Incidence, risk factors, and outcomes related with neurological events after liver transplantation in adult and pediatric recipients.

Controversy exists whether NE after LT are more frequently observed in children or adults. We aimed to compare the incidence and outcomes for NE after LT in pediatric and adult recipients. A single-center cohort study, including all LT between 2001 and 2013, was performed.

Progressive external ophthalmoplegia (PEO) due to a mutation in the C10orf2 (PEO1) gene mimicking a myasthenic crisis.

We described a case of a patient with autosomal dominant progressive external ophthalmoplegia (PEO) who presented with the acute onset dysphagia, quadriparesis, ptosis and respiratory insufficiency following a cardiac procedure and mimicking a myasthenic crisis. A pathogenic mutation in the C10orf2 (PEO1) gene was confirmed. The unusual presentation of our patient contributes to expand the clinical phenotype of PEO1 mutations and reinforces the need to consider mitochondrial myopathy as differential diagnosis of myasthenia gravis even in the case of acute onset symptoms.

Hereditary myopathy with early respiratory failure: occurrence in various populations.

Objective: Several families with characteristic features of hereditary myopathy with early respiratory failure (HMERF) have remained without genetic cause. This international study was initiated to clarify epidemiology and the genetic underlying cause in these families, and to characterise the phenotype in our large cohort.

Methods: DNA samples of all currently known families with HMERF without molecular genetic cause were obtained from 12 families in seven different countries.

[Neuropsychological study of 12 patients with pure degenerative cerebellar disease].

Introduction: The cerebellum has been traditionally associated with motor control learning and performance. However, since 1970 a growing body of clinical and experimental evidences has suggested that the cerebellum may be involved in nonmotor cognitive functions as well.

Objective: To explore the presence of eventual cognitive impairment in non-demented patients with isolated degenerative cerebellar diseases.

Undulating toe movements in brain death.

For many years, death implied immobility. Nevertheless, there are anecdotal reports of spontaneous or reflex movements (SRMs) in patients with Brain death (BD). The presence of some movements can preclude the diagnosis of BD, and consequently, the possibility of organ donation for transplantation.

Improvement of blepharospasm with Zolpidem.

Zolpidem (ZLP) is an imidazopyridine that binds to GABA receptors. We report on improvement of blepharospasm in 3 patients treated with ZLP. The GABAergic action of this drug on the output structures of the basal ganglia could explain the improvement of blepharospasm in these patients.

Spontaneous and reflex movements in brain death.

Spontaneous and reflex movements may be found in patients with brain death (BD). The authors prospectively evaluated their frequency using a standardized protocol. Among 38 patients who fulfilled criteria for BD, the authors found 15 (39%) with spontaneous or reflex movements.

Catalepsy after stroke.

Catalepsy denotes a tendency to maintain postures. We prospectively looked for catalepsy in 216 acute stroke patients. We found five patients with cataleptic postures.

Reliability of the NINDS Myotatic Reflex Scale.

The assessment of deep tendon reflexes is useful for localization and diagnosis of neurologic disorders, but only a few studies have evaluated their reliability. We assessed the reliability of four neurologists, instructed in two different countries, in using the National Institute of Neurological Disorders and Stroke (NINDS) Myotatic Reflex Scale. To evaluate the role of training in using the scale, the neurologists randomly and blindly evaluated a total of 80 patients, 40 before and 40 after a training session.

The effect of levodopa on the habituation of the acoustic-palpebral reflex in Parkinson's disease.

The aim of the present study was to assess the habituation of the blink responses evoked by repetitive auditory stimuli in patients with Parkinson's disease in different clinical states. We studied 28 parkinsonian patients. Eighteen patients without motor fluctuations were studied off and on levodopa, on two different sessions, one week apart.

[Hemichorea associated with cerebral toxoplasmosis and AIDS].

Only 12 AIDS cases with hemichorea were reported in the literature. We report the first case of hemichorea associated with AIDS and cerebral toxoplasmosis in our country. A 26-year-old man had 3 episodes of focal seizures on the left side with subsequent loss of consciousness.

Motor activation time in Parkinson's disease.

Motor activation time (MAT), considered in the present work as the actual intracerebral processing delay during a reaction time (RT) task, was assessed in 17 patients with Parkinson's disease (PD) and in 7 age-matched healthy volunteers. MAT was calculated by subtracting from the premotor RT the afferent and efferent times obtained by sensory and motor evoked potentials. When compared with healthy volunteers, patients exhibited significantly (p < 0.

Peripheral nervous system and spinal cord involvement in lymphoma.

Nine-hundred-eighty-nine patients with diagnosis of lymphoma were studied. Forty-six cases (4.6%) had compressions of the spinal cord or roots.

Craniocerebral involvement in lymphoma.

Nine-hundred-eighty-nine patients with lymphoma were studied. Fifty-three cases (5.3%) had lymphomatous craniocerebral infiltration.

In vivo 31P NMR profiles of Alzheimer's disease and multiple subcortical infarct dementia.

We used in vivo phosphorus 31 nuclear magnetic resonance (31P NMR) spectroscopy to study regional high-energy phosphate and phospholipid metabolism in brains of patients with dementia associated with probable Alzheimer's disease (AD) and multiple subcortical cerebral infarctions (MSID). The MSID patients demonstrated elevations of the phosphocreatine (PCr)/inorganic orthophosphate (Pi) ratio in both the temporoparietal and frontal regions. Phosphomonoesters (PME) and the ratio of PME to phosphodiesters were elevated in the temporoparietal region of AD.

Peripheral nerve involvement in Bell's palsy.

A group of patients with Bell's palsy were studied in order to disclose the presence of subclinical peripheral nerve involvement. 20 patients, 8 male and 12 female, with recent Bell's palsy as their unique disease were examined. In all cases other causes of polyneuropathy were ruled out.