Visual perseveration: a new side effect of maprotiline

OBJECTIVE: The aim of this paper is to describe a case of visual perseveration including palinopsia during maprotiline therapy. METHOD: A single case report. RESULTS: The patient, a 56-year-old depressive man, suffered from visual perseveration during maprotiline therapy. The visual perseveration was dose-related and disappeared with reduction and cessation of the therapy. CONCLUSION: The present findings suggest that maprotiline can induce visual perseveration including palinopsia in some patients.     

Application of a lag contingency to reduce perseveration on circumscribed interests

Objective: To evaluate the effects of a lag reinforcement contingency on the conversation topics of children diagnosed with autism who perseverated on circumscribed interests (CIs). Method: Single-case design study with two participants, age 11 and 12 years. A functional analysis employed an alternating-treatments design to assess effects of contingent attention on conversational behavior. A multiple-baseline with an embedded reversal design was employed in a subsequent lag contingency analysis, in which the conversation partner provided attention at the end of each 10 s interval if the content of the participant’s conversation met the requirement of a Lag 0, Lag 1, or Lag 2 contingency. Results: The participants’ conversational behavior was sensitive to a conversation-partner’s attention as a consequence. Lag contingencies successfully altered the prevalence of CI-related and-unrelated talk.Conclusion: Lag contingencies can shift conversation content away from CIs, but further research is needed to identify appropriate treatment goals with respect to variability and content.     

Myasthenia gravis and scleroderma: two cases and a review of the literature

Myasthenia gravis is uncommon in patients with scleroderma, and when diagnosed is usually associated with previous use of d-penicillamine. Clinically, both myasthenia and scleroderma may present with fatigue, weakness and bulbar symptoms, so one of diagnoses may be delayed. We report two new cases and review clinical features of 12 other reported cases of co-existing scleroderma and myasthenia gravis, unrelated to previous d-penicillamine therapy. Co-occurrence of myasthenia and scleroderma was reported almost exclusively (13/14) in women with a mean latency of 7.03 years. Most patients (10/11) had seropositive generalized myasthenia, and there were no cases with exclusively ocular symptoms. Three patients with pre-existing myasthenia were safely treated with d-penicillamine. Myasthenia and scleroderma occur in the context of an underlying autoimmune diathesis, but their co-occurrence could be underreported as the recognition of either disorder may be delayed by overlapping clinical symptoms. Our findings also suggest that d-penicillamine may be cautiously used in selected patients with pre-existing scleroderma and myasthenia, when potential benefits outweigh the risk of possible myasthenia exacerbation.     

The neuropathology of melioidosis: two cases and a review of the literature

Melioidosis is an infectious disease caused by Burkholderia pseudomallei and is hyperendemic in the Top End of the Northern Territory of Australia, as well as being widespread throughout tropical south east Asia. The infection is primarily acquired via the inoculation of compromised surface tissues by contaminated soils and water and it can cause an acute, rapidly fatal illness. Although pneumonia is the commonest manifestation, neurological presentations have been described, most notably encephalomyelitis. This paper presents the neuropathology of 2 fatal cases of neurological melioidosis and reviews the relevant literature.     

Meningioangiomatosis: report of two cases and literature review

Meningioangiomatosis (MA) is a rare benign intracraneal lesion. The majority of cases are sporadic although the association of this lesion with familial neurofibromatosis (NF) type 2 is well known. NF-associated MA may be multifocal and is often asymptomatic and diagnosed at autopsy. Non-associated cases are usually symptomatic, occurs in children and young adults and frequently arise in leptomeninges and underlying cerebral cortex. In the present work, we describe two new non-associated cases of MA in two boys, seven and one year old with seizures that disappeared after surgical excision. Histopathologically, the lesion was predominantly cellular in one case and more fibrous in the other. From the literature review we concluded that sporadic cases present as single lesions which manifest by seizures or persistent headaches. Rarely MA has been described to coexist with meningiomas. Histopathologically, MA is characterized by a plaque-like proliferation of meningothelial and fibroblast- like cells surrounding small vessels and trapping islands of gliotic cortical tissue. The lesion does not show significant atypia, mitosis or necrosis. Although all cases of MA share unifying features, there are different degrees of histological presentation with cases predominantly cellular and others more fibrous and calcified. This could correspond to different stages in the evolution of the MA. Symptoms disappear with the complete excision of the lesion.     

ECT post eye surgery: two cases and a review of the literature

Little is known about the safety of electroconvulsive therapy (ECT) following any surgical procedure. We present two cases of elderly patients who required treatment with ECT shortly after eye surgery. One patient received ECT 12 days post cataract surgery. The other patient received ECT 1 week post vitrectomy on one eye, and then a few years after that she received ECT 2.5 weeks post cataract surgery on the other eye. There were no complications or adverse events for either patient. We review the existing literature on the effects of ECT on intraocular pressure and discuss the implications on patients who have recently undergone ophthalmic surgery.     

