Amyotrophic lateral sclerosis (ALS) is the most common adult-onset motor neuron disease. ALS is a progressive neurodegenerative disorder, involving motor neurons in the cerebral cortex, brainstem and spinal cord, presenting with a combination of upper and lower motor neuron signs. Etiology remains undetermined, although a multifactorial origin is widely accepted including genetic factors, auto-immunity, oxidative stress, glutamate excitotoxicity and abnormal neurofilament aggregation. The absence of specific diagnostic testing, and variable clinical presentations make the diagnosis of ALS challenging, relying upon correlation of clinical, electrophysiological and neuroimaging data. The disease is relentlessly progressive, with dysarthria, dysphagia, tetraparesis, and respiratory insufficiency due to ongoing respiratory muscle paresis. There is no specific treatment for ALS. Riluzole, a glutamate antagonist, is the only FDA approved drug for ALS, but has only a modest effect on survival. The multiplicity and progressiveness of the disabilities in ALS, highlights the need for a coordinated multidisciplinary rehabilitation program managing symptoms, respiratory care, dysphagia and nutrition, dysarthria and communication, physical and occupational therapy. The main goals are to prolong independence, prevent complications and improve quality of life.

Background. Few data have been published regarding long-term mortality in patients with Parkinson's disease treated with DBS. Methods. This study analyzed long-term mortality rates, causes, and correlates in PD patients treated with DBS. Results. 184 consecutive patients were included; mean follow-up was 50 months. Fifteen deaths occurred (total 8.15%, annual mortality rate 1.94%). Mean age at disease onset and at surgery was 48 ± 2.4 and 63 ± 1.6 years, respectively. Mean disease duration until death was 21 ± 7.8 years. Most deaths related to stroke, myocardial infarction, other vascular/heart disorders, or severe infection; one suicide was recorded. Deceased PD patients were mostly male and had lower motor benefit after DBS, but univariate analysis failed to show significant differences regarding gender and motor benefit. Survival was 99% and 94% at 3 and 5 years. Conclusions. Long-term survival is to be expected in PD patients treated with DBS, possibly higher than previously expected. Death usually supervenes due to vascular events or infection.

No abstract

BackgroundFungal brain abscess is an uncommon disease, mostly associated with immunocompromised states and poorly controlled diabetes. Its incidence, however, is rising as a result of the increasing use of immunosuppressive agents, corticosteroids and broad-spectrum antimicrobial therapy. Candida species have emerged as the most prevalent etiologic agents of brain abscesses in autopsy studies.Case presentationA 46-year-old male with a history of injection drug abuse, chronic hepatitis C and diabetes mellitus presented to the Emergency Department of our hospital following a generalized tonic-clonic seizure without recovery of mental status. On admission, the patient was in coma, febrile, with severe acidemia with respiratory and metabolic acidosis, requiring invasive mechanical ventilation. Brain imaging revealed multiple ring-enhancing lesions with oedema and mass effect. Microbiologic studies, including cerebrospinal fluid, blood, sputum and urine cultures, were all negative. A stereotactic brain biopsy was performed and culture of brain specimens revealed Candida albicans. The patient was successfully treated with fluconazole therapy for 48 weeks presenting a good clinical response and a complete radiological resolution of brain abscesses.ConclusionDespite advances in diagnostic and therapeutic procedures, fungal brain abscess remains a life-threatening disease with a poor outcome. Successful treatment requires an early diagnosis and usually a combined medical and surgical approach. A long-term antibiotic regimen is a cornerstone of fungal brain abscesses treatment, with the endpoint determined by clinical and neuroimaging response. The authors report an uncommon case of successfully treated Candida albicans brain abscesses with anti-fungal therapy consisting of fluconazole alone. This case illustrates the importance of early recognition of predisposing factors and multidisciplinary approach in timely therapeutic intervention, in order to prevent neurologic sequelae and improve the outcome of the patients with this severe and challenging form of central nervous system infection.

No abstract