Changes In Neurological Symptoms Research Articles

Effect of Nurse's detection of neurological deterioration on the prognosis of patients with acute cerebral infarction

IntroductionThere is little evidence about the factors related to the detection of neurological deterioration by nurses. We examined the related factors and therapeutic outcomes of nurses' detections of patient's neurological deterioration. MethodsThis was a descriptive retrospective study. We included 549 adult stroke patients who were admitted to the acute stroke unit of a tertiary hospital between May 2018 and December 2019 and had changes in neurological symptoms that were detected by stroke nurses. We measured the following outcomes: stroke lesion progression, early neurological deterioration (increase in the total national institutes of health stroke scale score of 2 points or more, increase in the limb weakness score of 1 point or more, or decrease in the alertness score of 1 point or more), and additional clinical management (increasing intravenous fluids, diagnostic imaging, or neuro-intervention). Data was analyzed by logistic regression. ResultsA total of 651 new or aggravating symptoms were detected by nurses. The most detected symptom was motor aggravations (49.2 %). Symptoms were commonly detected during the day shift (51.0 %) and by scheduled neurochecks (71.3 %). Of 132 patients who underwent diagnostic imaging by nurses’ detection, 63.6 % cases had stroke lesion progression. Nursing experience over 4 years was positively associated with finding stroke lesion progression (OR: 2.49, 95 % CI = 1.09–5.67). Early neurological deterioration was found in 70.7 %, and it was significantly higher during scheduled neurochecks (OR:2.65, 95 % CI = 1.04–6.72) and in the group of large artery atherosclerosis (OR: 2.19, 95 % CI = 1.06–4.49) Additional clinical management was provided to 49.9 % of detection, and scheduled neurocheks (OR: 4.76, 95 % CI = 2.18–10.39) and changes of alertness (OR: 2.89, 96 % CI = 1.51–5.26) were the significant factors. ConclusionStroke nurses were able to detect a large number of stroke lesion progression and early neurological deterioration as well as to provide additional clinical management. Systematic guidelines for qualification of stroke nurses may be beneficial.

Ruxolitinib plus standard of care in severe hospitalized adults with severe fever with thrombocytopenia syndrome (SFTS): an exploratory, single-arm trial.

Severe fever with thrombocytopenia syndrome (SFTS) is an emerging tick-borne infectious disease, and its morbidity and mortality are increasing. At present, there is no specific therapy available. An exacerbated IFN-I response and cytokine storm are related to the mortality of patients with SFTS. Ruxolitinib is a Janus kinase (JAK) 1/2 inhibitor that can block proinflammatory cytokines and inhibit the type I IFN pathway. We aimed to explore the use of ruxolitinib plus standard of care for severe SFTS. We conducted a prospective, single-arm study of severe SFTS. We recruited participants aged 18years or older who were admitted to the hospital with laboratory-confirmed severe SFTS and whose clinical score exceeded 8 points within 6days of symptom onset. Participants received oral ruxolitinib (10mg twice a day) for up to 10days. The primary endpoint was 28-day overall survival. The secondary endpoints included the proportion of participants who needed intensive care unit (ICU) admission, total cost, changes in neurologic symptoms and clinical laboratory parameters, and adverse events (AEs) within 28days. A historical control group (HC group, n = 26) who met the upper criteria for inclusion and hospitalized from April 1, 2021, to September 16, 2022, was selected and 1:1 matched for baseline characteristics by propensity score matching. Between Sep 16, 2022, and Sep 16, 2023, 26 participants were recruited into the ruxolitinib treatment group (RUX group). The 28-day overall mortality was 7.7% in the RUX group and 46.2% in the HC group (P = 0.0017). There was a significantly lower proportion of ICU admissions (15.4% vs 65.4%, p < 0.001) and total hospitalization cost in the RUX group. Substantial improvements in neurologic symptoms, platelet counts, hyperferritinemia, and an absolute decrease in the serum SFTS viral load were observed in all surviving participants. Treatment-related adverse events were developed in 6 patients (23.2%) and worsened in 8 patients (30.8%), and no treatment-related serious adverse events were reported. Our findings indicate that ruxolitinib has the potential to increase the likelihood of survival as well as reduce the proportion of ICU hospitalization and being tolerated in severe SFTS. Further trials are needed. ChiCTR2200063759, September 16, 2022.

