Residual Neurological Dysfunction Research Articles

Acute necrotizing encephalopathy infected with the SARS-CoV-2 in children: Case series and literature review of clinical outcomes with the use of Tocilizumab

Background and objectiveSevere acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes acute necrotizing encephalopathy (ANE), which has a high mortality rate and severe sequelae. This study aimed to identify ANE early and evaluate the usefulness of tocilizumab in ANE treatment. MethodsWe retrospectively included eight paeefediatric ANE cases infected with SARS-CoV-2 at Xi'an Children's Hospital, China, from December 1, 2022 to May 1, 2023. A literature search was performed using the PUBMED, SPRING, SCOPUS, and EMBASE databases. This study included eleven patients. Clinical characteristics, laboratory test results, imaging features, and treatment options were analysed. ResultsEight of the 19 cases (42 %) died, one (5 %) recovered, and nine (47 %) improved with residual neurological dysfunction. Eighteen patients presented with fever, with 56 % having ≥40 °C. Twelve patients (63 %) presented with dysfunction consciousness. Eight (42 %) patients experienced frequent convulsions. All eight patients in our hospital had elevated procalcitonin levels (mean: 21.32 ng/mL, range: 0.10–89.40 ng/mL). Alanine aminotransferase levels were elevated (mean: 632.81 U/L, range: 13.00–2251.00 U/L) in six patients. Seven patients showed elevated uric acid levels(mean: 396.50 μmol/L, range: 157.00–660.00 μmol/L). Brain imaging indicated that all the patients had symmetrical injuries to the bilateral thalami, accompanied by symmetrical injuries in the cerebrum, cerebellum, basal ganglia, and brain stem. Compared with the classical treatment (n = 9), the combination with tocilizumab (n = 6) showed a statistically difference in mortality (p = 0.028 < 0.05). ConclusionThe typical clinical manifestations of ANE in children with SARS-CoV-2 infection are acute onset with high fever, frequent convulsions and rapidly worsening disturbance of consciousness. Tocilizumab treatment could reduces mortality in ANE.

Central nervous system complications in SARS-CoV-2-infected patients

ObjectiveTo investigate the clinical manifestations, treatment and prognosis of COVID-19-associated central nervous system (CNS) complications.MethodsIn this single-centre observation study, we recruited patients with COVID-19-associated CNS complications at the neurology inpatient department of the Eighth Affiliated Hospital, Sun Yat-Sen University (Futian, Shenzhen) from Dec 2022 to Feb 2023. Patients were analysed for demographics, clinical manifestations, cerebrospinal fluid properties, electroencephalographic features, neuroimaging characteristics, and treatment outcome. All patients were followed-up at 1 and 2 months after discharge until Apr 2023.ResultsOf the 12 patients with COVID-19-associated CNS complications, the CNS symptoms occur between 0 days and 4 weeks after SARS-CoV-2 infection. The most common CNS symptoms were memory deficits (4/12, 33%), Unresponsiveness (4/12, 33%), mental and behavioural disorders (4/12, 33%). Seven of 12 cases can be categorized as probable SARS-CoV-2 encephalitis, and 5 cases can be described as brainstem encephalitis, acute disseminated encephalomyelitis, optic neuritis, multiple sclerosis or tremor probably associated with SARS-CoV-2 infection. Six patients received antiviral therapy, and 11 patients received glucocorticoid therapy, of which 3 patients received human immunoglobulin synchronously. Nine patients recovered well, two patients had residual neurological dysfunction, and one patient passed away from complications associated with tumor.ConclusionIn this observational study, we found that the inflammatory or immune-related complications were relatively common manifestations of COVID-19-associated CNS complications, including different phenotypes of encephalitis and CNS inflammatory demyelinating diseases. Most patients recovered well, but a few patients had significant neurological dysfunctions remaining.

