ABSTRACT
A 69-year-old white man was admitted because of a clinical history of persistent cough and fever. Chest x-rays showed bilateral lung infiltrates with air bronchograms, whereas the urine antigen test resulted positive for Legionella pneumophila. The next day, he was transferred to the intensive care unit and intubated because of severe renal and respiratory distress. Neurological examination revealed distal weakness and loss of deep tendon reflexes in lower extremities. Nerve conduction studies displayed severe demyelinating sensorimotor polyneuropathy, and plasmapheresis was therefore applied with mild improvement. Few weeks after, dysphagia occurred and electrophysiologic tests showed progressive axonal involvement with spread of demyelination to the cranial nerves. The patient underwent a new plasmapheresis course and slowly reached stable clinical improvement of neurological status, which allowed him to be safely discharged. This case showed a critical onset with respiratory failure and kidney functional impairment due to L. pneumophila, subsequently disclosing Guillain-Barré syndrome.
Subject(s)
Guillain-Barre Syndrome/etiology , Legionnaires' Disease/complications , Respiratory Insufficiency/etiology , Aged , Guillain-Barre Syndrome/therapy , Humans , Male , PlasmapheresisABSTRACT
We report a case of midbrain malformation characterized by right deviation of the medulla oblongata associated with elongation and ectasia of the basilar and left vertebral arteries in a patient with a long history of migraine-like headache with autonomic symptoms.
Subject(s)
Autonomic Nervous System Diseases/etiology , Brain Diseases/complications , Mesencephalon/pathology , Migraine Disorders/etiology , Aged , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Female , Humans , Magnetic Resonance Imaging , Migraine Disorders/drug therapyABSTRACT
Migraine is a neurovascular disorder which affects one fifth of the general population. Disability due to migraine is severe and involves patients from infancy through senescence and it is aggravated by the fact there is no complete cure. However, various drugs for the symptomatic or prophylactic treatment of the disease are available. Recently, better knowledge of the neurobiological and pharmacological aspects of a subset of trigeminal primary sensory neurons has provided key information for the development of effective molecules that specifically target the activation of the trigeminovascular system and may represent a significant advancement in the treatment of the disease. These novel antagonists block the receptor for the sensory neuropeptide calcitonin gene-related peptide (CGRP), which upon release from peripheral terminals of trigeminal perivascular neurons dilates cranial arterial vessels. Whether neurogenic vasodilatation is the major contributing factor to generate the pain and the associated symptoms of the migraine attack or whether other sites of action of CGRP receptor antagonists are responsible for the antimigraine effect of these compounds is the subject of current and intense research.
Subject(s)
Calcitonin Gene-Related Peptide/metabolism , Migraine Disorders/metabolism , Pain/etiology , Receptors, Calcitonin Gene-Related Peptide/metabolism , Sensory Receptor Cells/metabolism , Trigeminal Nerve/metabolism , Afferent Pathways/metabolism , Afferent Pathways/physiopathology , Analgesics/therapeutic use , Animals , Calcitonin Gene-Related Peptide Receptor Antagonists , Cerebrovascular Circulation , Efferent Pathways/metabolism , Efferent Pathways/physiopathology , Humans , Migraine Disorders/complications , Migraine Disorders/drug therapy , Migraine Disorders/physiopathology , Neurogenic Inflammation/metabolism , Neurogenic Inflammation/physiopathology , Pain/drug therapy , Pain/metabolism , Pain/physiopathology , Sensory Receptor Cells/drug effects , Trigeminal Nerve/drug effects , Trigeminal Nerve/physiopathology , VasodilationABSTRACT
Pallidal stimulation is a convincing and valid alternative for primary generalized dystonia refractory to medical therapy or botulinum toxin. However, the clinical outcome reported in literature is variable most likely because of heterogeneity DBS techniques employed and /or to clinical dystonic pattern of the patients who undergo surgery. In this study, we report the long term follow up of a homogeneous group of eleven subjects affected by segmental dystonia who were treated with bilateral stimulation of the Globus Pallidus pars interna (GPi) from the years 2000 to 2008. All the patients were evaluated, before surgery and at 6-12-24-36 months after the treatment, in accordance with the Burke Fahn Marsden Dystonia Rating Scale (BFMDRS). Our study indicates that DBS promotes an early and significant improvement at 6 months with an even and a better outcome later on. The analysis of specific sub items of the BFMDRS revealed an earlier and striking benefit not only as far as segmental motor function of the limbs but also for the complex cranial functions like face, (eyes and mouth), speech and swallowing, differently from results reported in primary generalized dystonia. Deep Brain Stimulation of GPi should be considered a valid indication for both generalized and segmental dystonia when other therapies appear ineffective.
Subject(s)
Deep Brain Stimulation/methods , Dystonic Disorders/therapy , Globus Pallidus/physiology , Adult , Disability Evaluation , Dystonic Disorders/genetics , Dystonic Disorders/immunology , Dystonic Disorders/physiopathology , Female , Functional Laterality , Humans , Longitudinal Studies , Magnetic Resonance Imaging/methods , Male , Middle Aged , Molecular Chaperones/genetics , Outcome Assessment, Health Care , Retrospective Studies , Severity of Illness Index , Time FactorsABSTRACT
Calcitonin gene related peptide (CGRP) has been proposed to contribute to pain transmission and inflammation and for these reasons to the mechanism of migraine. CGRP is, in fact, expressed in and released from a subset of polymodal primary sensory neurons of the trigeminal ganglion. Release of CGRP in the dorsal spinal cord has been associated to nociceptive transmission, and release from perivascular nerve endings causes neurogenic vasodilatation. CGRP levels increase in the cranial circulation during migraine attacks, and GRP injection in migraineurs results in migraine-like attacks. Most importantly, two chemically unrelated CGRP-receptor antagonists, the parenteral agent, olcegepant, and the orally available telcagepant demonstrated efficacy in the treatment of migraine attacks, thus supporting CGRP as an important mediator in migraine.