Parkinsonism associated with Sj?gren's syndrome: three cases and a review of the literature.

Sj?gren's syndrome (SS) is a common multisystem autoimmune disorder. As with other autoimmune disorders such as systemic lupus erythematosus (SLE), SS has been associated with a wide range of neurologic abnormalities. Parkinsonism has been reported previously in five SS patients. We present three additional cases of SS with parkinsonism.     

Association of lower cranial nerve schwannoma with spinal ependymoma in ? NF2.

A 15 year old male, who had earlier been operated for intraspinal intramedullary ependymoma, subsequently developed a right cerebello pontine (CP) angle mass. A diagnosis of right CP angle ependymoma was considered, in view of established histology of previously operated spinal lesion. Histopathological examination of the well defined extra-axial mass, which was attached with ninth cranial nerve, however revealed a schwannoma. A diagnosis of Neurofibromatosis-2 (NF2) is strongly suspected, because of well established fact, that the spinal ependymomas may have association with lower cranial nerve schwannomas in NF2. Cranial and spinal MRI screening for early diagnosis of associated, asymptomatic lesions, in suspected cases of NF2, particularly in children, is recommended.     

Tremor associated with Klinefelter syndrome – A case series and review of the literature

BackgroundPrevious case series suggested a link between Klinefelter syndrome (KS) and essential tremor (ET) or an ET-like syndrome.MethodsWe investigated three KS-patients with tremor including tremor-analyzes and discuss our data in context to findings from a literature review. The clinical outcome after deep brain stimulation (DBS) is also reviewed.ResultsTremor in KS is predominantly a postural and kinetic tremor that resembles ET. Our patients were further characterized by absent family history for tremor in first degree relatives, lack of subjective alcohol responsiveness inquired by history, and tremor onset in childhood. One of our patients and two cases from literature improved after DBS of the ventral intermediate nucleus (VIM) of the thalamus.ConclusionsTremor in KS shares several features with ET. If other characteristics such as family history, alcohol responsiveness, and age at tremor onset may serve as discriminating factors from ET, needs to be further investigated. First observations suggest that VIM-DBS may be efficacious.     

Does vaginismus exist? A critical review of the literature.

The basic strategies and methods for assessing and treating vaginismus were proposed by the early 20th century and have not essentially changed. Etiological theories have changed over time but are not supported by controlled empirical studies. This critical review of the literature disputes the widely held belief that vaginismus is an easily diagnosed and easily treated sexual dysfunction. We propose a reconceptualization of vaginismus as either an aversion/phobia of vaginal penetration or a genital pain disorder.     

Chemical priming for spinal cord injury: a review of the literature. Part I��factors involved

Introduction  

There are significant differences between the propensity of neural regeneration between the central and peripheral nervous systems.     

Medulloepithelioma of the optic nerve with intradural extension – report of two cases and a review of the literature

Medulloepithelioma is an uncommon tumour. Among the various sites of origin the optic nerve is rare. We report here two cases of medulloepithelioma of the optic nerve that had extended intradurally into the cranial cavity. A review of the published cases is also given. Received: 30 April 1999 Revised: 17 June 1999     

The moral brain and moral behaviour in patients with Parkinson’s disease: a review of the literature

Morality is a complex and versatile concept that necessitates the integrated activity of multiple interacting networks in the brain. Numerous cortical and subcortical areas, many of which are implicated in either emotional and cognitive control or Theory of Mind, are involved in the processing of moral behaviour. Different methods have been used to investigate various aspects of morality, which has lead to confusing and sometimes opposing results. Emotional, cognitive and personality changes have long been recognized in Parkinson’s disease (PD) patients, suggesting a potential impact on moral aspects of behaviour in daily living situations. Alterations in social cognition have been described in all stages of PD but these are rather directly related to PD pathology and not to dopaminergic or DBS treatment. There are no convincing data supporting the hypothesis that dopaminergic treatment or deep brain stimulation of the STN per se interfere with morality in PD patients, although subgroups of patients may display socially unacceptable behaviour. Research in social cognition in PD patients is a fascinating topic that needs further attention in view of the impact on quality of life for PD patients and their caregivers.     

Langerhans cell histiocytosis of the parietal bone with epidural and extracranial expansion – case report and a review of the literature

Langerhans cell histiocytosis is a rare neoplasm that belongs to the histiocytic and dendritic cell neoplasm group according to the 2008 WHO classification. It has been defined as neoplastic proliferation of Langerhans cells that express CD1a and S-100 proteins and have Birbeck granules on the ultrastructural examination. Clinical presentation and behaviour are heterogeneous and can range from a solitary lytic bone lesion with a favourable course to a fatal disseminated leukaemia-like form, with a wide spectrum of intermediate clinical presentations between these two extremes. Here, we present a case report of a solitary calvarial lesion in an adolescent boy along with a review of the literature. Presenting features, initial diagnostic evaluation and treatment protocol of a unifocal monosystemic calvarial location of LCH are presented.     

