Pilot therapeutic education program in multiple system atrophy: Safety, quality of life and satisfaction from a national registry based longitudinal study.

BACKGROUND: Therapeutic education programs are effective in several chronic conditions. However, evidence is lacking in multiple system atrophy (MSA). We aimed to assess efficacy and safety of a comprehensive therapeutic education program in people with MSA (PwMSA) and their caregivers. METHODS: In this prospective longitudinal study we included 16 PwMSA and their main caregivers in 4 groups of 4 dyads each. The program consisted of eight 60-min interdisciplinary sessions: introduction, orthostatic hypotension, speech therapy, gait and respiratory physiotherapy, psychological support, urinary dysfunction, occupational therapy/social work. UMSARS, NMSS, PDQ39, EQ5 and Zarit scales were administered at baseline and 6 months later. After each session participants filled-out a modified EduPark satisfaction questionnaire and a Likert scale. Educational material was generated for each session after suggestions by participants. RESULTS: At baseline PwMSA and caregivers were comparable in age and sex, with significant correlation between UMSARS-IV (disability) and PDQ39 (quality of life). Adherence to sessions was of 94,92 %. Total modified EduPark scores and Likert scales did not differ in PwMSA vs. caregivers, mild-moderate vs. severe-advanced cases or between genders. The significant difference in satisfaction across sessions (p = 0.03) was driven by higher scores in speech, respiratory and occupational therapy sessions. Longitudinally there was no significant worsening in any scale, nor a significant increase post-vs. pre-program in the number of consultations. CONCLUSIONS: The healthcare education program in MSA was feasible, satisfactory, and safe for patients and caregivers. The educational material of the program is being forwarded to incident MSA cases attending our clinic.

Neuropatía múltiple como manifestación clínica de una recidiva de leucemia / Isolated multiple neuropathy as clinical manifestation of leukemia relapse

Introducció La neuroleucemiosis es una rara enfermedad del sistema nervioso periférico producida por la infiltración por células leucémicas. Caso clínico: Presentamos el caso de una paciente de 34 años con antecedente de una leucemia mielomonoblástica aguda en remisión, que presentaba una parálisis progresiva del nervio mediano derecho, del facial bilateral y del peroneal izquierdo. El electromiograma confirmó el diagnóstico de una neuropatía múltiple. La tomografía por emisión de positrones-tomografía computarizada mostró un hipermetabolismo de ambos nervios ciáticos, facial bilateral y mediano derecho. La biopsia de médula ósea confirmó la recidiva de la leucemia, por lo que se inició un nuevo ciclo de quimioterapia con mejoría de los déficits neurológicos. Conclusión: La infiltración del sistema nervioso periférico por células leucémicas puede simular múltiples síndromes neurológicos dependiendo de las estructuras afectadas. La barrera hematonerviosa actúa como defensa de las células leucémicas contra la quimioterapia y el sistema inmunitario, por lo que el sistema nervioso periférico constituye un reservorio de las células leucémicas. Por ello, la neuroleucemia debe considerarse en pacientes con antecedentes de leucemia que presenten síntomas aislados de afectación del sistema nervioso periférico.(AU)

Introduction: Neuroleukemia is a rare disorder of the peripheral nervous system due to leukemic cell infiltration. Case report: We present the case of a 34-year-old patient with history of acute myelomonoblastic leukemia in remission that presented progressive paresis of the right median, bilateral facial, and left peroneal nerves. The electromyogram confirmed the diagnosis of multineuropathy. A PET-CT showed hypermetabolism of both sciatic, facial, and right median nerves. A bone marrow aspirate confirmed the leukemia relapse so a new round of chemotherapy was performed with improvement of the neurological deficit. Conclusion: Peripheral nervous system infiltration by leukemic cells can mimic multiple syndromes depending on the structures involved. The nerve-blood barrier acts as a defense of leukemic cells against chemotherapy and the immune system. Thus, the peripheral nervous system constitutes a reservoir of leukemic cells. Neuroleukemia should be considered in patients with history of acute leukemia who have isolated symptoms of the peripheral nerve.(AU)

Isolated multiple neuropathy as clinical manifestation of leukemia relapse. / Neuropatía múltiple como manifestación clínica de una recidiva de leucemia.

INTRODUCTION: Neuroleukemia is a rare disorder of the peripheral nervous system due to leukemic cell infiltration. CASE REPORT: We present the case of a 34-year-old patient with history of acute myelomonoblastic leukemia in remission that presented progressive paresis of the right median, bilateral facial, and left peroneal nerves. The electromyogram confirmed the diagnosis of multineuropathy. A PET-CT showed hypermetabolism of both sciatic, facial, and right median nerves. A bone marrow aspirate confirmed the leukemia relapse so a new round of chemotherapy was performed with improvement of the neurological deficit. CONCLUSION: Peripheral nervous system infiltration by leukemic cells can mimic multiple syndromes depending on the structures involved. The nerve-blood barrier acts as a defense of leukemic cells against chemotherapy and the immune system. Thus, the peripheral nervous system constitutes a reservoir of leukemic cells. Neuroleukemia should be considered in patients with history of acute leukemia who have isolated symptoms of the peripheral nerve.