Super-selective injection of propofol into the intracranial arteries enables Patient's self-evaluation of expected neurological deficit

IntroductionIt is hard to realize the extent of the expected postoperative neurological deficit for patients themselves. The provision of appropriate information can contribute not only to examining surgical indications but also to filling the gap between patient and expert expectations. We hypothesized that propofol infusion into the intracranial arteries (ssWada) could induce focal neurological symptoms with preserved wakefulness, enabling the patients to evaluate the postsurgical risk subjectively. MethodsPresurgical evaluation using ssWada was performed in 28 patients with drug-resistant epilepsy. Based on anatomical knowledge, propofol was super-selectively infused into the intracranial arteries including the M1, M2, and M3 segments of the middle cerebral artery (MCA), A2 segment of the anterior cerebral artery, and P2 segment of the posterior cerebral artery to evaluate the neurological and cognitive symptoms. We retrospectively analyzed a total of 107 infusion trials, including their target vessels, and elicited symptoms of motor weakness, sensory disturbance, language, unilateral hemispatial neglect (UHN), and hemianopsia. We evaluated preserved wakefulness which enabled subjective evaluations of the symptoms and comparison of the subjective experience to the objective findings, besides adverse effects during the procedure. ResultsPreserved wakefulness was found in 97.2% of all trials. Changes in neurological symptoms were positively evaluated for motor weakness in 51.4%, sensory disturbance in 5.6%, language in 48.6%, UHN in 22.4%, and hemianopsia in 32.7%. Six trials elicited seizures. Multivariate analysis showed significant correlations between symptom and infusion site of language and left side, language and MCA branches, motor weakness and A2 or M2 superior division, and hemianopsia and P2. Transient adverse effect was observed in 8 cases with 12 infusion trials (11.2 %). ConclusionThe ssWada could elicit focal neurological symptoms with preserved wakefulness. The methodology enables specific evaluation of risk for cortical resection and subjective evaluation of the expected outcome by the patients.

Clinical course and predictors of outcome following surgical treatment of benign peripheral nerve sheath tumors, a single center retrospective study

Introduction Peripheral nerve sheath tumors are the most common tumor of the peripheral nerves. In general, surgery has a favorable outcome and is the treatment of choice. However, postoperative neurologic deficits are not uncommon, and predictors of outcome are poorly defined. Objective To evaluate clinical outcomes after surgical treatment of benign peripheral nerve sheath tumors and identify outcome predictors that may affect preoperative decision making and improve surgical outcomes. Method In this single center retrospective study, all patients surgically treated for a benign peripheral nerve sheath tumor between 2005 and 2020 were eligible for inclusion. Medical records and imaging data were reviewed. Studied outcomes were changes in neurological symptoms, pain, and tumor recurrence. Logistic regression was performed to identify possible outcome predictors. Results In total, 81 patients undergoing 85 separate surgeries for benign peripheral nerve sheath tumors were included. The most common preoperative symptoms were local pain (90%) followed by a noticeable mass (78%), radiating pain (72%), sensory deficit (18%), and motor deficit (16%). A postoperative improvement of symptoms was seen in 94% of those with pain, 48% of those with sensory deficits and 78% of those with motor deficits. However, 35% and 9% developed new postoperative sensory and motor deficits, respectively. Multivariable analysis showed complete tumor removal as a predictor of reduced pain (p = 0.033), and younger age and larger tumors were risk factors for persistent or increased sensory deficits (p = 0.002 and p = 0.005, respectively). There were no significant predictors of motor deficits. Neurocutaneous syndromes were associated with increased odds of tumor recurrence on univariable analysis (p = 0.008). Conclusion Surgery of benign peripheral nerve sheath tumors is a safe procedure with a favorable outcome in most cases. Younger age and larger tumors were risk factors for persistent or increased sensory deficits, while complete tumor removal was associated with reduced pain. Patients with neurocutaneous syndromes had a higher rate of tumor recurrence. To further evaluate outcome predictors, we recommend future studies to focus on longer follow-up periods to assess the natural course of postoperative neurological deficits.