Two rare cases of myelin oligodendrocyte glycoprotein antibody-associated disorder in children with leukodystrophy-like imaging findings

BackgroundChildren with acquired demyelinating syndromes (ADS) whose sera are positive for myelin oligodendrocyte glycoprotein (MOG) immunoglobulin (IgG) can be diagnosed with MOG-IgG associated disorder (MOGAD). Cases with leukodystrophy-like imaging findings with recurrent MOGAD have rarely been reported.Case presentationTwo children with MOGAD, whose onset age was 6 months and 3 years, respectively, were admitted to the hospital due to fever and altered consciousness. In both children, MOG-IgG was detected in the serum using live cell-based assay. Brain magnetic resonance imaging (MRI) revealed leukodystrophy-like lesions with diffuse bilateral white matter. Cerebrospinal fluid (CSF) analysis showed mild pleocytosis with normal or slightly increased protein levels and no oligoclonal bands. Metabolic and inflammatory blood/CSF markers were all negative. Full exon gene testing revealed normal results, and nuclear and mitochondrial DNA were normal. Despite regular immunotherapy and reduction of lesions based on brain MRI results, the patients repeatedly relapsed and had residual neurological dysfunction at 3–4 years of follow-up.ConclusionsAlthough MOGAD is a monophasic and benign condition, certain MOGAD patients can experience multiple relapses and residual neurologic deficits. The spectrum of clinical manifestations in MOGAD is wider in children than in previously reported cases, including cases with leukodystrophy-like imaging findings. Such imaging findings along with MOG-IgG may occur recurrently and result in severe neurological prognosis. Patients with extensive and confluent white matter lesions should undergo early testing of MOG-IgG to ensure early therapy. In refractory cases, MOGAD treatment may need to be escalated beyond the current therapy, which means second-line immunotherapy should be performed as early as possible and hormone levels should not be rapidly reduced. Early diagnosis and appropriate treatment may improve the prognosis of children with MOGAD.

The efficacy of haemoperfusion combined with continuous venovenous haemodiafiltration in the treatment of severe viral encephalitis in children

BackgroundThis study investigated the efficacy of the integrated blood purification mode of early haemoperfusion (HP) combined with continuous venovenous haemodiafiltration (CVVHDF) in children with severe viral encephalitis, and evaluated the correlation of cerebrospinal fluid (CSF) neopterin (NPT) levels with prognosis.MethodsThe records of children with viral encephalitis who received blood purification treatment in the authors’ hospital from September 2019 to February 2022 were retrospectively analysed. According to the blood purification treatment mode, they were divided into the experimental group (HP + CVVHDF, 18 cases), control group A (CVVHDF only, 14 cases), and control group B (16 children with mild viral encephalitis who did not receive blood purification treatment). The correlation between the clinical features, severity of the disease and the extent of lesions on brain magnetic resonance imaging (MRI) and the CSF NPT levels was analysed.ResultsThe experimental group and control group A were comparable with respect to age, gender and hospital course (P > 0.05). There was no significant difference in speech and swallowing functions between the two groups after treatment (P > 0.05) and no significant difference in 7 and 14-day mortality (P > 0.05). The CSF NPT levels in the experimental group before treatment were significantly higher compared with control group B (P < 0.05). The extent of brain MRI lesions correlated positively with CSF NPT levels (P < 0.05). In the experimental group (14 cases), the serum NPT levels decreased after treatment, whereas the CSF NPT levels increased after treatment, and the differences were statistically significant (P < 0.05). Dysphagia and motor dysfunction correlated positively with CSF NPT levels (P < 0.05).ConclusionEarly HP combined with CVVHDF in the treatment of severe viral encephalitis in children may be a better approach than CVVHDF only for improving prognosis. Higher CSF NPT levels indicated the likelihood of a more severe brain injury and a greater possibility of residual neurological dysfunction.

Pathophysiological mechanisms of chronic compressive spinal cord injury due to vascular events.

Cervical spondylotic myelopathy is the main cause of non-traumatic spinal cord injury, with chronic static and/or dynamic compressive spinal cord injury as the unique pathogenesis. In the progression of this condition, the microvascular network is compressed and destroyed, resulting in ischemia and hypoxia. The main pathological changes are inflammation, damage to the blood spinal cord barriers, and cell apoptosis at the site of compression. Studies have confirmed that vascular regeneration and remodeling contribute to neural repair by promoting blood flow and the reconstruction of effective circulation to meet the nutrient and oxygen requirements for nerve repair. Surgical decompression is the most effective clinical treatment for this condition; however, in some patients, residual neurological dysfunction remains after decompression. Facilitating revascularization during compression and after decompression is therefore complementary to surgical treatment. In this review, we summarize the progress in research on chronic compressive spinal cord injury, covering both physiological and pathological changes after compression and decompression, and the regulatory mechanisms of vascular injury and repair.

