Congenital Dislocation of the Patella - Surgical Treatment Rationale and Literature Review.

INTRODUCTION: Congenital dislocation of the patella (CDP) is a rare condition and its treatment is not well defined. In CDP, patella is dislocated on the lateral aspect of the distal femur, laterally from the trochlear groove, it cannot be reduced manually and it is almost always associated with genu valgum, lateral torsion of the proximal tibia, and flexion contracture of the knee. This condition is present at birth, but the clinical findings can be subtle at birth due to the size and character of the structures being examined; early diagnosis is essential, but it may be delayed to late childhood or adulthood. If CDP is not promptly treated, the disability increases during growth, so surgical correction should be planned as soon as the diagnosis is confirmed. CASE REPORT: We report a case of bilateral CDP associated with bilateral proximal radioulnar joint stiffness; the patient at the age of diagnosis was 11 years old and underwent a bilateral delayed surgical procedure; the 4-year follow-up results are satisfactory. Surgical treatment rationale and literature are reviewed. CONCLUSION: Surgical correction for CDP is generally recommended, but there is no agreement in literature on the ideal treatment and in some cases, with mild impairment of the knee function, observation alone has been suggested. The decision about a surgical treatment can be difficult and depends on the degree of disability of the patient.

Meditation training for people with amyotrophic lateral sclerosis and their caregivers.

OBJECTIVES: Amyotrophic lateral sclerosis (ALS) is a progressive and fatal neurodegenerative disease that is clinically characterized by progressive weakness leading to death by respiratory insufficiency, usually within three years. Although the patient's intellect and personality usually remain unimpaired, as the disease progresses, the patient becomes immobile, develops wasting, and speech becomes impaired, often resulting in social isolation and a high degree of psychological suffering. Mindfulness meditation has proven to be effective technique for reducing distress in many chronic diseases. However, to date, no study has investigated the effect of mindfulness meditation on patients with ALS. DESIGN: A mindfulness meditation training program for ALS patients needs to consider the particularities of ALS symptoms, including the loss of muscular functions and difficulties in respiration, together with the subsequent emotional impairments. With these caveats in mind, a modified protocol, based on original mindfulness meditation interventions, has been created specifically for the ALS population. This article describes the protocol and preliminary results.

Aggressiveness, sexuality, and obsessiveness in late stages of ALS patients and their effects on caregivers.

Clinical experience has shown an increase of behavioural and mood symptoms, especially in the areas of aggressiveness, sexuality and obsessiveness, during the late stages of ALS. The lack of conclusive data concerning these symptoms prompted us to assess the psychological aspects of ALS patients in advanced stages of the disease. Moreover, we evaluated the personality of their caregivers in order to analyse the relationship between the pair. For these purposes, we studied 10 patients with ALS in late stages (tracheostomized for 36 months) and their caregivers using a questionnaire specifically elaborated for patients' communication limits. To assess the state of anxiety and depression of both patients and caregivers, we used the Hospital Anxiety and Depression Scale (HADS). To investigate caregivers' personality, we administered the Big Five Questionnaire (BFQ). Data showed a trend of aggression and high level of obsessiveness in ALS patients, associated with several clinical characteristics. High levels of anxiety emerged in both patients and caregivers. Regarding BFQ, caregivers obtained higher scores in the dimension of Conscientiousness and very low scores in Extraversion and Emotional Stability. In conclusion, the study showed a potential and considerable effect of the long duration of ALS on patients' personality and caregivers' distress.

Respiratory function of people with amyotrophic lateral sclerosis and caregiver distress level: a correlational study.

BACKGROUND: Amyotrophic Lateral Sclerosis (ALS) is a rare, fatal neurodegenerative disorder with no curative treatment characterized by degeneration of motor neurons involving a progressive impairment of motor and respiratory functions. Most patients die of ventilator respiratory failure. Caregivers have a great influence on the patient"s quality of life as well as on the quality of care. Home influence of the caregiver on patient care is notable. To date, no study has investigated how psychological issues of caregivers would influence respiratory variables of ALS patients. The study aimed at finding out if there is a relationship between the respiratory function of ALS patients and the level of distress of their caregivers. METHODS: A cross-sectional study was conducted to investigate respiratory issues (PCF and FVC) and the perception of social support of ALS patients. Caregivers filled questionnaires about trait anxiety, depression, and burden of care. Forty ALS patients and their caregivers were recruited. RESULTS: FVC and PCF were positively related to patient perception of social support and negatively related to caregiver anxiety, depression, and burden. DISCUSSION: The distress of ALS caregivers is related to patient respiratory issues. The first and more intuitive explanation emphasizes the impact that the patient's clinical condition has with respect to the caregiver. However, it is possible to hypothesize that if caregivers feel psychologically better, their patient's quality of life improves and that a condition of greater well-being and relaxation could also increase ventilatory capacity. Furthermore, care management could be carried out more easily by caregivers who pay more attention to the patient's respiratory needs. CONCLUSION: Patient perception of social support and caregiver distress are related to respiratory issues in ALS.

Pain in Amyotrophic Lateral Sclerosis: a psychological perspective.

Pain in Amyotrophic Lateral Sclerosis is often underestimated and untreated by clinicians and few studies have investigated its specific features and impact. Pain experience was investigated with the Italian Questionnaire of Pain, together with the McGill Quality of Life Questionnaire for quality of life (QoL), at a baseline and at a 4-month follow-up. About half of ALS patients reported pain, described as nagging, sore, annoying, boring and exhausting, with periodic but enduring episodes. Pain was related with QoL and its intensity was able to predict QoL worsening. Obtained results indicate the importance of clinical investigation of pain in ALS patients and of the intervention with anti-pain treatment whenever necessary.