The safety and feasibility of transcranial direct current stimulation for patients with chronic severe traumatic brain injury in Japan, a phase I study

BackgroundDespite evidence regarding the benefits of transcranial direct current stimulation (tDCS) in patients with severe traumatic brain injury, few trials have examined the treatment safety. Therefore, we created a phase I trial in Japan to investigate the safety of tDCS after severe brain injury. MethodsWe verified the safety of tDCS through constant left frontal anodal stimulation at 2 mA for 20 min for 5 consecutive days. Additionally, we added a variable stimuli period to explore the effects of stimulus intensity, location, and duration. We adapted a traditional 3 + 3 study design. We assessed the rate of adverse effects, including skin burns and clinical seizure. Changes in neurological symptoms were evaluated using the Glasgow Coma Scale, Coma Recovery Scale-Revised, and 4 states of consciousness (coma, unresponsive wakefulness syndrome, minimally conscious state, and severe disability). ResultsSix patients completed the study. No obvious adverse events were observed during the constant stimulation period or the variable stimulation period (right frontal anodal stimulation, stimulation frequency 2–4 mA, 1 or 5 times per week for a period of 4 weeks). Regarding the Coma Recovery Scale-Revised, patients in 4 out of 6 instances (66.7 %) improved by more than 1 point. ConclusionOur phase I safety study indicated that a single course of tDCS was safe in patients with severe traumatic brain injury. The next step is a phase II trial to investigate the preliminary efficacy and safety of tDCS at 2 mA (in comparison with sham stimulation).

NEUROMYELITIS OPTICA. CASE REPORT

Introduction. Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease that causes severe demyelination, especially in the optic nerve and spinal cord with typical clinical manifestations of acute optic neuritis and transverse myelitis. The frequency of occurrence worldwide is estimated at 1-2 people out of every 100,000 people. Case report. Our patient was a 37-year-old woman. She felt a sudden vision loss since May 2022. She related this condition to another case of unknown etiology that she encountered in January of that year - numerous vesicular-type rashes appeared on the abdomen and back and lasted for 3 days, but the traces of those rashes remained for some time in the form of hypopigmentation. In November 2022, she has a sudden vision loss again. "Vitiligo-type" white spots were visible around the eyes and on the hands. In neurological examination: eyeball movements are complete in all directions, photoreaction is preserved (D=S), nose-lip folds are symmetrical (D=S), tongue is on the midline, swallowing is not disturbed. Muscle tone and muscle strength in upper and lower limbs are normal, reflexes have increased (D=S). No changes in gait and sensory disturbances have been observed. The functions of the pelvic organs have not been disturbed. Intellect appears appropriate for age, speech has not changed. Ophthalmologist's examination, both eyes have high degree of myopia, myopic astigmatism. Contrasted Cranial MRI- Angiography revealed increased signal in the intracranial segments of the bilateral optic nerve, chiasm, and bilateral optic tract level. The MRI image was initially evaluated in favor of bilateral optic neuritis. USG of the thyroid gland showed sonographic changes in favor of Hashimoto's thyroiditis, and autoimmune thyroiditis was detected. In chest MRI, a demyelinating lesion of 3 mm at C4 level, a 2 mm broad-based protrusion at C4-5 disc that minimally compresses the spinal cord, an annular swelling at C5-6 disc that compresses the anterior subarachnoid area, Th1 – 6 mm, Th5 – 12 mm, Th9 – 10-38 mm long demyelinating lesions were seen. Discussion. According to the available literature, the detection of AQP-4 protein antibodies in serum, which has a role in the pathogenesis of the disease, can play a decisive role in differential diagnosis. In clinical practice, despite the phenotypic and serological characteristics of this disease, the diagnosis is often difficult. Because there are various autoimmune, infectious and neoplastic etiologies phenotypic, manifestations of diseases can mimic neuromyelitis optica. However, based on the clinical symptoms and the results of instrumental examinations, our patient was diagnosed with neuromyelitis optica. She was treated with hormonal therapy and plasmapheresis, and a significant positive change in neurological symptoms was observed. Conclusion. Despite the difficulties in the differential diagnosis of neuromyelitis optica with other demyelinating diseases, especially with multiple sclerosis, it is possible to correctly assess the diagnostic criteria by understanding the detailed clinical, radiological and prognostic differences.