Cyclophosphamide‐induced posterior reversible encephalopathy syndrome: A case report

AbstractPosterior reversible encephalopathy syndrome (PRES) is an acute neurological disorder of vascular origin characterized by headache, seizures, encephalopathy, visual disturbances, and focal neurological deficits. Characteristic clinical findings along with T2 or fluid‐attenuated inversion recovery (FLAIR) hyperintensities on magnetic resonance imaging (MRI) in the bilateral parieto‐occipital region help confirm the diagnosis. In addition to common risk factors such as eclampsia, preeclampsia, renal failure, sepsis, and autoimmune diseases, PRES has also been reported in patients receiving chemotherapeutic drugs. We report a case of a 65‐year‐old woman with multiple myeloma who developed PRES following the administration of cyclophosphamide. Although our patients had multiple risk factors for PRES, such as renal insufficiency, bortezomib, cyclophosphamide, and steroids, a comprehensive clinical evaluation pointed toward cyclophosphamide as the cause of PRES. Active treatment to lower blood pressure, the use of antiepileptic drugs, and the withdrawal of cyclophosphamide led to rapid and complete recovery with no residual neurological dysfunction.

Medium- and long-term effects of endovascular treatments for severely stenotic basilar arteries supported by multimodal imaging

BackgroundTo evaluate the medium-and long-term effect of intravascular interventional therapy for symptomatic severe basilar artery stenosis supported by multimodal imaging.MethodAfter strict screening of 67 patients with symptomatic severe basilar artery stenosis (70–99%) with atherosclerotic stenosis, 67 patients with symptomatic recurrence after intensive drug treatment were treated with intravascular balloon dilatation and Enterprise stent implantation. Any stroke or death within 30 days after operation and any stroke and restenosis during medium-and long-term follow-up were recorded.Results①The mean age of 67 patients (67lesions) was 57 ± 8 years old, and the technical success rate was 100%; ②Preoperative angiography showed that the collateral circulation was poor, and TICI was 1-2a while postoperative angiography showed that TICI was significantly improved to 2b-3; ③The average preoperative stenosis rate was 82 ± 9%, and the postoperative stenosis rate was reduced to 17 ± 10%; ④Before surgery, abnormal perfusion was found in the posterior circulation CTP; After the postoperative re-examination, the posterior circulation of CTP perfusion was significantly improved; ⑤Postoperative symptoms and neurological conditions improved significantly; ⑥Complications of perforating branch event occurred in 1 case after operation, and symptoms were relieved after more than 1 month of medication treatment, and mild neurological dysfunction remained. 1 case developed subacute thrombosis in the stent, which improved after active intra-arterial thrombolysis, and there was no residual neurological dysfunction; and 1 case of micro-guide wire being trapped by the distal vasospasm. ⑦67 patients were followed up by telephone, WeChat or imaging for 36–66 months.ConclusionsIn summary intravascular balloon dilation + Enterprise stent implantation is safe and effective for the treatment of symptomatic severe atherosclerotic stenosis of the basilar artery, with high technical success rate, low perioperative complications, and good mid-term and long-term effects.

Spinal Epidural Abscess: Diagnosis, Management, and Outcomes.

An infection of the spinal epidural space, spinal epidural abscess (SEA) is a potentially devastating entity that is rising in incidence. Its insidious presentation, variable progression, and potential for precipitous neurologic decline make diagnosis and management of SEA challenging. Prompt diagnosis is key because treatment delay can lead to paralysis or death. Owing to the nonspecific symptoms and signs of SEA, misdiagnosis is alarmingly common. Risk factor assessment to determine the need for definitive MRI reduces diagnostic delays compared with relying on clinical or laboratory findings alone. Although decompression has long been considered the benchmark for SEA, considerable risk associated with spinal surgery is noted in an older cohort with multiple comorbidities. Nonoperative management may represent an alternative in select cases. Failure of nonoperative management is a feared outcome associated with motor deterioration and poor clinical outcomes. Recent studies have identified independent predictors of failure and residual neurologic dysfunction, recurrence, and mortality. Importantly, these studies provide tools that generate probabilities of these outcomes. Future directions of investigation should include external validation of existing algorithms through multi-institutional collaboration, prospective trials, and incorporation of powerful predictive statistics such as machine learning methods.

