Encefalopatía posterior reversible como primera manifestación del Síndrome de Guillain-Barré / Posterior reversible encephalopathy as the first manifestation of Guillain-Barré syndrome. Report of one case

Background: We report a 56year old male hypertensive, who presented with a posterior reversible encephalopathy syndrome (PRES) as an initial manifestation of Guillain-Barré syndrome (GBS). His first symptoms were right hemiparesis and hemihypoesthesia, followed by headache, dizziness, dysarthria and a general feeling of discomfort. On the third day, flaccid tetraparesis, impairment of consciousness, epileptic seizures and respiratory failure appeared, along with severe hypertension. Cerebral Magnetic Resonance Imaging showed the characteristic PRES lesions. Cerebrospinal fluid analyses revealed albumin-cytological dissociation and nerve conduction studies showed an axonal demyelinating polyradiculoneuropathy, which confirmed the diagnosis of GBS. Treatment with intravenous immunoglobulin was given together with antihypertensive therapy and mechanical ventilation, achieving an important clinical and imaging remission of PRES, but maintaining tetraparesis during the hospitalization. Twelve months after discharge and regular motor rehabilitation, the patient achieved complete autonomy on the activities of daily living. It has been postulated that the autonomic failure and the elevation of circulating pro-inflammatory cytokines in GBS may be the cause of a breach in the blood-brain barrier, thus causing PRES, that can completely remit with an adequate management.

[Posterior reversible encephalopathy as the first manifestation of Guillain-Barré syndrome. Report of one case]. / Encefalopatía posterior reversible como primera manifestación del Síndrome de Guillain-Barré

BACKGROUND: We report a 56 year old male hypertensive, who presented with a posterior reversible encephalopathy syndrome (PRES) as an initial manifestation of Guillain-Barré syndrome (GBS). His first symptoms were right hemiparesis and hemihypoesthesia, followed by headache, dizziness, dysarthria and a general feeling of discomfort. On the third day, flaccid tetraparesis, impairment of consciousness, epileptic seizures and respiratory failure appeared, along with severe hypertension. Cerebral Magnetic Resonance Imaging showed the characteristic PRES lesions. Cerebrospinal fluid analyses revealed albumin-cytological dissociation and nerve conduction studies showed an axonal demyelinating polyradiculoneuropathy, which confirmed the diagnosis of GBS. Treatment with intravenous immunoglobulin was given together with antihypertensive therapy and mechanical ventilation, achieving an important clinical and imaging remission of PRES, but maintaining tetraparesis during the hospitalization. Twelve months after discharge and regular motor rehabilitation, the patient achieved complete autonomy on the activities of daily living. It has been postulated that the autonomic failure and the elevation of circulating pro-inflammatory cytokines in GBS may be the cause of a breach in the blood-brain barrier, thus causing PRES, that can completely remit with an adequate management.

[Frontotemporal dementia: Review of 63 cases]. / Demencia frontotemporal: Experiencia clínica.

BACKGROUND: Front temporal dementias (FTD) are neurodegenerative disorders characterized by alterations in behavior, affection and language, with relative sparing of episodic memory. There are three major forms of FTD: the frontal or behavioral form, progressive non-fluent aphasia and semantic dementia (that may begin as a fluent progressive aphasia). AIM: To report a retrospective clinical experience of patients with frontotemporal dementia. MATERIAL AND METHODS: Review of 3,700 records of neuropsychological assessments of patients with behavioral disturbances, studied between 1981 and 2008. Of these, 63 patients (59% females) complied with the criteria for frontotemporal dementia. RESULTS: There were 47 cases with the frontal variant, four with non-fluent progressive aphasia and six with fluent progressive aphasias (2 evolved to semantic dementia). The mean age of onset was 60+/-11 years. There were no familiar cases of FTD. CONCLUSIONS: It is clinically difficult to diagnose FTD, since evaluation of attitude or language is required. In addition to structural images, functional images were helpful in some cases, but the definitive diagnosis is anatomical.

Demencia frontotemporal: experiencia clínica / Frontotemporal dementia: review of 63 cases

Background: Front temporal dementias (FTD) are neurodegenerative disorders characterized by alterations in behavior, affection and language, with relative sparing of episodic memory. There are three major forms of FTD: the frontal or behavioral form, progressive non-fluent aphasia and semantic dementia (that may begin as a fluent progressive aphasia). Aim: To report a retrospective clinical experience of patients with frontotemporal dementia. Material and methods: Review of 3,700 records of neuropsychological assessments of patients with behavioral disturbances, studied between 1981 and 2008. Of these, 63 patients (59 percent females) complied with the criteria for frontotemporal dementia. Results: There were 47 cases with the frontal variant, four with non-fluent progressive aphasia and six with fluent progressive aphasias (2 evolved to semantic dementia). The mean age of onset was 60±11 years. There were no familiar cases of FTD. Conclusions: It is clinically difficult to diagnose FTD, since evaluation of attitude or language is required. In addition to structural images, functional images were helpful in some cases, but the definitive diagnosis is anatomical.