Status of neurological deficits after mechanical thrombectomy in elderly patients with ischemia

BackgroundNumerous studies report poorer outcomes after mechanical thrombectomy (MT) in elderly patients than in non-elderly patients. We aimed to investigate the changes in neurological symptoms after MT in elderly patients. MethodsWe included 151 patients with acute stroke who independently performed activities of daily living before admission, underwent MT at our hospital between January 1, 2019 and December 31, 2021, and had successful recanalization (thrombolysis in cerebral infarction grade >2b). The subjects were divided into two groups according to whether they were aged ≥80 years (elderly group) or <80 years (non-elderly group), and the National Institutes of Health Stroke Scale (NIHSS) scores and deep and subcortical white matter hyperintensity (DSWMH) grades were compared between both groups. ResultsThere were 57 (38 %) and 94 (62 %) patients in the elderly and non-elderly groups, respectively. After propensity score matching, the median (interquartile range) NIHSS scores were significantly higher in the elderly group than in the non-elderly group at both 24 h (12 [5.5–19] vs 8; [2–14] P = 0.040) and 1 week (8 [3–12] vs 2.5 [0–13.5]; P = 0.041) after MT but not before MT (18 [13.5–22.5] vs 20 [14.5–24]; P = 0.549). DSWMH grades were significantly higher in the elderly group than in the non-elderly group (2 [1–3] vs 1 [0–2]; P = 0.018). ConclusionsMT in the elderly group was associated with poorer improvement in neurological symptoms from the early period after MT than in the non-elderly group, even when successful recanalization was achieved. White matter lesions may contribute to the poor neurological improvement after recanalization in the elderly group.

Changes in cerebral blood flow in the postoperative chronic phase after combined cerebral revascularization for moyamoya disease with ischaemic onset.

The purpose of this study was to examine the effects of combined revascularization for ischaemic-onset moyamoya disease (MMD) on cerebral haemodynamics by comparing cerebral blood flow (CBF) during the postoperative chronic phase with preoperative CBF. A retrospective cohort of 24 MMD patients (representing 31 surgeries) who received single photon emission computed tomography (SPECT) before and more than 6months after surgery was investigated. The CBF value of each vascular territory was extracted from SPECT data, and the value relative to the ipsilateral cerebellar value (relative CBF, or RCBF) was calculated. The correlation between the revascularization effect and the proportional change in RCBF before and after surgery (calculated as post-RCBF/pre-RCBF ("post/pre-RCBF")) was analysed. Furthermore, the relationships between changes in neurological symptoms and post/pre-RCBF were investigated. Preoperative and postoperative mean RCBF values were 0.92 ± 0.15 and 0.96 ± 0.13 (p = 0.619) in the anterior cerebral artery territory, 0.99 ± 0.17 and 1.01 ± 0.17 (p = 0.598) in the middle cerebral artery territory and 1.15 ± 0.22 and 1.14 ± 0.19 (p = 0.062) in the posterior cerebral artery territory, respectively. No significant correlation was found between the revascularization score and post/pre-RCBF. The revascularization score and post/pre-RCBF were not significant predictors of worsening neurological symptoms postoperatively. No significant change in RCBF was observed in any vascular territory in the chronic phase after revascularization. Combined revascularization may assist in the redirection of blood flow from the internal to the external carotid system and contribute to CBF maintenance.

