Acute encephalopathy associated with ingestion of a mushroom,Pleurocybella porrigens (angel's wing), in a patient with chronic renal failure

The authors report an autopsy case of acute encephalopathy in which generalized convulsion and coma occurred after ingestion of Pleurocybella porrigens (angel's wing mushroom). The patient was a 65‐year‐old man who had undergone hemodialysis for 3 months due to chronic renal failure. Pathologic examination of the brain revealed extensive postinfarction‐like cystic necrosis in the bilateral putamens and multiple spotty necroses in the deep cerebral and cerebellar cortices. In 2004, similar acute encephalopathy related to ingestion of the mushroom was endemic in Japan, the pathogenesis of which remains to be elucidated.     

Clinical, neuroimaging and electroencephalographic findings of encephalopathy occuring after the ingestion of "sugihiratake" (Pleurocybella porrigens), an autumn mashroom: a report of two cases]

We report a 54-year-old man (case 1) and a 79-year-old woman (case 2) who presented with encephalopathy of unknown cause. Both patients were on hemodyalysis and took an autumn mashroom, "sugihiratake" (Pleurocybella porrigens), two to three weeks prior to the onset of neurological alterations. The clinical syndrome of those patients was characterized by weakness and involuntary movements of the extremities (cases 1 and 2) or dysarthria (case 1) at the onset of the disease and subsequent intractable focal motor seizures, resulting in generalized status epilepticus or comatose state, six (case 1) or three (case 2) days after the disease onset. Epileptic seizures were gradually improved in both cases. On brain MRI of case 1, no relevant lesions were detectable at the onset day, but, 6 days after onset, T2-high intensity lesions were noted in the subcortical white matter of the insular cortex, claustrum, external capsule, putamen and globus pallidus on both sides. On brain CT scan of case 2, there were no apparent lesions at the onset day, but, 4 days after onset, low density areas were noted bilaterally in the subcortical white matter of the insular cortex. Electroencephalography of the two patients taken on a day of comatose state showed periodic synchronous discharge (PSD), which disappeared when their consciousness levels were improved. As far as we have examined, there was no findings to suggest the cause of the encephalopathy in routine laboratory examinations and various viral antibody studies of the blood and cerebrospinal fluid. The reported patients could constitute a newly recognized disease entity, "sugihiratake" encephalopathy. Our observations suggest that it can be an encephalopathy with subacute progression and affect mainly the basal ganglia. Neuroimaging study and electroencephalographic findings may help the diagnosis, although they may remain unremarkable for several days after onset of the neurological alterations.     

Primary new daily persistent headache (NDPH): clinical characteristics of forty-three cases in Japan]

Although the primary chronic daily headache is subdivided into chronic migraine, chronic tension-type headache, hemicrania continua, and new daily persistent headache (NDPH), the characteristics of NDPH have not been well studied in Japan. The purpose of this study is to investigate the clinical characteristics of the primary NDPH. Twenty-one men and twenty-two women with primary NDPH, aged 14 to 73 years were diagnosed at Toyonaka Municipal Hospital from November 1997 to April 2003. Age of the onset ranged from 13 to 73 years (mean, 34.8). The onset of headache occurred in relation to a stressful life event in 16%, a systemic infection in 12% and an extracranial surgery in 7%. Any precipitating events could not be identified in approximately two-third of patients (65%). Quality of the headache was described as dull and/or pressure-like sensation in 91%, throbbing and/or pulsating sensation in 28%. NDPH needs further study, because it appears to be one of the most refractory headaches.     

