age range = 17-47 years) had intracerebral calcification: the globus pallidus was involved in all, putamen in two, head of the caudate nucleus in one, thalamus in one, centrum semiovale in two and cerebellum in three patients. Two patients had extensive calcifications of most of the basal ganglia, centrum semiovale and cerebellum. There was no relationship between the presence/degree of calcification and age of patients/duration or type of neurological presentation. The pathogenesis of cerebral calcification in CL is unknown. Cerebral lupus must now be included in the differential diagnosis of intracerebral calcification.

Peripheral nerve abnormalities in adrenomyeloneuropathy

Adrenomyeloneuropathy (AMN) is one of the most frequent phenotypes of X-linked adrenoleukodystrophy. Whether the polyneuropathy in AMN results from primary demyelination or axonal degeneration is uncertain. We examined 23 patients (18 men with AMN and five female carrier with AMN symptomatology), performed electroneurography and EMG, and compared our results with standardized electrodiagnostic criteria for primary demyelination. Both clinically and electrodiagnostically, the lower extremities were most frequently and most severely affected. A longer duration of symptoms was related to more severe pyramidal dysfunction (p < 0.004) and spasticity (p < 0.04), and to a more severe impairment of vibration sense (p < 0.05). There were on correlations between the different electrophysiologic studies and the duration of neurologic symptoms. Only two AMN patients (9%) fulfilled the electrodiagnostic criteria for primary demyelination. However, both had abnormally low compound muscle action potentials, which may have been a reflection of primary axonal degeneration. Six other patients (26%) partially fulfilled the criteria for primary demyelination, of whom five also manifested low compound muscle action potentials. In 15 patients (65%), we found polyneuropathy with predominantly axonal, sensorimotor features. We conclude that the neuropathy in AMN patients is due to primary axonal degeneration.

Surgical results and complications in elderly patients with benign lesions of the spinal canal.

To describe the long-term outcome and complications of spinal surgery in a population of elderly patients. Retrospective chart review and clinical follow-up. A 700-bed tertiary care midwestern hospital. Patients 70 years of age or older who underwent elective surgical procedures for the treatment of benign lesions of the spinal canal. Data obtained from chart review included age, sex, nature and duration of neurological symptoms, activities of daily living (ADL), associated illnesses, length of hospitalization, type of surgery, and complications attributable to surgery. Outcome measurements included changes in neurological symptoms and ADL and a standardized scale obtained from consecutive outpatient visits, telephone interviews, or written responses. Seventy-eight patients with lumbar canal stenosis or soft disc herniations underwent laminectomies, foraminotomies or discectomies. Eleven patients underwent cervical laminectomies for multilevel spondylitic myelopathy. Limb and spine pain were the most common neurological symptoms in patients with lumbar lesions; diminished ADLs were present in eight patients with cervical myelopathies. Six patients died of unrelated causes, and 78 were followed for an average of 27.1 months. Twenty-three (85.2%) of 27 patients with lumbar disc herniations reported improvements or resolution of limb pain while 35 (81.4%) of 43 patients with lumbar canal stenosis reported fair or good results. Five of eight patients with multilevel cervical spondylosis reported improvements of myelopathic symptoms. Twenty-six (28.6%) complications were attributable to 91 surgical procedures including five severe complications and one fatality. Patients with three or more associated illnesses demonstrated a significantly higher incidence of postoperative complications. Elderly patients with benign lesions of the spinal canal may achieve significant reduction in limb pain and increases in ADL following appropriate surgery, with a tolerable rate of severe complications. The preoperative physical condition of the elderly patient served as an indicator for operative morbidity.

Cognitive impairment in patients with clinically isolated lesions of the type seen in multiple sclerosis. A psychometric and MRI study.

This study reports the performance on psychometric tests of a group of 48 patients with clinically isolated lesions of the type seen in multiple sclerosis (optic neuritis, brainstem and cord lesions). The cognitive functions studied were: 'IQ deficit', verbal and visual memory, abstracting ability, visual and auditory attention, and naming ability. The presence of brain pathology was investigated by means of magnetic resonance imaging (MRI). A group of 46 patients with rheumatic and neurological conditions not known to cause brain disease was used for comparison. Normative MRI data were obtained from a group of 40 normal volunteers. An overall 'Cognitive Ability Index' was significantly worse in patients with clinically isolated lesions when compared with physically disabled controls. IQ deficit and defective auditory attention were the more severely affected parameters. These deficits were significantly correlated with the duration of neurological symptoms and with the degree of brain pathology detected by MRI.

Glucose intolerance in Friedreich's Ataxia: Association with insulin resistance and decreased insulin binding

Friedreich's Ataxia (FA) is a neurologic disorder associated with a high prevalence of diabetes mellitus. To assess insulin secretion and insulin resistance, glucose and insulin responses to oral glucose and insulin binding to circulating monocytes and dextran gradient fractionated and unfractionated red blood cells (RBCs) were compared in 11 subjects with FA to 11 age-matched controls. Glucose and insulin responses were elevated from one to three hours after oral glucose in FA. The mean corrected insulin responses were not different while peripheral insulin activity (A) was significantly decreased (1.38 ± 0.22 v 0.77 ± 0.16, control v FA, P < 0.025) indicating the presence of insulin resistance. A significant correlation between the degree of insulin resistance (A) and duration of neurologic symptoms was found ( r = .65. P < 0.025). Resistance to exogenous insulin was confirmed in ten subjects with FA by intravenous insulin tolerance tests (KITT, %/min, 6.25 ± 0.90 v 3.93 ± 0.61, P < .05). Both FA and control groups showed highest insulin binding to fraction A (youngest) RBCs, but no difference was observed between the two groups. However, insulin binding to monocytes was significantly decreased in subjects with FA (% specific binding/10 7 cells/mL, 6.37 ± 0.71 v 4.51 ± 0.39, P < 0.05, control v FA). This was associated with a decrease in apparent receptor affinity. We conclude that FA is associated with insulin resistance, which increases with the duration of neurologic impairment. The insulin binding to monocytes suggests that the insulin resistance may be partially explained by a receptor defect.