Acute Ischemic Stroke in the Postpartum Period

During pregnancy and delivery, the risk of cerebrovascular disorders increases due to several physiological changes. The elevated level of estrogen stimulates the production of clotting factors, which deteriorates the risk of thromboembolism. Increased plasma volume and total blood volume lead to the risk of hypertension, and the high level of progesterone in the third trimester of pregnancy promotes the permeability of small blood vessels. The risk of developing cerebrovascular complications remains high in the postpartum period. The article describes two clinical cases of postpartum ischemic stroke as a complication after surgical delivery in young women with an aggravated somatic medical history. The treatment and follow-up of these patients during their stay in the intensive care unit included changes in management tactics, use of antiplatelet, anticoagulant, and metabolic therapy, and subsequent rehabilitation. This article describes the case-management interventions and changes in neurological symptoms of these patients, also it discusses the most likely etiological factors of cerebrovascular disorders in these cases

Clinical application of cervical pedicle screw implantation technique under regional method

To investigate the effectiveness of cervical pedicle screw implantation technique under regional method. The clinical data of 85 patients who met the selection criteria between April 2010 and May 2018 were retrospectively analyzed. There were 57 males and 28 females, aged 35-68 years, with an average of 57.6 years. Among them, there were 10 cases of ossification of posterior longitudinal ligament, 68 cases of cervical spondylosis with multilevel stenosis, 3 cases of cervical tumor, 1 case of congenital malformation, and 3 cases of cervical trauma; the lower cervical spine lesions involved C 3-C 7. Preoperative Frankel spinal cord injury grading: 2 cases of grade C, 51 cases of grade D, and 32 cases of grade E. Cervical pedicle screw implantation technique under regional method was performed with a total of 618 pedicle screws. Postoperative changes in neurological symptoms were observed; cervical mouth opening anteroposterior and lateral X-ray films and cervical CT examinations were performed to evaluate the pedicle screws position. The operation time was 2.5-4.0 hours, with an average of 3.0 hours. The intraoperative blood loss was 180-550 mL, with an average of 345 mL. No intraoperative vascular or nerve injury occurred. The patients with neurological symptoms were relieved to varying degrees. There were 2 cases of superficial incision infection after operation, the wound healed after enhanced dressing change. The postoperative hospital stay was 5-14 days, with an average of 8.4 days. At discharge, Frankel neurological grading was grade D in 26 patients and grade E in 59 patients. All the patients were followed up 6-24 months, with an average of 13 months. At last follow-up, cervical X-ray films showed the good pedicle screw fixation without loosening. Cervical CT evaluated the position of pedicle screws: 523 pedicle screws (84.7%) in grade Ⅰ, 80 (12.9%) in grade Ⅱ, and 15 (2.4%) in grade Ⅲ; the accuracy rate of the screw position was 97.6%. Cervical pedicle screw implantation technique under regional method can significantly improve the success rate of screw implantation. It is easy to operate, does not destroy the bone cortex, and has stable fixation.

Secondary spondylogenic epidural abscess

The article presents the results of a retrospective analysis of 200 patients with hematogenous osteomyelitis of the spine who were treated in the Tyumen Regional Clinical Hospital #2. Neurological disorders were present in 37 patients (group I); 163 patients (group II) had no neurological disorders; according to CT and MRI, secondary spondylogenic epidural abscess was diagnosed in 24 patients. At admission, the severity of neurological deficit assessed using the Frankel scale was as follows: Grade A - 6 cases, Grade B - 7 cases, Grade C - 17 cases, Grade D - 5 cases, and Grade E - 2 cases (epidural abscess without neurological deficit). At discharge, the severity of neurological deficit was as follows: Grade A - 3 cases, Grade B - 2 cases, Grade C - 13 cases, Grade D - 11 cases, and Grade E - 8 cases. In group I, 35 out of 37 patients underwent surgery; of these, 21 patients had improvements. There were no statistically significant differences in neurological deficit changes between patients with and without epidural abscess. In 12 patients, the neurological status remained unchanged. Two patients died. We present an analysis of the severity of neurological symptoms, depending on the spinal lesion level, and the results of microbiological tests. Changes in neurological symptoms in lesions of various spinal parts are described. Cervical spine lesions and the process caused by S. aureus are shown to significantly increase the risk of neurological disorders. An active surgical approach for complicated forms of spinal osteomyelitis provided partial or complete regression of neurological disorders in 62.2% of cases.