Application value of preoperative high-resolution magnetic resonance vessel wall imaging evaluation on symptomatic middle cerebral artery stenosis stenting

Objective To investigate the value of preoperative high-resolution magnetic resonance vessel wall imaging (HRMR-VWI) in stenosis stenting for symptomatic middle cerebral artery. Methods The clinical data of 35 patients with atherosclerotic stenosis in middle cerebral artery (MCA) M1 segment who were treated with stenting therapy (stenting angioplasty) at Department of Neurosurgery, Tianjin Huanhu Hospital from August 2011 to May 2019 were analyzed retrospectively. HRMR-VWI results were used to evaluate the location, composition and stability of plaques before operation. The mortality and morbidity at 30 days post operation were determined by clinical follow-up. The ischemic stroke and restenosis rates at 12 months post operation were confirmed by imaging follow-up. Results The preoperative HRMR-VWI of 35 patients showed that the plaques in 15 cases were located on the ventral vessel wall, those in 12 on the inferior, and those in 8 on the upper vessel wall. Seven patients had unstable plaques in the M1 segment of MCA. The stents were successfully placed in 35 patients and well adhered with smooth blood flow. Technical complications occurred in 2 (5.7%) patients. One patient (2.8%) died of cerebral hernia due to reperfusion hemorrhage in 30 days after surgery, although decompressive craniectomy and hematoma clearing were performed. One patient (2.8%) had residual neurological dysfunction. Twelve months after the operation, no ischemic stroke occurred in 34 patients, and no restenosis occurred in the 5 patients who were followed up by imaging. Conclusion HRMR-VWI has a high diagnostic value for the location and stability of plaques in stenotic sites of MCA, which could improve the safety and effectiveness of stenting of MCA M1 segment stenosis. Key words: Middle cerebral artery; Endovascular procedures; Stents; Atherosclerotic stenosis; High resolution magnetic resonance vessel wall imaging

Clinical analysis of relapsed demyelinating disease associated with myelin oligodendrocyte glycoprotein antibody in children

Objective To investigate the clinical characteristics, treatment and prognosis of relapsed demyeli-nating disease (RDD) associated with myelin oligodendrocyte glycoprotein antibodies (MOG abs) children in southern China. Methods Children with RDD associated with MOG abs at Department of Neurology in Guangzhou Women and Children′s Medical Center from January 2015 to December 2018 were retrospectively analyzed.The annualized relapse rates (ARRs) and expand disability status scale (EDSS) were used to assess the recurrence frequency and neurological dysfunction respectively. Results Ten children were included with the age of (6.4±3.6) years old, and male to female ratio was 4∶6.(1)Clinical phenotype: all children had 24 episodes during follow-up, with acute disseminated encephalomyelitis (ADEM)(7/10 cases) and neuromyelitis optica spectrum disorders (NMOSD)(3/10 cases) on the first episode.Among 14 recurrent episodes, ADEM (9/14 times) was the most common, followed by optic neuritis(ON)(3/14 times)and brainstem encephalitis (2/14 times). By the final follow-up, the final diagnosis was multiphasic disseminated encephalomyelitis(MDEM)(6/10 cases), NMOSD(3/10 cases), ADEM-ON(1/10 case), respectively.(2)Laboratory examination: all the children had positive serum MOG abs in the acute stage.The serum MOG abs titer high group(≥1∶640)(6 cases)on the first episode complicated ON (3 cases) and long segment myelitis (3 cases) more common than those of low group(1∶320)(4 cases). (3)Imaging changes: 25 times of bain magnetic resonance imaging (MRI) were performed in the acute stage, MRI changes mostly involved the cortex and subcortical white matter.Four cases had abnormal spinal cord MRI.(4)Treatment and prognosis: intravenous methylprednone (IVMP) combined with intravenous immunoglobulin (IVIG) were administrated in acute stage.Rituximab (2/10 cases), mycophenolate mofetil (4/10 cases), IVIG (2/10 cases) monthly and low dose prednisone orally (2/10 cases) were given respectively in maintains stage.ARRs decreased from 1.4 to 0 and EDSS score improved significantly after these treatments above.Seven cases had residual neurological dysfunction with 3 cases of NMOSD, 3 cases of MDEM and 1 case of ADEM-ON, including motor dysfunction, learning disability and inattention, symptomatic epilepsy and visual impairment. Conclusions ADEM is the most common form of RDD associated with MOG abs in children.Those with high serum MOG abs titer on the first episode are prone to have ON or long segment myelitis.Immunomodification therapy is effective in the relapsed patients, residual neurological sequelae were related to the type of repeated demyelination. Key words: Myelin oligodendrocyte glycoprotein; Relapsed demyelinating disease; Child