De Clérambault syndrome. A case report and a review of the literature on the difference between primary and secondary erotomania

This case report is about a female patient with an erotomanic delusion, who is convinced that a man is deeply in love with her. Because the specific course of primary erotomania and its response to treatment have been documented, the literature was studied to find out whether it distinguishes between primary and secondary erotomania. The search, however, showed that erotomania occurs so rarely and is defined in such ambiguous terms that it is impossible to divide these subtypes into two separate entities.     

Clinical course of spontaneous spinal epidural haematoma mimicking Guillain-Barré syndrome in a child: a case report and literature review

We describe a 9-year-old female with thoracic epidural haematoma. The clinical course simulated Guillain-Barré syndrome (GBS) so intravenous immunoglobulin therapy was started at the paediatric clinic. Magnetic resonance imaging (MRI) 3 days after admission showed thoracic epidural haematoma between T2 and T8. An emergency laminectomy was performed and the patient's neurological symptoms began to improve immediately after surgery and she made a full recovery during the 2 weeks of follow-up. Time is a very important factor in achieving reversibility of symptoms of compressive cord lesions, such as spinal epidural haematoma, and MRI is mandatory for patients with progressive paraplegia, even though the signs and symptoms might suggest GBS.     

Spontaneous spinal epidural haematoma mimicking meningitis in a 2-year-old child—a case report and literature review

Introduction

We report the case of a 2-year-old boy with suspected meningitis who presented with acute onset neck pain and stiffness associated with right-sided weakness and ataxia.

Management

Despite intravenous antibiotics and antiviral treatment, his condition deteriorated. Magnetic resonance imaging demonstrated spontaneous cervical epidural haematoma (C4–C7) extending down to thoracic (T7) level with associated compression of the spinal cord. He was treated successfully by neurosurgical decompression and made a complete recovery.

Discussion

Spinal epidural haematoma is a neurosurgical emergency characterised by extravasation of blood in the spinal epidural space. The clinical presentation particularly in young children can masquerade other conditions such as meningitis. In this article, we discuss our case and review the literature on spontaneous spinal epidural hematoma with an aim to improve awareness of this condition which if not recognised and treated early can lead to significant lifelong morbidity.     

DBS in tremor with dystonia: VIM,GPi or both? A review of the literature and considerations from a single-center experience

Journal of Neurology - Deep brain stimulation (DBS) is an established treatment for dystonia and tremor. However, there is no consensus about the best surgical targeting strategy in patients with...     

Does recurrent catatonia manifest in a similar fashion in all the episodes of mood disorder? A case series with literature review

Catatonia, originally conceptualised by Kahlbaum in 1868, is a neuropsychiatric condition that has been found to occur concomitantly with several organic and psychiatric conditions. Starting from the era of Kraepelin and Bleuler, this condition was faultily linked with schizophrenia alone; however, over time, greater associations have been found between catatonia and mood disorders. Despite the availability of several reports supporting this finding, there is a relative paucity of studies that specifically focus on catatonia to be the first symptom manifestation heralding a subsequent mood episode. In addition, there is scant literature to determine whether there are specific presentations of catatonia that show greater associations with mood disorders and whether these signs and symptoms recur in a stereotypical fashion in the subsequent mood episodes in the lifetime of an individual. We hereby report two cases with a diagnosis of mood disorders (bipolar disorder and recurrent depressive disorder) who had catatonia as the initial symptom not only at presentation but also at subsequent episodes. The report emphasises that recurrent catatonia can be the initial clinical manifestation of an underlying mood episode, which appears otherwise masked behind the catatonic presentation. These catatonic symptoms can be interestingly similar in all the subsequent episodes. A detailed clinical evaluation is thus warranted after catatonia has been duly treated to provide a holistic management.     

