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. McNair and Meador [(1992), Mov Dord7: 345-347] described the presence of undulating toe flexion movements (UTF) in BD patients. UTF consists in a sequential brief plantar flexion of the toes. Our aim was to determine the frequency, characteristics and predisposing factors of UTF movements in a prospective multicenter cohort study of patients with BD. Patients with confirmed diagnosis of BD were assessed to evaluate the presence of UTF using a standardized protocol. All patients had a routine laboratory evaluation, CT scan of the head, and EEG. Demographic, clinical, hemodynamic and blood gas concentration factors were analyzed. amongst 107 BD patients who fulfilled the AAN requirements, 47 patients (44%) had abnormal movements. UTF was observed in 25 (23%) being the most common movement (53%). Early evaluation (OR 4.3, CI95% 1.5-11.9) was a predictor of UTF in a multivariate regression model. The somato-sensory evoked potential (SSEPs) as well as brainstem auditory evoked potentials (BAEPs) did not elicit a cortical response in studied patients with UTF. This spinal reflex is probably integrated in the L5 and S1 segments of the spinal cord. Abnormal movements are common in BD, being present in more than 40% of individuals. UTF was the most common spinal reflex. In our sample, early evaluation was a predictor of UTF. Health care professionals, especially those involved in organ procurement for transplantation, must be aware of this sign. The presence of this motor phenomenon does not preclude the diagnosis of BD.

[Reversible posterior leucoencephalopathy syndrome: a case report and review of its physiopathology based on neuroradiological findings]. / Leucoencefalopatia posterior reversible: descripcion de un caso y revision de la fisiopatologia basada en hallazgos neurorradiologicos.

INTRODUCTION: Reversible posterior leucoencephalopathy is a clinico radiological syndrome first described in the last decade. The physiopathological mechanism governing it is not very well known. The currently accepted hypotheses are the generation of a vasogenic edema caused by failure of the mechanism that allows self regulation of the cerebral blood flow, and the production of a cytotoxic edema due to ischemia. Both experimental studies carried out in rats and the use of magnetic resonance imaging (MRI) to evaluate the diffusion of cerebral water support the first hypothesis. We report the case of a patient with reversible posterior leucoencephalopathy syndrome that was complicated by areas of cerebral infarction and we review the physiopathological mechanisms involved, basing our analysis on the findings obtained with MRI. CASE REPORT: A female patient was admitted to our hospital because of bilateral transient amaurosis, headaches, vomiting and arterial hypertension. MRI brain scans showed bilateral cortico subcortical lesions in the occipital parietal regions, frontal and right cerebellar hemisphere convexity, with hyperintense signal in T2 and diffusion weighted imaging. Some occipital parietal regions present a diminished signal on the apparent diffusion coefficient map. The patient was treated with intravenous antihypertensive drugs and full recovery was accomplished within 10 days. A month later, according to MRI scans, there were still hyperintense areas in T2 on both occipital lobes, which were compatible with cerebral infarcts. DISCUSSION: The mechanism that is most frequently involved in this entity is probably vasogenic edema. In our case there were, at the same time, areas of cytotoxic edema that progressed to cerebral infarcts. MRI with sequences that evaluate the diffusion of cerebral water is useful in distinguishing between the two mechanisms.

Variable patterns of anti-GM(1) IgM-antibody populations defined by affinity and fine specificity in patients with motor syndromes: evidence for their random origin.