急性脑出血患者的缺血危险因素分析

目的：分析急性脑出血患者的脑缺血相关危险因素,探讨急性脑出血的合理治疗方案。方法：动态观察了54例急性脑出血和75例急性脑梗死患者治疗前后的血液流变学,血浆渗透压,血小板聚集率以及头颅CT的改变。结果：脑出血组和脑死组患者的全血粘度,血浆渗透压,红细胞压积,纤维蛋白原,血小板浓度无明显差异,脑梗死组血小板聚集率明显高于脑出血组（P＜0．05）。31例脑出血患者治疗两周后复查头颅CT,其中有4例患者出现新的脑梗死 灶,此4例患者平均年龄以及纤维蛋白原水平高于脑出血组（P＜0．01）,结论：脑出血和脑死是发生于脑血管病变基础上的两个相关联的病变,二者有许多共同的危险因素,脑出血发生及演变过程中可继发病脑梗死,而该过程中血小板聚集率及纤维蛋白原的增高可能与脑出血继发脑梗死的发生有一定的关系,脑出血治疗过程 应强调沦的脑功能监测,尽量有脑出血急性期后早期改善脑循环并同时给予脑保护治疗。     

Acute encephalopathy among patients with renal dysfunction after ingestion of "sugihiratake", angel's wing mushroom--study on the incipient cases in the northern area of Niigata Prefecture]

An outbreak of acute encephalopathy among 6 patients with renal dysfunction after eating "Sugihiratake" mushroom in the northern area of Niigata Prefecture was reported. All of the patients had varying degrees of renal dysfunction, and 3 of them were on dialysis treatment. Patients initially presented with shaking limbs and difficulty in walking. Several days later, disturbances in consciousness developed, which were followed by status epileptics. Four patients were put on mechanical ventilation for seizure control. Two of the 6 cases died. CSF examination showed elevated protein without pleocytosis; bacterial, rickettsial and viral screenings was negative. Brain CT and MRI studies showed edema in the claustrum and external capsule, and in the white matter of the base of the frontal, parietal, and temporal lobes. No common drug administration was recognized. All of the patients had a history to have ingested "Sugihitarake" mushroom (angels' wing mushroom, pleurocybella porrigens) in varying quantities and frequencies prior to the onset of the illness. This mushroom may have induced toxic encephalopathy although no similar case have been reported in the past.     

高血压脑病影像学特征分析

目的分析高血压脑病的影像学特征,探讨其影像学诊断和鉴别诊断。方法回顾性分析35例临床确诊为高血压脑病患者的头部CT、MRI表现及临床资料(侧重影像学)。结果 35例患者均行头颅CT检查,表现阳性12例,阴性23例;35例中18例同时行MRI检查,12例表现阳性,6例表现为阴性。阳性病例影像学主要表现为脑水肿,伴灶状脑出血6例。患者收缩压为180～230mmHg、舒张压为120~150mmHg,均伴有不同程度颅内压升高临床表现。结论高血压脑病的影像学改变主要为脑水肿表现,其诊断与鉴别需密切结合临床。     

Charcot—Marie—Tooth病的遗传,临床和电生理观察（附20例临床分析）

本文报道 ２０例 Ｃｈａｒｃｏｔ－Ｍａｒｉｅ－Ｔｏｏｔｈ病的遗传、临床和电生理资料。其中男 １６例,女 ４例,平均发病年龄为２６．７５岁。发现４例显性遗传,３例隐性遗传,１０例散发,３例遗传情况不详。主要症状有高弓足、垂足、鹤腿和腱反射消失;上肢前臂有肌萎缩者占１／４。所有病人电生理检查均有失神经现象,特别是ＭＣＶ有明显减慢。且发现ＭＣＶ的减慢和临床严重程度无相关联系。     

An analysis of quality of life (QOL) in patients with epilepsy and comorbid psychogenic nonepileptic seizures (PNES) after vagus nerve stimulation (VNS)