A case of thoracic disc herniation characterized by marked posture-related dynamic changes in neurological symptoms

Abstract Thoracic disc herniation is less common than lumbar and cervical disc herniations. It is usually accompanied by severe myelopathy, which often leads to surgery. Because the thoracic spine is less mobile, thoracic disc herniation is considered to be minimally affected by dynamic spine factors in cases with myelopathy. We experienced a case of thoracic disc herniation (T4/5 and T6/7) characterized by posture-related dynamic changes in neurological symptoms; that is, numbness extending from the trunk to the entire lower limbs was deteriorated in the standing and sitting positions, was relieved in the supine position, and disappeared in the prone position. In addition, the patient reported dysuria with a delay when attempting to urinate in the standing position. Computed tomographic myelography revealed diffuse idiopathic skeletal hyperostosis extending from T3 to T11, and the kyphosis angles at T1 to T11 levels were 68 degrees in the half-sitting position and 58 degrees in the prone position, showing posture-related changes. The patient underwent the posterior fusion in the prone position, by which symptoms disappeared, without undergoing disc herniotomy or laminectomy, and favorable outcomes were achieved. Thoracic disc herniation with marked posture-related neurological symptoms is extremely rare. Here we report a case presentation and literature review of pathophysiology observed in our patient.

Treatment of a glioblastoma multiforme dural metastasis with stereotactic radiosurgery: A case report and select review of the literature

Glioblastoma multiforme (GBM) is a primary brain neoplasm accounting for approximately 75% of all high grade gliomas. It is diffusely infiltrative and exhibits rapid proliferation with a poor overall prognosis. Maximum surgical resection and postoperative radiotherapy, accompanied by concurrent and adjuvant temozolomide chemotherapy, remain the standard of care without major therapeutic advances over the past 10 years. Herein, we present the case of a 64-year-old Caucasian male with a GBM who subsequently developed a left frontal dural metastasis, subsequently treated with stereotactic radiosurgery (20 Gy in 1 fraction). With six month follow-up, the patient showed near complete resolution of his dural metastases and no overall change in neurological symptoms or side effects following radiosurgery. Due to the paucity of clinical literature regarding dural metastases from GBM, its optimal treatment remains unknown. While the role of SRS has yet to be defined in this setting, here we provide evidence suggesting its overall efficacy in the treatment of select dural GBM metastases.

Repeat head imaging in blunt pediatric trauma patients: Is it necessary?

Children with confirmed brain injury usually undergo follow-up computed tomography (CT) scan of the head within 24 hours of admission. To date, no evidence exists to validate the diagnostic or therapeutic value of these repeat CTs. The purpose of this study was to (1) evaluate progression of traumatic brain injuries, (2) determine if routine repeat imaging changes management, and (3) compare the efficacy of recognizing worsening hemorrhage with serial neurological examination versus repeat imaging. A 5-year retrospective review was conducted of all patients aged under 18 years with blunt traumatic head injury (n = 95). Data included demographics, type and size of intracranial hemorrhage, exam findings, diagnostic and management changes, and hospital outcomes. Most patients (68.4%) had at least one repeat CT; of these, 67.7% (n = 44) showed no change or reduced hemorrhage. In only one patient did a repeat CT scan result in a surgical procedure; however, that CT scan was prompted by a change in neurological status. Among patients with more than two repeat head CTs, 42.9% led to a change in management, most frequently an additional CT scan. Presence of neurological symptoms was associated with having repeat CT scans (p = 0.025). Changes in Glasgow Coma Scale score were associated with increased hemorrhage (p = 0.012) but not repeat scans (p = 0.496). In the majority of cases, increased hemorrhage only resulted in an additional head CT and prolonged intensive care unit stay. Excluding patients who arrived with brain death, there was no difference in mortality between patients with and without repeat imaging. Findings from this study support a selective approach for repeating head CTs with emphasis on changes in neurological symptoms and Glasgow Coma Scale score. Prospective studies on timing and indications for repeat CT scans are needed to support development of clinical guidelines. Therapeutic study, level III.