Elevated titers of serum antibodies against GM(1)-ganglioside are associated with a variety of autoimmune neuropathies. Although much evidence indicates that these autoantibodies play a primary role in the disease processes, the mechanism of their appearance is unclear. Low-affinity anti-GM(1) antibodies of the IgM isotype are part of the normal human immunological repertoire. In patients with motor syndromes, we found that in addition to the usual anti-GM(1) antibodies, the sera contain IgM-antibodies that recognize GM(1) with higher affinity and/or different specificity. This latter type of antibodies was not detected in other autoimmune diseases. We studied the fine specificity of both normal and motor disease-associated antibodies using HPTLC-immunostaining of GM(1) and structurally related glycolipids, soluble antigen binding inhibition, and GM(1) affinity columns. Normal low-affinity anti-GM(1) antibodies cross-react with GA(1) and/or GD(1b). In the motor syndrome patients, different populations of antibodies characterized by their affinity and cross-reactivity were detected. Although one population is relatively common (low affinity, not cross-reacting with GA(1) and GD(1b)), there are remarkably few sera having the same set of populations. These results suggest that the appearance of the new antibody populations is a random process. When the different antibody populations were analyzed in relation to the three-dimensional structure of GM(1), a restricted area of the GM(1) oligosaccharide (the terminal Galbeta1-3GalNAc) was found to be involved in binding of normal anti-GM(1) antibodies. Patient antibodies recognize slightly different areas, including additional regions of the GM(1) molecule such as the NeuNAc residue. We hypothesize that disease-associated antibodies may originate by spontaneous mutation of normal occurring antibodies.

Clinical features and prognosis of nonepileptic seizures in a developing country.

PURPOSE: To determine the predictive value of clinical features and medical history in patients with nonepileptic seizures (NESs). METHODS: One hundred sixty-one consecutive ictal video-EEGs were reviewed, and 17 patients with 41 NESs identified. NES diagnosis was defined as paroxysmal behavioral changes suggestive of epileptic seizures recorded during video-EEC without any electrographic ictal activity. Clinical features, age, sex, coexisting epilepsy, associated psychiatric disorder, social and economic factors, delay in reaching the diagnosis of NES, previous treatment, and correlation with outcome on follow-up were examined. RESULTS: The study population included 70% female patients with a mean age of 33 years. Mean duration of NESs before diagnosis was 9 years. Forty-one percent had coexisting epilepsy. The most frequent NES clinical features were tonic-clonic mimicking movements and fear/anxiety/hyperventilation. The most common psychiatric diagnosis was conversion disorder and dependent and borderline personality disorder. Seventy-three percent of patients with pure NESs received antiepileptic drugs (AEDs), and 63.5% of this group received new AEDs. Fifty-nine percent of the patients received psychological/psychiatric therapy. At follow-up, 23.5% were free of NESs. CONCLUSIONS: All seizure-free patients had two good prognostic factors: having an independent lifestyle and the acceptance of the nonepileptic nature of the episodes. Video-EEG monitoring continues to be the diagnostic method to ensure accurate seizure classification. Establishing adequate health care programs to facilitate access to new technology in public hospitals as well as the implementation of continuous education programs for general practitioners and neurologists could eventually improve the diagnosis and treatment of patients with NESs.

Facial myokymia in brain death.

BACKGROUND: Brain death (BD) is the irreversible loss of all functions of the brain and brainstem. Spontaneous and reflex movements of the limbs have been described in this condition. However, facial myokymia (FM) in BD has not been previously reported. The origin of that motor phenomenon in alive patients is still uncertain, since supranuclear, nuclear and peripheral mechanisms have been proposed. OBJECTIVE: We describe the presence of FM in a patient who fulfilled the criteria for BD. A 40-year-old-man had right-sided weakness and impaired consciousness. After 14 h admission, he fulfilled the criteria for BD. A CT scan of the head showed a large putaminal hemorrhage. The EEG was isoelectric. At that time, fine spontaneous twitches of the left cheek were noticed. They consisted of repetitive and rhythmic movements in groups of 3-5 lasting for < 5 s. These movements appeared every 2-10 min during 6 h. DISCUSSION: Spinal reflexes have been described in BD. The presence of any movements other than the recognized reflexes may question this diagnosis and limit organ procurement for transplantation. The recognition of FM as an accepted movement in BD patients has practical and legal implications.

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. The most common movement was finger jerks. Undulating toe flexion sign, triple flexion response, Lazarus sign, pronation-extension reflex, and facial myokymia also were seen. These movements may be more common than reported and do not preclude the diagnosis of BD.