PurposePatients with epilepsy (PWE) may suffer from comorbid psychogenic nonepileptic seizures (PNES). The efficacy of vagus nerve stimulation (VNS) in the treatment of epilepsy and depression is established, however the impact on PNES is unknown. Since many patients with PNES have comorbid depression, we explored the impact on quality of life (QOL) that VNS has on PWE and PNES.MethodsThe video electroencephalogram (vEEG) of all patients who underwent VNS at our institution was reviewed. Patients diagnosed with both psychogenic seizures and epileptic seizures on their vEEG were included in this study. These patients were contacted, and given a QOLIE-31 survey to assess their quality of life after VNS. Patients also completed a separate survey created by our group to categorize the quartile of their improvement. Pre-operative psychiatric disease was retrospectively reviewed.ResultsFrom a period of 2001 to 2016, 518 patients underwent placement of VNS for drug resistant epilepsy (DRE) at our institution. In total, 16 patients were diagnosed with both epilepsy and PNES. 11/16 patients responded to our questionnaire and survey. 9 out of 11 patients felt that their epileptic seizures had improved after VNS, while 7 of the 11 patients felt that their psychogenic episodes had improved. 2(28.6%), 1 (14.3%), and 4 (57.1%) of participants said their PNES improved by 25–50%, 50–75%, and 75–100%, respectively. 3(27.3%), 3 (27.3%), 1 (9.1%), and 4 (36.4%) of the participants said their epileptic seizures improved by 0–25%, 25–50%, 50–75%, and 75–100%, respectively. The average overall score for quality of life for the study participants was found to be 51 (± 8) out of 100.ConclusionPatients with epilepsy and comorbid PNES may benefit from VNS. It is unclear whether the benefit is conferred strictly from decreased epileptic seizure burden. The possible effect on PNES may be related to the known effect of VNS on depression. Further studies are necessary to elucidate the role of VNS in the treatment of PNES and possibly other psychiatric disease.     

强直性肌营养不良1型患者的临床特点及CTG重复次数分析

目的 总结强直性肌营养不良1型患者的临床、神经电生理和遗传学特点。方法 收集3例强直性肌营养不良1型患者的临床症状、肌电图、肌肉病理及基因检测结果。结果 3例患者（男性1例）均为成年起病,慢性病程。临床表现为四肢远端无力和肌强直;2例患者存在眼外肌或面肌无力;1例伴随前额脱发;2例存在心脏传导紊乱如阵发性室性心动过速、左前束支传导阻滞、右束支传导阻滞;2例出现脑白质病变。3例强直性肌营养不良1型患者DMPK基因3'非翻译区的突变CTG重复次数分别为104、150、299,均大于50次。3例患者肌电图检查所检肌肉均可见肌强直放电。结论 强直性肌营养不良1型患者肌无力主要出现在远端肌群,心脏传导紊乱和脑白质病变是其多系统受累的显著表现。肌电图可以发现临床下肌强直放电,是最敏感的筛查手段。     

Macrosomia, macrocrania and motor disorders in childhood, Sotos syndrome (McKusick 11755): report of 7 cases and review of clinical aspects of 198 reported cases]

Children with Sotos syndrome have growth acceleration, macrocephaly, acromegaloid features and delay in neuropsychomotor development during infancy. Syndrome delineation and differential diagnosis are based on evaluation of phenotypic characteristics and evolutive history of the patients. Seven patients with this syndrome are reported, and the relative occurrence of the phenotypic characteristics present in 198 reported cases are reviewed. Motor difficulties present in those patients during early infancy are responsible for the poor performance on IQ tests. Oriented stimulation should be encouraged in order to help the affected children to overcome their initial difficulties and to achieve normal scholarity and life performance.     

An analysis of clinical outcomes of 91 pregnancies in 83 women treated with danaparoid (Orgaran)