Chapter 17 - Acute and chronic viral infections

A large number of viruses belonging to various families are able to cause central nervous system (CNS) infections and contribute significantly to burden of disease in humans globally. Most viral CNS infections are benign and self-limiting, and most remain undiagnosed. However, some viruses can cause severe inflammation, leading to morbidity and mortality, and result in severe long-term residual damage and neurologic dysfunction in patients. The potential of viruses to cause CNS inflammation greatly varies depending on host factors, such as age, sex, and genetic background, as well as viral factors. Despite the need for protection against viral invasion and replication, the extent of the immune response in the CNS is carefully regulated to prevent excessive inflammation and tissue destruction leading to irretrievable loss of neurons. Direct cytopathology is for many virus infections a major cause of neurologic symptoms; however, the antiviral immune response can in some instances contribute substantially to pathology. This chapter highlights a selection of clinically important neurotropic viruses that infect the CNS and cause neurologic diseases such as meningitis, encephalitis, and myelitis in humans, with a focus on neuropathologic findings.

Prediction of 3- to 5-Month Outcomes from Signs of Acute Bilirubin Toxicity in Newborn Infants

To evaluate the ability of the bilirubin-induced neurologic dysfunction (BIND) score to predict residual neurologic and auditory disability and to document the relationship of BIND score to total serum bilirubin (TSB) concentration. The BIND score (assessing mental status, muscle tone, and cry patterns) was obtained serially at 6- to 8-hour intervals in 220 near-term and full-term infants with severe hyperbilirubinemia. Neurologic and/or auditory outcomes at 3-5 months of age were correlated with the highest calculated BIND score. The BIND score was also correlated with TSB. Follow-up neurologic and auditory examinations were performed for 145/202 (72%) surviving infants. All infants with severe acute bilirubin encephalopathy (BIND scores 7-9) either died or suffered residual neurologic and auditory impairment. Of 24 cases with moderate encephalopathy (BIND 4-6), 15 (62.5%) resolved following aggressive intervention and were normal at follow-up. Three of 73 infants with mild encephalopathy (BIND scores 1-3) but severe jaundice (TSB ranging 33.5-38 mg/dL; 573-650 µmol/L) had residual neurologic and/or auditory impairment. A BIND score ≥4 had a specificity of 87.3% and a sensitivity of 97.4% for predicting poor neurologic outcomes (receiver operating characteristic analysis). BIND scores trended higher with severe hyperbilirubinemia (r2 = 0.54, P < .005), but 5/39 (13%) infants with TSB ≥36.5 mg/dL (624 µmol/L) had BIND scores ≤3, and normal outcomes at 3-5 months. The BIND score can be used to evaluate the severity of acute bilirubin encephalopathy and predict residual neurologic and hearing dysfunction.

Posterior Reversible Encephalopathy Syndrome in Two Omani Children with Underlying Renal Diseases.

