Electroconvulsive therapy in Parkinson's syndrome with “on-off” phenomenon

Summary During long-term treatment with L-dopa in Parkinson's syndrome on-off phenomenon develops in many cases, often entailing considerable therapeutic problems. Decreased sensitivity in postsynaptic striatal dopamine (DA) receptors has been shown to occur in parkinsonian patients during long-term treatment with L-dopa. This has been suggested as one possible mechanism for development of the on-off phenomenon. In contrast to L-dopa treatment electroconvulsions have been shown to increase sensitivity in the DA receptors, when administered to animals.The antiparkinsonian effect of electroconvulsive therapy (ECT) was investigated in five parkinsonian patients with on-off phenomenon, with or without concomitant signs of mental depression. ECT was administered according to praxis in treatment of mental depression. Drug therapy, including L-dopa, was maintained on previously adjusted doses during and after ECT.A marked improvement in the parkinsonian symptoms as well as in the on-off phenomenon occurred in three of the patients, persisting for several months. The other two patients showed only slight and transient improvement.It thus seems that ECT may be useful as a supplementary treatment in parkinsonian patients with on-off phenomenon. The antiparkinsonian effect of ECT is probably mediated by increased sensitivity in postsynaptic DA structures.     

Electroconvulsive therapy against the patients’ will: A case series

Objectives: Electroconvulsive therapy (ECT) is the most effective therapy for severe depressive and psychotic disorders. As patients may be subject to legal incapacity and lack of insight, treatment may be administered against the patient’s will. There is only limited evidence on the use of ECT against the patient’s non-autonomous will.

Methods: We report a series of eight patients who received ECT against their will in two German university medical hospitals between 2014 and 2016. The effectiveness, tolerability and patients’ perspective are described.

Results: Seven of eight patients were much or very much improved according to the Clinical Global Impression Scale. Capacity to consent was restored in seven patients. Transient side effects occurred in four patients. Seven patients agreed to receive further ECT in the treatment course.

Conclusions: This case series suggests that ECT may (and sometimes should) be administered to severely ill, non-consenting patients against their will with good effectiveness. Potential and mostly moderate and transient side effects have to be weighed up against the potentially life-saving character of ECT in patients with, e.g., suicidality and food refusal. Most importantly, the patients’ retrospective appraisal also justifies the administration of ECT in situations of severe mental illness and legal incapacity.     

Inflammatory infiltrates in the spinal cord of patients with Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is defined as an acute inflammatory demyelinating polyradiculoneuropathy (AIDP) of the peripheral nervous system. Reports on central nervous system involvement in patients with GBS are rare and the histopathological analysis was usually restricted to conventional staining techniques. We were able to investigate four cases with GBS at autopsy in respect to the inflammatory infiltrates and histopathological changes in the spinal cord by immunohistochemistry using a panel of antibodies recognizing lymphocytes and different macrophage-activation antigens. There were increased inflammatory cell infiltrates comprising lymphocytes and macrophages in the spinal cord of two cases. In one of these two cases, GBS predominantly affecting the motor system similar to acute motor axonal neuropathy (AMAN) developed following hepatitis B vaccination; in the second one, GBS developed rapidly 4 days after onset of intravenous purified GM1-ganglioside application affecting the motor as well as the sensory system, resembling acute motor sensory axonal neuropathy (AMSAN). Impairment of the spinal anterior horn cells with their axons was suggested to be responsible for prolonged motor symptoms and the predominantly axonal type of neuropathy at least as a late-stage feature in these two cases with fatal outcome. Insignificant cellular infiltrates in the spinal cord were noted in the other two GBS cases. Focal cellular infiltration of spinal nerve roots and meninges was similar in all cases.     

Electroconvulsive therapy in patients taking steroid medication: should supplemental doses be given on the days of treatment?

Patients who take chronic steroid medication are often prescribed extra "stress doses" before procedures involving general anesthesia. The rationale for this practice is that the chronic steroid use has suppressed the ability of the endogenous hypothalamic-pituitary-adrenal steroid stress-reactivity system to handle the systemic stress of surgery. Whether the stress of treatments is sufficient enough to warrant this practice in electroconvulsive therapy (ECT) has not been broached in the literature. In this case series, we describe our experience treating 27 ECT patients taking prednisone. We conclude that use of "stress doses" of extra steroid medication is unnecessary in ECT practice and recommend that patients receive their usual morning dose of steroid before ECT treatments.     