复发性Fisher-Bickerstaff综合征2例临床分析

目的探讨复发性Fisher综合征(FS)叠加Bickerstaff脑干脑炎(BBE)的临床特点和疾病命名。方法分析2例复发性Fisher-Bickerstaff综合征的患者的临床资料,并复习相关文献。结果例1表现为复发性睡眠增多、走路不稳、视物成双,肌电图检查提示下肢神经源性损害;例2表现为复发性视物成双、走路不稳,第2次发病出现四肢无力,查体双下肢远端肌力3级,双侧Babinski征阳性,肌电图提示四肢神经源性损害;2例患者除具有眼肌麻痹和共济失调的症状外,都有中枢和周围神经受损的表现;依据患者的病史、查体、临床检测和疾病经过,诊断为复发性Fisher-Bickerstaff综合征,第2例患者叠加吉兰-巴雷综合征。结论具有周围神经损害的BBE和具有中枢神经损害的FS从临床表型上不能截然分开,Fisher-Bickerstaff综合征的概念更能准确地表述这类患者的表型特点。     

Infant botulism: two recent cases and literature review

Infant botulism is a cause for significant pediatric morbidity in the United States, though early recognition and supportive care can greatly improve clinical outcomes. Since the approval of human botulism immune globulin by the United States Food and Drug Administration (FDA) for the treatment of infant botulism in 2003, the importance of prompt initiation of therapy has been emphasized, with clinical suspicion remaining the mainstay of diagnosis. In this report, 2 cases of infant botulism are described. Each presented to the Pediatric Neurology service at our institution in Upstate New York in the spring and summer months of 2007 and were felt to be related to markedly dusty environmental conditions and the probable ingestion of C. botulinum organisms present in soil. Following this, a comprehensive review of the literature regarding infant botulism in the United States is presented, wherein the pathophysiology, clinical features, epidemiology, and treatment are discussed.     

Episodic paroxysmal hemicrania: two new cases and a literature review.

Episodic paroxysmal hemicrania (EPH) is a rare disorder characterized by discrete bouts of hemicranial headache separated by headache-free remissions. Although EPH resembles episodic cluster headache in the location and quality of pain as well as the pattern of associated autonomic features, it is distinguished by the greater frequency and shorter duration of individual headaches. Differentiation of these disorders is important because EPH almost invariably responds to treatment with indomethacin but not to standard cluster headache therapy.     

Meningo encephalitis revealing Q fever: two cases and a review of the literature

Q fever is a zoonosis caused by Coxiella burnetii. The acute Q fever is usually characterized by a self-limited flu-like syndrome, fever, pneumonia and hepatitis. Symptoms of the chronic Q fever (evolution>3 months) mainly consist of endocarditis with negative culture. Focal neurological symptoms are rarely observed. Neurological symptoms of acute Q fever consist of meningitis or meningo-encephalitis. Neurological symptoms of chronic Q fever are cerebral embolisms from Coxiella burnetii infected heart valves. We herein report two patients with meningoencephalitis revealing acute Q fever.     

Attentional dysfunction of the central executive in AD: evidence from dual task and perseveration errors

This study examined the capacity of 27 Alzheimer's disease (AD) patients to divide attention between two simultaneous tasks, as compared to 27 elderly controls. In order to determine whether distribution of attention is affected by age, a younger group was included. The results showed a marked impairment in the capacity of the AD patients to combine performance in two simultaneous tasks compared to the elderly controls, but the latter group did not differ from the younger participants, indicating a disease rather than an age effect, and replicating the results of Baddeley et al. (2001). The present study also analysed whether perseverations committed in a previous study (Sebastian et al., 2001) by some AD and elderly control participants in the Brown-Peterson (B-P) task (Brown, 1958; Peterson and Peterson, 1959) were related to their attentional capacity in the dual task. Although the elderly controls committed perseveration errors, a negative correlation was only found in the AD group between the index of attention distribution in the dual task and the frequency of perseverations in the B-P task. Our results would therefore suggest that AD patients show a dysfunction of the central executive and/or a deficit in their ability to allocate cognitive resources. This could be interpreted in terms of problems in updating the contents of working memory and suppressing activation of no-longer-relevant information in the early stages of AD, leading to perseveration errors in the B-P task.     

