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Cannabinoids comprehend endocannabinoids, phytocannabinoids, and synthetic cannabinoids, with actions both in the central and peripherical nervous systems. A considerable amount of publications have been made in recent years, although cannabis has been known for over a thousand years. Scientific Departments from the Brazilian Academy of Neurology described evidence for medical use in their areas. Literature is constantly changing, and possible new evidence can emerge in the next days or months. Prescription of these substances must be discussed with patients and their families, with knowledge about adverse events and their efficacy.

Background: Specific comorbidities affect older patients with dementia admitted to general hospitals and may complicate the recognition of dementia. The aim of the present study was to estimate the prevalence of dementia among elderly inpatients admitted to hospital medical wards and to identify its distribution across clinical and sociodemographic conditions., Methods: From June 2011 to May 2012, a sample of elderly inpatients (≥60 years old) were screened for dementia with the Mini-Mental State Examination and the Bayer Activities of Daily Living Scale to identify cognitive and functional impairment (CFI). Subjects with CFI underwent a diagnostic procedure for dementia using the Cambridge Mental Disorders of the Elderly Examination and the Diagnostic and Statistical Manual of Mental Disorders, 4th edition. The elderly inpatients also completed a standard questionnaire to investigate sociodemographic and clinical variables and a screening procedure for depression and delirium. The data obtained were submitted to univariate and multivariate analyses., Results: The sample of 224 subjects had a mean age of 71.5 years and was mostly men (62.2%), poorly educated (≤4 years of schooling: 74.6%), and married (53.4%). CFI was observed in 84 subjects (prevalence: 37.4%; 95% confidence interval: 31.1-43.7), and dementia was observed in 31 subjects (prevalence: 17.2%; 95% confidence interval: 12.3-22.1). Dementia was related to older age and the presence of delirium, stroke, and pneumonia., Conclusions: The prevalence of CFI and dementia was high among the elderly inpatients examined. The identification of medical and sociodemographic conditions associated with a dementia diagnosis in a general hospital may be useful in the development of preventative actions., (© 2015 The Authors. Psychogeriatrics © 2015 Japanese Psychogeriatric Society.)

We report the case of a 3-(1/2)-year-old girl with hypotonia, multiple joint contractures, hip luxation, arachnodactyly, adducted thumbs, dolichostenomelia, and abnormal external ears suggesting the diagnosis of congenital contractural arachnodactyly (CCA). The serum muscle enzymes were normal and the needle electromyography showed active and chronic denervation. The muscle biopsy demonstrated active and chronic denervation compatible with spinal muscular atrophy. Analysis of exons 7 and 8 of survival motor neuron gene through polymerase chain reaction did not show deletions. Neurogenic muscular atrophy is a new abnormality associated with CCA, suggesting that CCA is clinically heterogeneous.

We present the case of a patient that after chronic use of anticonvulsant drugs without proven epilepsy, developed Dupuytren's and Ledderhose's diseases. We discuss the most frequent predisposing factors, and their relationship with chronic use of anticonvulsants, particularly phenobarbitone.

We describe a patient who had difficulty in walking since toddling stage and presented proximal upper and lower member weakness which have evolved to a progressive limitation of neck and trunk flexure, compatible with rigid spine syndrome. The serum muscle enzymes were somewhat elevated and the electromyography showed a myopatic change. The muscle biopsy demonstrated an active and chronic myopathy. The DNA analysis through PCR did not display any abnormality for dystrophin gene. The dystrophin by immunofluorescence was present in all fibers, but some interruptions were found in the plasma membrane giving it the appearance of a rosary. The test for merosin was normal.

Spinocerebellar ataxia type 1 (SCA1), spinocerebellar ataxia type 2 (SCA2) and Machado-Joseph disease or spinocerebellar ataxia type 3 (MJD/SCA3) are three distinctive forms of autosomal dominant spinocerebellar ataxia (SCA) caused by expansions of an unstable CAG repeat localized in the coding region of the causative genes. Another related disease, dentatorubropallidoluysian atrophy (DRPLA) is also caused by an unstable triplet repeat and can present as SCA in late onset patients. We investigated the frequency of the SCA1, SCA2, MJD/SCA3 and DRPLA mutations in 328 Brazilian patients with SCA, belonging to 90 unrelated families with various patterns of inheritance and originating in different geographic regions of Brazil. We found mutations in 35 families (39%), 32 of them with a clear autosomal dominant inheritance. The frequency of the SCA1 mutation was 3% of all patients; and 6% in the dominantly inherited SCAs. We identified the SCA2 mutation in 6% of all families and in 9% of the families with autosomal dominant inheritance. The MJD/SCA3 mutation was detected in 30% of all patients; and in the 44% of the dominantly inherited cases. We found no DRPLA mutation. In addition, we observed variability in the frequency of the different mutations according to geographic origin of the patients, which is probably related to the distinct colonization of different parts of Brazil. These results suggest that SCA may be occasionally caused by the SCA1 and SCA2 mutations in the Brazilian population, and that the MJD/SCA3 mutation is the most common cause of dominantly inherited SCA in Brazil.

Pure sensory neuropathies are clinically characterized by paresthesias, sensory ataxia and areflexia without muscle weakness. We report the case of a 21 years-old female patient with acute onset of distal paresthesias, marked sensory ataxia and hyporeflexia. Motor strength was normal. Sensory nerve conduction was absent and motor nerve conduction slightly decreased. CSF showed 2 leucocytes/mm3 and 1.06 g/dL protein. Collagen disorder and neoplasia were not found. Type 2 fiber atrophy was observed on muscle biopsy, and axonal demyelination on sural nerve biopsy. The patient was treated with prednisone. After 1.5 years she was recovered, but a minor proprioceptive deficit persisted. A revision is made on the etiology, pathophysiology and clinical manifestations of the disease.

Purpose of Review: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described treatable, inflammatory, brainstem predominant encephalomyelitis. The diagnosis of CLIPPERS is challenging without a specific biomarker, and thus it is important to consider if both the clinical and radiographic features are consistent with the diagnosis, or rather a disease mimicker. Recent Findings: Many patients with CLIPPERS-like lesions have been described in the literature with follow-up revealing a range of alternative diagnoses, such as malignancies, vasculitis, and other specific inflammatory diseases. As a result, some have proposed that CLIPPERS might represent a pre-malignancy state or simply an initial clinical syndrome of a variety of possible etiologies. Summary: We describe the typical clinical, radiographic, and pathological features of CLIPPERS and emphasize consideration for alternative diagnoses when findings are not classic. A recommended diagnostic evaluation and initial treatment plan is provided. [ABSTRACT FROM AUTHOR]

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids ( CLIPPERS) is an inflammatory disease in the central nervous system characterized by brainstem symptoms and distinctive radiological features, and responds favorably to steroid therapy. To date, only a few case reports pertaining to this condition have been published, and thus single-photon emission computed tomography findings of CLIPPERS have not been reported. Here, we document our experiences with an 86-year-old patient who we diagnosed with CLIPPERS according to her clinical presentation, radiological findings and favorable response to steroid therapy. N-isopropyl-p-[123I]iodoamphetamine single-photon emission computed tomography showed hypoperfusion in the pons, which was improved by steroid therapy. This case highlights the utility of N-isopropyl-p-[123I]iodoamphetamine single-photon emission computed tomography for assessing pontine dysfunction in CLIPPERS patients both before and after steroid therapy. [ABSTRACT FROM AUTHOR]

An author index for the "European Journal of Neurology," for the 2012 issue is presented.

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