Posterior reversible encephalopathy syndrome (PRES) is a neurological condition with a combination of clinical and radiological features. Clinical symptoms include headaches, confusion, seizures, disturbed vision or an altered level of consciousness. Classic magnetic resonance imaging (MRI) findings indicate subcortical and cortical oedema, affecting mainly the posterior cerebral region. We report two paediatric cases of PRES with underlying renal diseases presenting at the Sultan Qaboos University Hospital in Muscat, Oman, in April 2010 and August 2011. The first case was an 11-year-old girl diagnosed with systemic lupus erythematosus and the second was a six-and-a-half-year-old boy on peritoneal dialysis due to multi-drug-resistant nephrotic syndrome. Both patients were hypertensive and treated with blood pressure control medications. No residual neurological dysfunction was noted in the patients at a one-year follow-up and at discharge, respectively. The role of hypertension in paediatric PRES cases, among other important risk factors, is emphasised. Additionally, MRI is an important diagnostic and prognostic tool. Prompt diagnosis and aggressive management is fundamental to preventing permanent neurological damage.

A Quantative Test for Olfaction Is the Most Sensitive Physical Examination Biomarker for Residual Neurological Dysfunction Due to Mild Traumatic Brain Injury (mTBI) That Can Be Performed in the Setting of a Clinical Examination Room (P04.022)

A Quantative Test for Olfaction Is the Most Sensitive Physical Examination Biomarker for Residual Neurological Dysfunction Due to Mild Traumatic Brain Injury (mTBI) That Can Be Performed in the Setting of a Clinical Examination Room (P04.022)

Decision making in primary sacral tumors

Background context Primary tumors of the sacrum are extremely rare lesions. Their management is governed by an interplay of complex factors. Appropriate decision making is crucial to obtain the best possible outcome in terms of maximizing disease control while attempting to minimize neurological dysfunction. Purpose Our study presents the results of a group of patients with primary tumors of the sacrum who were surgically treated by the same multidisciplinary team at a specialist oncology center over a relatively short period of time (5 years). Study design/setting Patients were identified by a retrospective review from a prospectively maintained database. Patient sample Between January 2000 and December 2005, 17 primary sacral tumors were surgically treated at our institution, a referral center for oncology. Outcome measures We evaluated the outcome in terms of local disease control, residual neurological dysfunction, and complications as a result of surgical intervention. Methods There were 12 males and 5 females. The diagnosis included chordoma in six patients, giant cell tumor in seven patients, aneurysmal bone cyst in two patients, and a chondrosarcoma and an osteoblastoma in one patient each. Sixteen of these patients were analyzed. Four lesions had their upper extent at S1, six lesions had their upper extent at S2, four lesions had their upper extent at S3, and two lesions were below S3. Ten cases were treated with wide excision and underwent partial sacral amputations. Five cases had a midline sacral amputation through S1, three through S2, and two through S3. Six benign lesions were treated with curettage. None of the patients received chemotherapy. Four cases received postoperative radiation. The follow-up duration ranged from 18 to 44 months with a mean of 31 months. Results None of the six patients who presented with loss of bladder and bowel control regained it after surgery. Of the 10 patients who had intact bladder and bowel control preoperatively only 4 retained bladder and bowel control postoperatively. Of the six patients who lost bladder and bowel control postoperatively, four patients had a wide excision where bilateral S2 roots were sacrificed. The other two cases in whom the disease extended up to S1 had curettage. Local recurrence occurred in 4 of the 10 lesions treated with wide excision. All the patients who had inadequate margins recurred. Local recurrence occurred in two of the six lesions treated with curettage. Three of the four cases who received postoperative irradiation developed recurrence. Our wound complication rate was 13%. Conclusion Wide resection with adequate margins gives the best chance of local control and should be the surgery of choice for all malignant primary sacral tumors and in benign lesions involving lower segments when preservation of both S3 roots is possible. Intralesional curettage has a higher risk of local recurrence without providing the certainty of retaining neurological function. To retain bladder and bowel control and minimize neurological dysfunction, it may be worthwhile managing benign sacral tumors that extend above S3 with serial embolization. The administration of parentral bisphosphonates may prove beneficial in cases of giant cell tumor managed with serial embolization.

Exertional Heat Stroke: A Lucky Bunch of Overly Motivated Policemen!