Danaparoid case reports of 91 pregnancies in 83 patients with a history of thrombophilia and/or intra-uterine growth retardation have been analysed. All had intolerance to the heparins including HIT and acute or past thromboses or a history of repeated pregnancy loss (RPL).Danaparoid was started in the first, second and third trimesters in 60.2%, 19.3% and 20.5% pregnancies respectively at a dosing intensity of 1000 to 7500 U/day. Subcutaneous and/or intravenous administration was continued for a median 105 days (range 1-252) during pregnancy and 7 days (range 2 to 56) post-partum.The live birth rate was 90.4% (75/81) and danaparoid was restarted after 37 deliveries. Maternal adverse events in 46.2% of the pregnancies included 2 post caesarian deaths (a failed post-operative resuscitation and a major bleed in a patient refusing transfusion), 3 non-fatal major bleeds (associated with caesarian section and faulty placental implantation), 3 thrombo-embolic events unresponsive to danaparoid dose increase and 10 recurrent rashes.Seven early miscarriages, 1 therapeutic termination and 1 neonatal death occurred. In 13 reports a maternal, but no fetal, adverse event was attributed to danaparoid.Anti-Xa activity levels in maternal plasma were between 0.1 and 1.2 U/mL, absent from 6 fetal cord blood samples and 0 - 0.07 U/mL in the 5 maternal breast milk samples tested.

Conclusion

The successful birth rate and adverse event profile indicates that danaparoid can be an effective and safe alternative anti-thrombotic in pregnancies complicated by HIT or intolerance or resistance to (LMW)heparins.     

难治性颞叶癫痫显微外科手术疗效分析(附27例报道)

目的 探讨难治性颞叶癫痫的术前评估、标准前颞叶切除术的实际应用及其疗效的影响因素.方法 在显微镜下对27例难治性颞叶癫痫患者进行标准前颞叶切除术治疗.部分患者同时行皮质热灼术.术后均随访1年以上,根据手术预后按Engel's分级情况将患者分为满意组(患者分级为Ⅰ级)与非满意组(患者分级为Ⅱ级以上),并对两组患者术前发作形式、影像学改变和脑电图监测等情况进行回顾分析.结果 本组27例患者均无严重并发症发生.术后疗效Engel's分级Ⅰ级20例(74.1％),Ⅱ级2例(7.4％),Ⅲ级4例(14.8％),Ⅳ级1例(3.7％),其中满意组患者20例,非满意组患者7例.两组间比较,满意组术前无继发性强直阵挛发作、影像学无双重改变和发作间期刺激区(IZ)局限于单侧颞区的患者比例高(均P＜0.05).结论 手术前充分认识发作形式、影像学检查结果和脑电图特点,有助于提高标准前颞叶切除术的疗效.     

Correlates of local cerebral blood flow (CBF) in normal pressure hydrocephalus patients before and after shunting--A retrospective analysis of [(15)O]H(2)O PET-CBF studies in 65 patients

OBJECTIVES: Findings in local cerebral blood flow (rCBF) in Normal pressure hydrocephalus (NPH) have always been challenged by the variable and inconsistent relation to clinical symptoms before and after shunt treatment. [(15)O]H(2)O PET data from a consecutive cohort of 65 idiopathic NPH patients were retrospectively analyzed questioning whether the functional status before and after shunt treatment might correlate with local blood flow. PATIENTS AND METHODS: Using statistical parametric mapping (SPM99, Wellcome Department of Cognitive Neurology, London), the [(15)O]H(2)O uptake was correlated with the preoperative clinical scores, graded according to a modified Stein and Langfitt score. Furthermore, differences in the uptake in the pre-and post-shunt treatment study after seven to 10 days in patients with and without clinical improvement were studied. RESULTS: A higher clinical score significantly correlated with a reduced tracer uptake in mesial frontal (k=1,239 voxel, Z=4.41) and anterior temporal (k=469, Z=4.07) areas. In the mesial frontal areas, tracer uptake showed significant reciprocal changes in the clinically improved vs. the unimproved patients. CONCLUSION: Matched with the existing literature, the regional blood flow alterations are suggested relevant to the NPH syndrome and to post-treatment functional changes. The present rCBF findings warrant prospective studies on the accuracy of neuroimaging studies as they may provide a more specific insight into disease mechanisms.     