Primary cytomegalovirus infection in patients with Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is frequently associated with the presence of CMV-specific IgM-antibodies or CMV-DNA in serum. Detection of IgM-antibodies or viremia may indicate primary infection, but also reactivation or reinfection. We identified 46 GBS patients with detectable CMV-specific IgM- or IgG-antibodies, or both. Sera from these patients were tested for the presence of CMV-specific, low-avidity IgG-antibodies, which indicate primary infection that occurred <6 months before sample collection, and for the presence of CMV-DNA by polymerase chain reaction (PCR). Primary infection was identified by the presence of low-avidity IgG-antibodies in 9/46 (20%) or by detection of IgM-antibodies in the absence of IgG-antibodies in 1/46 (2%) patients. CMV-DNA was detectable in 17/46 (37%) sera. In contrast, CMV-DNA was detected in only 2% of sera from 46 age-matched patients with neuroborreliosis. The likelihood of viremia decreased in GBS patients significantly with increasing antibody-avidity (P=0.041). Detection of IgM-antibodies correlated with that of CMV-DNA in patients with low-avidity IgG-antibodies (P=0.048) but not in those with high-avidity IgG-antibodies (P=0.543). In 45 age-matched healthy controls, low-avidity IgG-antibodies and CMV-DNA were detected in only 2% and 0% of sera, respectively. Our findings further strengthen evidence for an association between CMV infection and GBS. Primary CMV infection was identified in almost one-fourth of patients with detectable CMV-specific antibodies. Nevertheless, endogenous reactivation and reinfection have to be considered also as relevant events associated with GBS.     

Ketogenic diet in patients with Lennox–Gastaut syndrome

PurposeThe ketogenic diet (KD) has been used as an alternative to antiepileptic drugs (AEDs) for patients with refractory epilepsy. Lennox–Gastaut syndrome (LGS) belongs to the group of epileptic encephalopathies that often prove refractory to AED treatment.In this prospective study we assess the efficacy and tolerability of the KD in patients with LGS.MethodsBetween March 1, 1990 and April 1, 2013, 61 patients who met diagnostic criteria of LGS were seen at our department. Twenty of them were placed on the KD and followed for a minimum of 16 months.ResultsThe children had previously received a mean of 6.5 different AEDs and were on a mean of 2.5 AEDs when the diet was started. Eighteen months after initiating the diet, fifteen of the initial patients (75%) remained on the diet; three patients (15%) were seizure free, three (15%) had a 75–99% decrease in seizures, two (10%) had a 50–74% decrease in seizures, and the remaining seven children (35%) had a <50% decrease in seizures. Three seizure-free patients were tapered off the diet after remaining seizure free. In the three patients who had a 75–99% decrease in seizures AEDs were reduced.ConclusionThe KD is an effective and well-tolerated treatment option for patients with LGS, not only for those with cryptogenic, but also for those with structural LGS. The diet should be considered early in the course of this syndrome.     

Cognitive evaluation in adult patients with Möbius syndrome

Abstract Objective To establish the occurrence of mental retardation in a group of patients with Möbius syndrome and subsequently, if mental retardation is absent, to screen major aspects of memory and attention, in order to assess possible pervasive dysfunction in these cognitive domains which might be responsible for the current view that mental retardation occurs frequently in Möbius syndrome. Methods In a group of 12 Dutch Möbius patients, intellectual performance, memory function and attention were assessed using a number of standardized neuropsychological tests. Results The mean general intellectual performance did not differ significantly from that of the Dutch population. Screening of selective attention and memory did not provide indications of pervasive dysfunctions in these domains. Conclusion The rate of occurrence of mental retardation in our group of Möbius patients did not differ from that in the normal Dutch population. Furthermore, there was no evidence of attention and memory dysfunction in our group of Möbius patients.     

Factors predicting extubation success in patients with Guillain-Barré syndrome

Introduction  

Accurate prediction of successful extubation in patients with Guillain-Barré syndrome (GBS) is an important clinical problem. We hypothesized that reversal of clinical indices used to intubate a patient (i.e., declining vital capacity [VC]) predict extubation.     

Interleukin-10 promoter polymorphisms in patients with Guillain-Barré syndrome

Interleukin-10 (IL-10) may have both pro- and anti-inflammatory effects in Guillain-Barré syndrome (GBS). The distribution of polymorphisms in the IL-10 gene (-1082 (G/A), -819 (T/C) and -592 (A/C)) was analysed to determine whether they could influence disease susceptibility or clinical course in GBS. The -592 CC and -819 CC genotypes associated with increased IL-10 response were more frequent in the GBS patients than in the controls (P=0.027), but the polymorphisms did not influence the clinical course of the disease.     