TITLE: Neuropatía múltiple como manifestación clínica de una recidiva de leucemia.Introducción. La neuroleucemiosis es una rara enfermedad del sistema nervioso periférico producida por la infiltración por células leucémicas. Caso clínico. Presentamos el caso de una paciente de 34 años con antecedente de una leucemia mielomonoblástica aguda en remisión, que presentaba una parálisis progresiva del nervio mediano derecho, del facial bilateral y del peroneal izquierdo. El electromiograma confirmó el diagnóstico de una neuropatía múltiple. La tomografía por emisión de positrones-tomografía computarizada mostró un hipermetabolismo de ambos nervios ciáticos, facial bilateral y mediano derecho. La biopsia de médula ósea confirmó la recidiva de la leucemia, por lo que se inició un nuevo ciclo de quimioterapia con mejoría de los déficits neurológicos. Conclusión. La infiltración del sistema nervioso periférico por células leucémicas puede simular múltiples síndromes neurológicos dependiendo de las estructuras afectadas. La barrera hematonerviosa actúa como defensa de las células leucémicas contra la quimioterapia y el sistema inmunitario, por lo que el sistema nervioso periférico constituye un reservorio de las células leucémicas. Por ello, la neuroleucemia debe considerarse en pacientes con antecedentes de leucemia que presenten síntomas aislados de afectación del sistema nervioso periférico.

Colonic Oxidative and Mitochondrial Function in Parkinson's Disease and Idiopathic REM Sleep Behavior Disorder.

OBJECTIVE: To determine potential mitochondrial and oxidative alterations in colon biopsies from idiopathic REM sleep behavior disorder (iRBD) and Parkinson's disease (PD) subjects. METHODS: Colonic biopsies from 7 iRBD subjects, 9 subjects with clinically diagnosed PD, and 9 healthy controls were homogenized in 5% w/v mannitol. Citrate synthase (CS) and complex I (CI) were analyzed spectrophotometrically. Oxidative damage was assessed either by lipid peroxidation, through malondialdehyde and hydroxyalkenal content by spectrophotometry, or through antioxidant enzyme levels of superoxide dismutase-2 (SOD2), glutathione peroxidase-1 (Gpx1), and catalase (CAT) by western blot. The presence of mitochondrial DNA (mtDNA) deletions was assessed by long PCR and electrophoresis. RESULTS: Nonsignificant trends to CI decrease in both iRBD (45.69 ± 18.15; 23% decrease) and PD patients (37.57 ± 12.41; 37% decrease) were found compared to controls (59.51 ± 12.52, p: NS). Lipid peroxidation was maintained among groups (iRBD: 27.46 ± 3.04, PD: 37.2 ± 3.92, and controls: 31.71 ± 3.94; p: NS). Antioxidant enzymes SOD2 (iRBD: 2.30 ± 0.92, PD: 1.48 ± 0.39, and controls: 1.09 ± 0.318) and Gpx1 (iRBD 0.29 ± 0.12, PD: 0.56 ± 0.33, and controls: 0.38 ± 0.16) did not show significant differences between groups. CAT was only detected in 2 controls and 1 iRBD subject. One iRBD patient presented a single mtDNA deletion.

123I-MIBG cardiac uptake, smell identification and 123I-FP-CIT SPECT in the differential diagnosis between vascular parkinsonism and Parkinson's disease.

UNLABELLED: Vascular parkinsonism (VP) may occur as a distinct clinicopathological entity but the comorbid presence of vascular damage in Parkinson's disease (PD) is very frequent too. This differential diagnosis has therapeutic and prognostic implications but remains challenging as the usefulness of a number of supporting tools is still controversial. OBJECTIVE: To ascertain the clinical value of cardiac (123)I-meta-iodobenzylguanidine ((123)I-MIBG) SPECT, olfactory function and (123)I-FP-CIT SPECT as supporting tools in the differential diagnosis between VP and PD. METHODS: Cross-sectional study of 15 consecutive patients with suspected VP, 15 PD patients and 9 healthy subjects. Cardiac (123)I-MIBG SPECT (heart-to-mediastinum ratio) and olfactory testing (University of Pennsylvania Smell Identification Test-UPSIT) were performed in all of them. (123)I-FP-CIT SPECT was performed in VP-suspected patients. RESULTS: Heart-to-mediatinum ratio was significant lower in suspected VP (mean 1.45) and PD (mean 1.16) compared to control group (mean 1.69) (p = 0.017 and p < 0.0001). VP patients presented a higher ratio than PD patients (p = 0.001). Control group presented a significant higher UPSIT score (mean 30.71) when compared to both VP (mean 18.33) and PD (mean 15.29) (p = 0.001 for both groups). Those VP with a cardiac (123)I-MIBG non suggestive of PD were more likely to have a higher UPSIT score (p = 0.006). (123)I-FP-CIT SPECT imaging was heterogeneous (7/15 VP normal, 3/15 abnormal suggestive of PD and 5/15 abnormal but atypical for PD). CONCLUSIONS: The use of cardiac (123)I-MIBG SPECT and to a lesser extent UPSIT could assist the differential diagnosis between VP and PD in subjects in which the diagnosis remains uncertain despite (123)I-FP-CIT SPECT imaging.