Long-term treatment with thiamine as possible medical therapy for Friedreich ataxia.

Thiamine (vitamin B1) is a cofactor of fundamental enzymes of cell energetic metabolism; its deficiency causes disorders affecting both the peripheral and central nervous system. Previous studies reported low thiamine levels in cerebrospinal fluid and pyruvate dehydrogenase dysfunction in Friedreich ataxia (FRDA). We investigated the effect of long-term treatment with thiamine in FRDA, evaluating changes in neurological symptoms, echocardiographic parameters, and plasma FXN mRNA levels. Thirty-four consecutive FRDA patients have been continuously treated with intramuscular thiamine 100mg twice a week and have been assessed with the Scale for the Assessment and Rating of Ataxia (SARA) at baseline, after 1month, and then every 3months during treatment. Thiamine administration ranged from 80 to 930days and was effective in improving total SARA scores from 26.6±7.7 to 21.5±6.2 (p<0.02). Moreover, deep tendon reflexes reappeared in 57% of patients with areflexia at baseline, and swallowing improved in 63% of dysphagic patients. Clinical improvement was stable in all patients, who did not show worsening even after 2years of treatment. In a subgroup of 13 patients who performed echocardiogram before and during treatment, interventricular septum thickness reduced significantly (p<0.02). Frataxin mRNA blood levels were modestly increased in one-half of treated patients. We suppose that a focal thiamine deficiency may contribute to a selective neuronal damage in the areas involved in FRDA. Further studies are mandatory to evaluate thiamine role on FXN regulation, to exclude placebo effect, to verify our clinical results, and to confirm restorative and neuroprotective action of thiamine in FRDA.

Emerging therapies in Friedreich's ataxia.

Friedreich's ataxia (FRDA) is an inherited, progressive neurodegenerative disease that typically affects teenagers and young adults. Therapeutic strategies and disease insight have expanded rapidly over recent years, leading to hope for the FRDA population. There is currently no US FDA-approved treatment for FRDA, but advances in research of its pathogenesis have led to clinical trials of potential treatments. This article reviews emerging therapies and discusses future perspectives, including the need for more precise measures for detecting changes in neurologic symptoms as well as a disease-modifying agent.

The prognostic value of tumor necrosis in patients undergoing stereotactic radiosurgery of brain metastases

