patients with DoC. The state of consciousness should be classified according to the highest level revealed by any of these three approaches.
RESULTS
Clinical examinationPICO questions 1-3 refer to clinical signs, PICO questions 4-8 to clinical rating scales. Thirteen
Sensory disturbances are part of the clinical picture of Parkinson's disease. Abnormalities in sensory processing, through a basal ganglia involvement, are thought to be responsible for the sensory dysfunction since sensory nerve conduction velocity (NCV) is usually normal. However, NCV does not examine small fibres or terminal endings of large sensory fibres, whereas skin biopsy is more suitable for these purposes. To evaluate peripheral sensory nerves in Parkinson's disease, we studied cutaneous free and encapsulated sensory nerve endings in 18 patients and 30 healthy controls using 3-mm punch biopsies from glabrous and hairy skin. Ten patients had additional skin biopsies from the contralateral side. Further evaluation included NCV and Quantitative Sensory Testing. Parkinson's disease patients showed a significant increase in tactile and thermal thresholds (P < 0.01), a significant reduction in mechanical pain perception (P < 0.01) and significant loss of epidermal nerve fibres (ENFs) and Meissner corpuscles (MCs) (P < 0.01). In patients with bilateral biopsies, loss of pain perception and ENFs was higher on the more affected side (P < 0.01). We found evidence suggesting attempts at counteracting degenerative processes as increased branching, sprouting of nerves and enlargement of the vascular bed. Morphological and functional findings did not correlate with age or disease duration. Disease severity correlated with loss of MCs and reduction in cold perception and pain perception. We demonstrated a peripheral deafferentation in Parkinson's disease that could play a major role in the pathogenesis of the sensory dysfunction.
This clinical study demonstrates that late recovery of responsiveness and consciousness is not exceptional in patients with traumatic and nontraumatic VS, although with residual severe disability.
Objective.This international multi-center, prospective, observational study aimed at identifying predictors of short-term clinical outcome in patients with prolonged Disorders of Consciousness (DoC) due to acquired severe brain injury.Methods.Patients in vegetative state/unresponsive wakefulness syndrome (VS/UWS) or in minimally conscious state (MCS) were enrolled within 3 months from their brain injury in 12 specialized medical institutions. Demographic, anamnestic, clinical and neurophysiological data were collected at study entry. Patients were then followed-up for assessing the primary outcome, i.e. clinical diagnosis according to standardized criteria at 6 months post-injury.Results.We enrolled 147 patients (44 women; mean age: 49.4 [95% confidence intervals: 46.1-52.6] years; VS/UWS= 71, MCS= 76; traumatic= 55, vascular= 56, anoxic= 36; mean time post-injury= 59.6 [55.4-63.6] days). The 6-month follow-up was complete for 143 patients (VS/UWS= 70; MCS= 73). With respect to study entry, the clinical diagnosis improved in 72 patients (VS/UWS= 27; MCS= 45). Younger age, shorter time post-injury, higher Coma Recovery Scale-Revised total score and presence of EEG reactivity to eye opening at study entry predicted better outcome, whereas etiology, clinical diagnosis, Disability Rating Scale score, EEG background activity, acoustic reactivity and P300 on event related potentials were not associated with outcome.Conclusions.Multimodal assessment could identify patients with higher likelihood of clinical improvement in order to help clinicians, families and funding sources with various aspects of decision-making. This multi-center, international study aims to stimulate further research that drives international consensus regarding standardization of prognostic procedures for patients with DoC.
Summary: Purpose: To describe the etiology, characteristics, and clinical evolution of epilepsy in patients with gelastic seizures (GSs).Methods: Nine patients whose seizures were characterized by typical laughing attacks were observed between 1986 and 1997. Patients were selected based on electroencephalogram (EEG) or video-EEG recordings of at least one GS and on magnetic resonance imaging (MRI) study.Results: Five patients were affected by symptomatic localization-related epilepsy (LRE), with four of the patients' disorders related to a hypothalamic hamartoma (HH) and one to tuberous sclerosis (TS) without evident hypothalamic lesions. In four patients (the cryptogenic cases) MRI was negative also in these cases, clinical and EEG data suggested a focal origin of the seizures. The epileptic syndrome in the HH cases was usually drug-resistant, and was surgically treated in two of the patients. The patient with TS became seizure free with vigabatrin. In the cryptogenic cases, the ictal, clinical, and EEG semiology were similar to the symptomatic cases; the clinical evolution was variable, with patients having transient drug resistance or partial response to treatment. No cognitive defects were observed in the cryptogenic patients. None of the nine patients had precocious puberty.Concfusions: We confirm the frequent finding of HHs in GSs and further underline how GSs may also be observed in patients without MRI lesions and with normal neurologic status. In these patients, clinical and EEG seizure semiology is similar to symptomatic cases, but the clinical evolution is usua l l y more benign.
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