McArdle disease (gycogenosis type V): analysis of clinical, biological and genetic features of five French patients

INTRODUCTION: McArdle disease (glycogenosis type V) is an autosomal recessive metabolic myopathy. Defect in glycogen breakdown is due to mutations of the gene for myophosphorylase (PYGM). Among patients of the department, we searched for correlations between disease phenotype, biochemistry analysis of muscle samples and PYGM genotype. METHODS: We included five patients whose muscle biopsy showed deposits of glycogen and negative histochemical staining for myophosphorylase. RESULTS: All patients exhibited exercise intolerance and high serum CK levels (mean 4400). Two of them had an acute renal insufficiency caused by rhabdomyolysis. One patient developed moderate late-onset muscle weakness of the proximal part of upper limbs. Muscle glycogen concentration was high (three times the normal). Myophosphorylase activity was undetectable in four muscle samples out of five. Two patients were homozygous and two other heterozygous for the R50X mutation of PYGM. The other one had a novel missense mutation S814N. Patients homozygous for R50X mutation had higher CK levels (8080 versus 1457, p=0.046), but disease severity and muscle glycogen concentrations were equivalent. CONCLUSIONS: Our patients had typical clinical and laboratory features of McArdle disease. Diagnosis was suggested by exercise intolerance with high CK levels. The R50X mutation was the most common (60% of the mutated alleles). We found no relationship between clinical severity, PYGM genotype and biochemistry analysis of muscle samples.     

An analysis of regional cerebral blood flows (rCBF) in 120 patients with cerebrovascular disease

The rCBF in a group of 120 patients with cerebrovascular disease (CVD) diagnosed clinically as well as with CT scans and a group of 120 healthy subjects as controls was measured with the 133Xenon inhalation method. The result showed that the rCBF of the CVD group was significantly lower than that of the control group (P less than 0.01). The tally rate between the rCBF reduction areas and the lesion sites was 85 per cent. The areas of rCBF reduction measured with 133Xenon inhalation were larger than the extents of the lesions shown by CT scans in 47 cases of cerebral infarction. The rCBF reductions of bilateral cerebral hemispheres were found in 16 cases of cerebral infarction. The average hemispheric rCBF obviously decreased in 12 cases of multiple cerebral infarction with dementia, but showed no decrease of rCBF in 18 cases of small focal cerebral infarction.     

Preliminary clinical trial of intrathecal rt-PA (TD-2061) for the prevention of cerebral vasospasm in patients with aneurysmal subarachnoid hemorrhage]

The results of preliminary clinical trial (a multicenter, open-label, dose escalation study) of intrathecal recombinant tissue plasminogen activator (rt-PA) for the prevention of cerebral vasospasm were reported. Seventeen patients admitted within 48 hours of subarachnoid hemorrhage (SAH) were enrolled in this study. Patients ranged from 42 to 69 years of age. All cases enrolled were classified in clinical grade II, III or IV according to the classification of Hunt and Kosnik and in group 3 or 4 according to Fisher's CT grading scale. Surgery for clipping the aneurysms were performed and a small silicone catheter was left in the subarachnoid space. Twenty four hours after the surgery intrathecal bolus infusion of rt-PA was started through the silicone catheter at 6-hour intervals for 3 days. Patients were divided into 4 groups based on the dosage of rt-PA for each infusion. The dosage of rt-PA for each infusion and a number of cases in each group was as follows; 25 KIU in 4 cases, 75 KIU in 6 cases, 200 KIU in 4 cases and 600 KIU in 3 cases. There was no significant difference in the clearance of subarachnoid clots between four groups. However, the occurrence of both symptomatic and angiographic vasospasm was less in the 75 KIU group than in other three groups. Intracranial bleeding complications were noted in 4 patients (1/6 in the 75 KIU group, 2/4 in the 200 KIU group and 1/3 in the 600 KIU group). Serial coagulation studies demonstrated no evidence of systemic fibrinolysis. Disorientation was noted in 2 out of 3 patients of the 600 KIU group.(ABSTRACT TRUNCATED AT 250 WORDS)