IntroductionExertional heat stroke is a true medical emergency resulting from thermoregulatory failure. The risk of death is related directly to the peak temperature, duration of exposure and acclimatisation period. This case report illustrates the management of multiple casualties of heat stroke in the emergency department and their outcomes.Clinical featureNine policemen arrived in the emergency department in semiconscious state after being retrieved from a group of policemen who were loaded into a bus without proper ventilation during a selection program. All of them presented with hyperpyrexia, hypotension, tachycardia and altered sensorium.TreatmentImmediate evaporative cooling with support of organ‐system dysfunction was commenced. Six patients were intubated and admitted to the intensive care unit. One patient was monitored in high dependency ward while the other two were admitted to the general medical ward.OutcomeAll patients were extubated within 48 hours. None of them had residual neurological dysfunction. One patient developed coagulopathy which required blood product transfusion. Three patients were discharged in less than one week and the rest were observed in the general ward in stable condition for liver profile monitoring.

Inherited focal, episodic neuropathies

Hereditary neuropathy with liability to pressure palsies (HNPP; also called tomaculous neuropathy) is an autosomal-dominant disorder that produces a painless episodic, recurrent, focal demyelinating neuropathy. HNPP generally develops during adolescence, and may cause attacks of numbness, muscular weakness, and atrophy. Peroneal palsies, carpal tunnel syndrome, and other entrapment neuropathies may be frequent manifestations of HNPP. Motor and sensory nerve conduction velocities may be reduced in clinically affected patients, as well as in asymptomatic gene carriers. The histopathological changes observed in peripheral nerves of HNPP patients include segmental demyelination and tomaculous or “sausage-like” formations. Mild overlap of clinical features with Charcot-Marie-Tooth (CMT) disease type 1 (CMT1) may lead patients with HNPP to be misdiagnosed as having CMT1. HNPP and CMT1 are both demyelinating neuropathies, however, their clinical, pathological, and electrophysiological features are quite distinct. HNPP is most frequently associated with a 1.4-Mb pair deletion on chromosome 17p12. A duplication of the identical region leads to CMT1A. Both HNPP and CMT1A result from a dosage effect of the PMP22 gene, which is contained within the deleted/duplicated region. This is reflected in reduced mRNA and protein levels in sural nerve biopsy samples from HNPP patients. Treatment for HNPP consists of preventative and symptom-easing measures. Hereditary neuralgic amyotrophy (HNA; also called familial brachial plexus neuropathy) is an autosomal-dominant disorder causing episodes of paralysis and muscle weakness initiated by severe pain. Individuals with HNA may suffer repeated episodes of intense pain, paralysis, and sensory disturbances in an affected limb. The onset of HNA is at birth or later in childhood with prognosis for recovery usually favorable; however, persons with HNA may have permanent residual neurological dysfunction following attack(s). Episodes are often triggered by infections, immunizations, the puerperium, and stress. Electrophysiological studies show normal or mildly prolonged motor nerve conduction velocities distal to the affected brachial plexus. Pathological studies have found axonal degeneration in nerves examined distal to the plexus abnormality. In some HNA pedigrees there are characteristic facial features, including hypotelorism. The prognosis for recovery of normal function of affected limbs in HNA is good, although recurrent episodes may cause residual deficits. HNA is genetically linked to chromosome 17q25, where mutations in the septin-9 (SEPT9) gene have been found.

Inherited Focal, Episodic Neuropathies: Hereditary Neuropathy With Liability to Pressure Palsies and Hereditary Neuralgic Amyotrophy