The clinical and biochemical investigation of L-deprenyl in Parkinson's disease with special reference to the “on-off” effect

Summary The effect of L-deprenyl in combination with L-Dopa was studied in 22 Parkinsonian patients, with various forms of on-off phenomena. L-deprenyl is a selective, irreversible inhibitor of monoamine oxidase type B, without tyramine potentiating property. The results were very encouraging with regard to amelioration of on-off effect. More than 80% of the patients with nocturnal akinesia, early morning akinesia, end-of-dose akinesia and non-dose-dependent akinesia, showed improvement. In 9 out of 22 patients, the off period disappeared. The platelet monoamine oxidase activity, which is of type B and similar to the brain enzyme, was fully inhibited by the daily dose of L-deprenyl. There was no significant change in serum prolactin level during the duration of 6 weeks' treatment with L-deprenyl.     

Long–term intrathecal baclofen therapy in ambulatory patients with spasticity

Background Intrathecal baclofen (ITB) is an effective treatment for severe spasticity. In ambulatory patients with spasticity, it is possible that ITB treatment may compromise ambulatory function by unmasking underlying muscle weakness. This retrospective study is designed to determine the long–term ambulatory function in patients receiving ITB. Methods Thirty–six patients with severe spasticity previously screened for response to ITB were implanted with programmable pumps that allowed for continuous infusion of ITB. Patients were followed after implantation from 1 to 13 years. Results All 36 patients had decreased spasticity and retained ambulatory function. Three of the 36 patients eventually became paraplegic related to underlying disease progression. Conclusion ITB therapy may be used in selected ambulatory patients with spasticity and is not associated with loss of ambulatory function.     

Abnormalities of neuromuscular transmission in patients with Miller–Fisher syndrome

The mechanism of motor weakness in patients with Miller–Fisher syndrome (MFS) remains to be fully elucidated. We performed stimulated single fibre electromyography (sSFEMG) in a clinically weak frontalis muscle in a patient with MFS. Stimulate single fiber EMG revealed increased jitter in over 50% of the apparent single fibre action potentials from the frontalis muscle in addition to increased mean jitter. The findings in the present study suggest dysfunction of neuromuscular transmission in patients with MFS.     

Metabolic syndrome in antipsychotic naïve patients diagnosed with schizophrenia

Aim: The article aims to study the prevalence of metabolic syndrome (MS) and subthreshold MS in antipsychotic naïve patients with schizophrenia. Materials and methods: Forty‐six antipsychotic naïve patients diagnosed with schizophrenia were evaluated for the presence of metabolic abnormalities using International Diabetes Federation and modified National Cholesterol Education Program – Third Adult Treatment Panel criteria. Results: Five patients (10.86%) fulfilled International Diabetes Federation criteria for MS and six patients (13.04%) met modified National Cholesterol Education Program – Third Adult Treatment Panel criteria for MS. Additionally, 14 (30.43%) more patients fulfilled 2 out of the 5 criteria for MS and another 19 (41.3%) fulfilled 1 criterion for MS. Of the 19 patients who fulfilled one criterion for MS, 18 had an abnormality other than increase in waist circumference. Conclusion: Findings of the present study suggest that although only few antipsychotic naïve patients diagnosed with schizophrenia have MS, a significantly large proportion of patients have subsyndromal MS. Awareness of this in clinicians can have implications in the selection of antipsychotic medication.     

Psychotic symptoms and emotional distress in patients with Guillain-Barré syndrome

Psychological disturbances in 49 most severely compromised Guillain-Barré syndrome patients were prospectively studied by a semistructured interview and assessed by repeat psychiatric examination during the patients' stay in the neuro-intensive care unit (ICU). Additional information was obtained from attending physicians, nurses and relatives. Anxiety (82%), acute stress disorder, depressive episodes (67%) and brief reactive psychosis (25%) were observed, with oneiroid psychosis (14%) among the latter. Psychotic episodes were strongly associated (p < 0.001) with severe tetraparesis, artificial ventilation and multiple cranial nerve dysfunction. CSF protein concentration was also correlated with the occurrence of psychotic symptoms. Patients themselves experienced loss of communication to be the most difficult condition to cope with. Fifty-five percent explicitly felt reassured by the environment of the ICU and 90% described contact with relatives to be most helpful. Our data suggest that motor deprivation and loss of communication are the conditions most closely connected with the occurrence of psychotic symptoms. Therapeutically, continuous psychosocial support and psychopharmacological measures may both be valuable tools to ameliorate distress.