ABSTRACT
Forehead tremor has only been reported in two patients with essential tremor, one with rhythmic tremor and the other with dystonic tremor. We report 4 new patients with essential tremor who present a 4-6 Hz frontal tremor registered by electromyography and unusual features like frontal tremor preceding limb tremor or unilateral involvement. Frontal tremor is present in some patients with essential tremor, sometimes preceding limb tremor. Treatment with botulinum toxin may be useful.
ABSTRACT
Neuronal cell death by apoptosis is a mechanism involved in Parkinson's disease and indirect signs of apoptosis have been found in brain neurons and blood lymphocytes. The present study was aimed to directly assess the presence of enhanced apoptosis in lymphocytes from 89 idiopathic Parkinson's disease patients, 33 untreated and 56 treated, compared with 33 healthy individuals. The study of both spontaneous and activation-induced apoptosis of T-lymphocyte subsets by annexin-V binding and flow cytometry showed that Parkinson patients increased the expression of Fas in circulating CD4(+) T cells, mainly "naive," that correlated with the decrease of these cells in blood. Spontaneous and activation-induced apoptosis of CD4(+) T-cell subsets were also significantly increased. Thus, in Parkinson patients, peripheral blood CD4(+) T cells have an increased susceptibility to apoptosis with Fas involvement. This fact explains the decrease in the number of CD4(+) T-cell subsets observed in Parkinson and could be related to the neurodegenerative process.
Subject(s)
Apoptosis/immunology , CD4-Positive T-Lymphocytes/immunology , Nerve Degeneration/immunology , Nerve Degeneration/physiopathology , Parkinson Disease/immunology , Parkinson Disease/physiopathology , Aged , Annexin A5/metabolism , Binding, Competitive , Biomarkers/metabolism , CD4-Positive T-Lymphocytes/metabolism , Cells, Cultured , Cohort Studies , Female , Flow Cytometry , Humans , Lymphocyte Activation/physiology , Male , Middle Aged , Nerve Degeneration/metabolism , Neurons/immunology , Neurons/metabolism , Parkinson Disease/metabolism , fas Receptor/analysis , fas Receptor/metabolismABSTRACT
Recent studies have shown the co-existence of alpha-synuclein and phosphorylated tau (pTau) in several neurodegenerative diseases. Here, we report two autopsy cases of combined multiple system atrophy (MSA) and Alzheimer's disease (AD). In both cases, abundant alpha-synuclein-positive glial and neuronal cytoplasmic inclusions were found in the brainstem, amygdala and hippocampal formation. pTau-positive neurofibrillary tangles (NFTs) were widely distributed in case 1 (Braak stage VI) and moderate in case 2 (Braak stage III). Although alpha-synuclein and pTau pathology co-occurred in the hippocampus and entorhinal cortex, only a few neurons showed co-existence of these two proteins. Immunoreactivity for p62, a ubiquitin proteasome system related protein, was found in the majority of NFTs, but in only a small proportion of neuronal alpha-synuclein inclusions. In addition, UBB+1, a mutant form of ubiquitin and a marker for proteasomal dysfunction, was present in the majority of NFTs, whereas co-existence of alpha-synuclein and UBB+1 was found in only a few neurons. These findings indicate that alpha-synuclein and phosphorylated tau co-occur in certain brain regions in cases of combined MSA and AD and that the proteasomal pathways differ between alpha-synuclein- and pTau-bearing neurons.