BackgroundThis retrospective study investigated the outcome of patients with brain metastases after radiosurgery with special emphasis on prognostic impact of visible intratumoral necrosis on survival and local control.MethodsFrom 1998 through 2008, 149 patients with brain metastases from solid tumors were treated with stereotactic radiotherapy at Luebeck University. Median age was 58.4 years with 11%, 78%, 10% in recursive partitioning analysis (RPA) classes I, II, III, respectively. 70% had 1 metastasis, 29% 2-3 metastases, 2 patients more than 3 metastases, 71% active extracranial disease. Median volume of metastatic lesions was 4.7 cm3, median radiosurgery dose 22 Gy (single fraction). 71% of patients received additional whole-brain irradiation (WBI). All patients were analyzed regarding survival, local, distant failure and prognostic factors, side effects and changes in neurologic symptoms after radiotherapy. The type of contrast-enhancement in MR imaging was also analyzed; metastatic lesions were classified as containing necrosis if they appeared as ring-enhancing with central areas of no or minimal contrast enhancement.ResultsMedian survival was 7.0 months with 1-year and 5-year survival rates of 33% and 0.4%, respectively. Tumor necrosis (ring-enhancement) was visible on pretreatment MRI scans in 56% of all lesions and lesions with necrosis were larger than non-necrotic lesions (6.7 cm3 vs. 3.2 cm3, p = 0.01). Patients with tumor necrosis had a median survival of 5.4 months, patients without tumor necrosis 7.2 months. Local control rate in the irradiated volume was 70%, median survival without local failure 17.8 months. Control in the brain outside the irradiated volume was 60%, median survival without distant failure 14.0 months. Significant prognostic factors for overall survival were KPS (p = 0.001), presence of tumor necrosis on pretreatment MRI (p = 0.001) with RPA-class and WBI reaching marginal significance (each p = 0.05). Prognostic impact of tumor necrosis remained significant if only smaller tumors with a volume below 3.5 cm3 (p = 0.03). Side effects were rare, only one patient suffered from serious acute side effects.ConclusionsResults of this retrospective study support that stereotactic radiotherapy is an effective treatment option for patients with metastatic brain lesions. The prognostic impact of visible tumor necrosis (ring-enhancement) on pretreatment MRI scans should be further investigated.

Effect of Radical Scavenger N-Tert-Butyl-α-Phenylnitrone on Stroke in a Rat Model Using a Telemetric System

We used malignant stroke-prone spontaneously hypertensive rats (M-SHRSP) as a stroke model to explore the effects of the radical scavenger N-tert-butyl-alpha-phenylnitrone (PBN) on stroke. PBN was administrated in drinking water to M-SHRSP. Circadian rhythms in heart rate, blood pressure, and locomotive activity in M-SHRSP were monitored with a telemetric system, in addition to measurement of water intake and body weight. Stroke-onset was assessed by changes in neurological symptoms, water intake, and body weight. Circadian rhythms were basically the same between PBN-treated and control rats several days after stroke onset. Significant differences were seen in blood pressure, relative weight of brain and water intake, heart rate, and locomotive activity between two groups. As a result, no significant difference in age of stroke onset was seen between PBN-treated and control rats, but PBN-treated rats displayed prolonged mean life spans. PBN might be effective in prolonging life span.

The Severity of Minamata Disease Declined in 25 Years: Temporal Profile of the Neurological Findings Analyzed by Multiple Logistic Regression Model

Minamata disease (MD) was caused by ingestion of seafood from the methylmercury-contaminated areas. Although 50 years have passed since the discovery of MD, there have been only a few studies on the temporal profile of neurological findings in certified MD patients. Thus, we evaluated changes in neurological symptoms and signs of MD using discriminants by multiple logistic regression analysis. The severity of predictive index declined in 25 years in most of the patients. Only a few patients showed aggravation of neurological findings, which was due to complications such as spino-cerebellar degeneration. Patients with chronic MD aged over 45 years had several concomitant diseases so that their clinical pictures were complicated. It was difficult to differentiate chronic MD using statistically established discriminants based on sensory disturbance alone. In conclusion, the severity of MD declined in 25 years along with the modification by age-related concomitant disorders.

Wilson's disease and hepatic transplantation.

To investigate the changes in neurological symptoms and signs, as well as serum copper, serum ceruloplasmin after hepatic transplantation in patients with Wilson's disease, neurological symptoms and signs, serum copper, serum ceruloplasmin before and after hepatic transplantation in 18 patients with Wilson's disease were observed, and those changes were followed up in 20 non-operative controls treated with penicillamine. Our results showed that the neurological symptoms and signs, serum copper and serum ceruloplasmin were improved in the operative group but deteriorated in the non-operative control group. Our study showed that hepatic transplantation is better than penicillamine in the treatment of Wilson's disease.