Neurological involvement in Behçet's disease. 154 cases from a cohort of 925 patients and review of the literature

INTRODUCTION: Neurological involvement is well described in Beh?et's Disease (BD), with variable prevalence of 5.3 to 30p.cent. The purpose of this retrospective study was to analyze the clinical patterns of neuro-Beh?et (NB) and to compare them with different clinical features of 925 BD registered in the same period and the literature. METHODS: All patients of NB fulfilled the International Study Group Criteria for the diagnosis of BD. 93 patients with headache, without other neurological symptoms were excluded. The findings were supported by cerebrospinal fluid, computed tomography scan, magnetic resonance imaging and angiography. RESULTS: NB was present in 16.64p.cent of BD. A sex ratio male/female: 4.31. The mean age of patients with NB was 31.76 years. The average age of onset of NB was 29.83. The mean duration of the NB disease was 3.81 years. The findings were categorised in 2 main types: parenchymal and non-parenchymal involvement. Some patients had features of both types (mixed patterns). 94 patients (61.03p.cent) presented with parenchymal central nervous system (CNS) involvement. The most common findings were pyramidal signs, cranial nerve palsies, pseudobulbar syndrome and cerebellar signs. 27 patients (17.53p.cent) without parenchymal CNS which were divided into: intracranial hypertension in 24 patients (15.58p.cent) presenting headache, vomiting and bilateral papilloedema; cerebro-arterial involvement in 3 cases (one of them had cerebrovascular aneurysms). Mixed patterns were observed in 9 cases. Erythema nodosum and vascular involvement were more frequent in intracranial hypertension than in BD. Other clinical features were reported: pure meningeal pattern in 15 cases, pure peripheral nervous system involvement in 4 cases, isolated cranial nerves in 8 cases, chorea in one patient and pseudotumor of cervical medulla in other patient. 6 cases of juvenile BD and one case of familial Beh?et were observed. 133 patients were treated (86.36p.cent). Mean duration of treatment was 7.46 month. We used corticosteroids, immunosuppressive agents (cyclophosphamid, azathioprine, chlorambucil) and anticoagulant in intracranial hypertension. The course of disease was good in only 54.13p.cent of cases, and was bad in 18.79p.cent. CONCLUSION: NB occurred frequently in men and is more serious especially in parenchymal CNS involvement: 7 deaths (6 from parenchymal CNS).     

Direct motor evoked potentials and cortical mapping using the NIM® nerve monitoring system: A technical note

Motor evoked potentials (MEPs) are commonly used to prevent neurological injury when operating in close proximity to the motor cortex or corticospinal pathway. We report a novel application of the NIM® nerve monitoring system (Medtronic@ NIM response 3.0) for intraoperative direct cortical (dc)-MEPs monitoring. A 69-year-old female patient presented with a 4 month history of progressive left hemiparesis resulting from a large right sided posterior frontal meningioma that abutted and compressed the motor cortex. Motor cortical mapping and MEPs were indicated. The patient was anesthetized and maintained on total intravenous anesthetics. Compound muscle action potentials (CMAP) of the right upper limb were monitored using the NIM system. After a craniotomy was performed, we first used the Ojemann stimulator (monopolar) for dc-stimulation and then switched to use the monopolar nerve stimulator probe of the NIM system. The CMAP response was successfully elicited using the NIM stimulating probe (pulse width = 250 s, train frequency = 7 pulses/s, current = 20 mA). A gross total resection of the tumor was achieved with intermittent cortical mapping of MEPs. There were no intraoperative complications and the patient’s motor function was preserved after the surgery. In this case, we reported the successful use of the NIM nerve monitoring system to elicit dc-MEPs under general anesthesia. The advantages of using this system include a simple set up and application, neurosurgeon familiarity, wide availability and lower cost. dc-MEPs can be achieved using the NIM system. We conclude that the NIM nerve monitoring system is a feasible alternative to standard neurophysiological monitoring systems.     

Is the EEG helpful in diagnosing and monitoring lithium intoxication? A case report and review of the literature

Lithium is potentially toxic to the central nervous system. Clinical lithium neurotoxicity may appear at any time during therapy and may go unrecognized. Failure to appreciate this fact leads to delays in diagnosis and treatment, placing the patient at risk of permanent neurological damage or death. In spite of a largely clinical diagnosis of lithium intoxication, the EEG provides an objective criterion of intoxication. We report a case of lithium intoxication with neurotoxic symptoms associated with marked EEG changes despite moderate lithium serum levels. In contrast to the interindividually varying EEG changes under uncomplicated lithium therapy, pathological EEG findings are the rule in the case of intoxication. Several reports evince a closer relationship between neurotoxic symptoms with EEG changes than with serum levels of lithium. This is of clinical interest with respect to intoxication under therapeutic lithium serum levels, since the EEG is the only examination indicating an intoxication. In patients with intoxication, the phenomenon of long-lasting EEG changes after discontinuation of lithium is discussed with respect to neuronal storing of lithium and persisting neurological disturbances.