Supratentorial enterogenous cyst: a report of two cases and review of literature

Enterogenous cyst is extremely rare in the supratentorial compartment. Two adult patients with histologically variefied supratentorial entrogenous cyst are described. Light microscopy and immunohistochemistry examination revealed the endodermal origin of the cyst. Enterogenous cyst should be considered in the differential diagnosis of non-enhancing cyst in the supratentorial compartment. Total excision of the cyst wall should be done wherever feasible. Preventing spillage of the cyst contents during surgery is mandatory to avoid postoperative intractable seizures and craniospinal dissemination.     

Productive and defective impairments in the neglect syndrome: graphic perseveration, drawing productions and optic prism exposure

The effects of adaptation to prisms displacing rightwards the field of vision on omission errors, and on perseveration and other graphic productions in a line cancellation task, were assessed in nine right-brain-damaged patients with left unilateral spatial neglect. Prism adaptation improved both neglect, as indexed by omission errors, and perseveration behaviour, up to a delay of 60 min. No correlation was found between omission and perseveration errors in all assessments. The suggestion is made that perseveration and other complex graphic productions made by right brain-damaged-patients with left spatial unilateral neglect is due to a defective monitoring of complex motor behaviour, frequently associated to cerebral damage involving the right frontal lobe. Interpretations of perseveration behaviour in terms of allochiria and directional hypokinesia are considered, and their limits discussed.     

Pedicle fracture of the axis: report of two cases and a review of literature

Clinical and neuroradiological features of two cases with unilateral pedicle fractures of C2 were presented and the literature of this rare traumatic fracture was reviewed. Both 37- and 57-year-old woman had traffic accident and were admitted in our institute. Both patients were neurologically free except for neck pain. Plain X-ray of cervical spine did not show abnormal findings. Computerized tomography (CT) with bone window showed the location and extension of the traumatic fracture in C2 pedicle. The fracture was located in the portion between the lateral border of the odontoid process and pars interarticularis. Fractures of the C2 pedicle are rarely seen in acute traumatic fractures. They can be overlooked because there is confusion in the describing of anatomical location of C2 pedicle in some medical literature. For proper diagnosis of pedicle fracture of the axis, understanding of the anatomy of C2 and CT findings with bone window are important.     

Pediatric cervical chordoma: report of two cases and a review of the current literature

Introduction  

The majority of chordomas occur between the fifth and seventh decades of life and are thus extremely rare in children, which account for less than 5% of all spinal chordoma cases.     

Giant cerebral cavernous hemangiomas: a report of two cases and review of the literature

Giant cerebral cavernous malformations (GCM) are rare vascular anomalies. They occur predominantly as solitary lesions in the supratentorial compartment. They are usually not associated with any other vascular malformations. Clinical Presentation GCM are common in the second decade, affecting females predominantly, and occur without familial association. They are all symptomatic due to their giant size and location. The GCM mimic neoplastic lesions because of their size and need to be considered in the differential diagnosis. A complete surgical extirpation is the treatment of choice. Use of intraoperative neuronavigation, diffusion tensor imaging (DTI) of fiber tracts and electrophysiological monitoring assist in safe and total excision of the lesions. A complete surgical excision of GCM is possible without significant surgical morbidity and results in long term cure.     

Primary dural lymphoma: a report of two cases with review of the literature

Primary lymphoma arising in dura is exceedingly rare. We report the clinicopathologic findings of two patients with primary B-cell lymphoma of dura. Both were female, 38 and 45 years old. Prior to biopsy they were felt to have meningioma on preoperative magnetic resonance imagery. Histologically, tumors were classified as MALT-type lymphoma. Literature describe only 14 reports of similar entity. Primary lymphomas arising in dura appear to have a more favourable clinical course compared to PCNSL and may require a less aggressive treatment.     

Antley-Bixler syndrome Description of two new cases and a review of the literature

Antley-Bixler syndrome was first described in 1975, and to date 20 cases have been reported. In addition to brachycephaly, the syndrome is associated with midface hypoplasia, often with choanal stenosis or atresia, bilateral radiohumeral synostosis, multiple joint contractures, femoral bowing and long bone fractures, ``pear-shaped' nose, dysplastic ears and, occasionally, urogenital or cardiac defects. Survival is closely linked to upper airway obstruction. This, in addition to craniosynostosis, also affects mental prognosis. The cluster of malformations and their severity are variable, and while numerous children have died early from respiratory distress, one third of them are alive and have had quite satisfactory development. With early and effective prevention of respiratory complications and early treatment of craniosynostosis, the overall prognosis can be favorable. The mode of inheritance is probably autosomal recessive, and midtrimester prenatal diagnosis is feasible. Genetic counseling depends on accurate prognostic and therapeutic data. We describe two new cases, a 4-year-old boy with unilateral coronal synostosis and radio-humeral synostosis on the same side and an 18-month-old girl with brachycephaly and imperforate anus. Received: 30 October 1996