Hereditary neuropathy with liability to pressure palsies (HNPP; also called tomaculous neuropathy) is an autosomal-dominant disorder that produces a painless episodic, recurrent, focal demyelinating neuropathy. HNPP generally develops during adolescence, and may cause attacks of numbness, muscular weakness, and atrophy. Peroneal palsies, carpal tunnel syndrome, and other entrapment neuropathies may be frequent manifestations of HNPP. Motor and sensory nerve conduction velocities may be reduced in clinically affected patients, as well as in asymptomatic gene carriers. The histopathological changes observed in peripheral nerves of HNPP patients include segmental demyelination and tomaculous or "sausage-like" formations. Mild overlap of clinical features with Charcot-Marie-Tooth (CMT) disease type 1 (CMT1) may lead patients with HNPP to be misdiagnosed as having CMT1. HNPP and CMT1 are both demyelinating neuropathies, however, their clinical, pathological, and electrophysiological features are quite distinct. HNPP is most frequently associated with a 1.4-Mb pair deletion on chromosome 17p12. A duplication of the identical region leads to CMT1A. Both HNPP and CMT1A result from a dosage effect of the PMP22 gene, which is contained within the deleted/duplicated region. This is reflected in reduced mRNA and protein levels in sural nerve biopsy samples from HNPP patients. Treatment for HNPP consists of preventative and symptom-easing measures. Hereditary neuralgic amyotrophy (HNA; also called familial brachial plexus neuropathy) is an autosomal-dominant disorder causing episodes of paralysis and muscle weakness initiated by severe pain. Individuals with HNA may suffer repeated episodes of intense pain, paralysis, and sensory disturbances in an affected limb. The onset of HNA is at birth or later in childhood with prognosis for recovery usually favorable; however, persons with HNA may have permanent residual neurological dysfunction following attack(s). Episodes are often triggered by infections, immunizations, the puerperium, and stress. Electrophysiological studies show normal or mildly prolonged motor nerve conduction velocities distal to the affected brachial plexus. Pathological studies have found axonal degeneration in nerves examined distal to the plexus abnormality. In some HNA pedigrees there are characteristic facial features, including hypotelorism. The prognosis for recovery of normal function of affected limbs in HNA is good, although recurrent episodes may cause residual deficits. HNA is genetically linked to chromosome 17q25, where mutations in the septin-9 (SEPT9) gene have been found.

Induced hypothermia following out-of-hospital cardiac arrest; initial experience in a community hospital

Successful resuscitation from sudden cardiac death is frequently accompanied by severe and often fatal neurologic injury. Induced hypothermia (IH) may attenuate the neurologic damage observed in patients after cardiac arrest. This study examined a population of nonselected patients presenting to a community hospital following successful resuscitation of sudden cardiac death. We sought to determine whether a program of induced hypothermia would improve the clinical outcome of these critically ill patients. We initiated a protocol of IH at the Oklahoma Heart Hospital in August of 2003. Study patients were consecutive adults admitted following successful resuscitation of out-of-hospital cardiac arrest. Moderate hypothermia was induced by surface cooling and maintained for 24 to 36 h in the Intensive Care Unit with passive rewarming over 8 h. Forty-nine patients who were resuscitated and had the return of spontaneous circulation completed the hypothermia protocol. The cause of cardiac arrest was acute myocardial infarction in 24 patients and cardiac arrhythmias in 19 patients. Nineteen patients (39%) survived and were discharged. Sixteen of the patients discharged had no or minimal residual neurologic dysfunction and 3 patients had clinically significant residual neurologic injury. A program of induced hypothermia based in a community hospital is feasible, practical, and requires limited additional financial and nursing resources. Survival and neurologic recovery compare favorably with clinical trial outcomes.

Stroke in children.

Stroke is defined as the sudden occlusion or rupture of cerebral arteries or veins resulting in focal cerebral damage and neurological deficits. Forms of stroke resulting from vascular occlusion are arterial ischemic stroke (AIS) and sinovenous thrombosis (SVT) and those resulting from vascular rupture are called hemorrhagic stroke. Stroke in children is relatively rare and frequently results in a lack of recognition and delay in diagnosis. The etiologies of stroke in children are legion and multiple risk factors coexist unlike unifactorial etiology in adults. Heart disease whether congenital or acquired, malformations, metabolic and hematological disorders and vasospastic conditions like migraine are seen more often in childhood strokes. The purpose of diagnostic evaluation includes confirmation of the presence of a cerebrovascular lesion, exclusion of other types of neurological dysfunction and identification of etiology of the stroke. The treatment of stroke in children has been primarily directed toward stabilizing systemic factors and management of the underlying causes. Various antithrombotic and non antithrombotic therapies are discussed. The use of anticoagulant therapy appears to be increasing in pediatric AIS. Mortality after stroke in children ranges from 20% to 30% depending on the location and the underlying cause. Residual neurological dysfunction is present in more than 50% of survivors.