ABSTRACT
The authors report the case of a 69-year-old woman suffering from paroxysmal hemicrania (PH), intolerant to indomethacin and resistant to multiple therapies, in which sphenopalatine endoscopic ganglion block (SPG) dramatically modified the clinical outcome. SPG blockade could be considered a reasonable alternative in drug-resistant PH cases where indomethacin is contraindicated.
Subject(s)
Anesthetics, Local/therapeutic use , Autonomic Nerve Block/methods , Facial Neuralgia/drug therapy , Paroxysmal Hemicrania/drug therapy , Aged , Drug Resistance , Endoscopy , Female , Humans , Pterygopalatine FossaSubject(s)
Basilar Artery/diagnostic imaging , Foramen Ovale, Patent/diagnostic imaging , Middle Cerebral Artery/diagnostic imaging , Ultrasonography, Doppler, Transcranial , Vertebral Artery/diagnostic imaging , Adult , Aged , Basilar Artery/physiopathology , Cerebrovascular Circulation , Female , Femoral Artery/diagnostic imaging , Femoral Artery/physiopathology , Foramen Ovale, Patent/physiopathology , Humans , Male , Middle Aged , Middle Cerebral Artery/physiopathology , Regional Blood Flow , Vertebral Artery/physiopathologyABSTRACT
Hemicrania continua (HC) is an indomethacin-responsive headache characterized by a chronic, strictly unilateral, side-locked without side-shifting, persistent headache. We report three cases of HC with atypical features in which an acute administration of indomethacin 50 mg IM (INDOTEST) was performed. In all three cases INDOTEST predicted chronic responsiveness to indomethacin. Thus, in cases of HC with atypical features, INDOTEST could help for a correct diagnosis and therapy.
Subject(s)
Headache/diagnosis , Headache/drug therapy , Indomethacin/administration & dosage , Adult , Aged , Chronic Disease , Double-Blind Method , Female , Humans , Predictive Value of TestsSubject(s)
Brain Diseases/complications , Brain Diseases/pathology , Hypertensive Encephalopathy/complications , Postpartum Period , Adult , Antihypertensive Agents/therapeutic use , Brain Diseases/drug therapy , Female , Humans , Hypertensive Encephalopathy/drug therapy , Hypertensive Encephalopathy/pathology , Labetalol/therapeutic use , Magnetic Resonance Imaging , Syndrome , Tomography, X-Ray ComputedSubject(s)
Arm/physiopathology , Cyclosporine/adverse effects , Immunosuppressive Agents/adverse effects , Motor Cortex/drug effects , Paralysis/chemically induced , Adult , Arm/innervation , Bone Marrow Transplantation , Brain Edema/chemically induced , Brain Edema/pathology , Brain Edema/physiopathology , Female , Functional Laterality , Graft Rejection/drug therapy , Graft Rejection/immunology , Humans , Leukemia/therapy , Magnetic Resonance Imaging , Motor Cortex/pathology , Motor Cortex/physiopathology , Paralysis/pathology , Paralysis/physiopathology , Recovery of Function , Withholding TreatmentSubject(s)
Endometriosis/complications , Pupil/physiology , Tonic Pupil/immunology , Adult , Anisocoria/diagnosis , Anisocoria/immunology , Anisocoria/physiopathology , Autoantibodies/immunology , Endometriosis/diagnosis , Endometriosis/physiopathology , Female , Humans , Iris/innervation , Iris/physiopathology , Ovarian Neoplasms/complications , Ovarian Neoplasms/diagnosis , Ovarian Neoplasms/surgery , Paraneoplastic Syndromes/diagnosis , Paraneoplastic Syndromes/immunology , Paraneoplastic Syndromes/physiopathology , Progesterone/therapeutic use , Tomography, X-Ray Computed , Tonic Pupil/diagnosis , Tonic Pupil/physiopathology , Treatment OutcomeABSTRACT
Miller Fisher syndrome is an autoimmune neuropathy characterised by ataxia, areflexia and ophthalmoplegia, with minimal if any limb weakness, and in the majority of cases by high titres of IgG anti-GQ1b ganglioside antibodies. In vitro electrophysiological experiments have demonstrated that these antibodies induce a transmission blockade at neuromuscular junction either pre- or post-synaptically. We report the case of a 63-year-old man with MFS that shows blood serum negative for anti-GQ1b but presents an impairment of neuromuscular transmission detected by single fibre electromyography. To the best of our knowledge, this represents the first case in the literature using jitter technique and suggests that other antibodies may be involved in the function of motor end plates by bindings to the synaptic membranes.
Subject(s)
Miller Fisher Syndrome/physiopathology , Muscle, Skeletal/physiopathology , Neuromuscular Junction/physiopathology , Peripheral Nerves/physiopathology , Electric Stimulation/methods , Electromyography/methods , Evoked Potentials, Motor/physiology , Evoked Potentials, Motor/radiation effects , Gangliosides/immunology , Humans , Immunoglobulin G/blood , Male , Middle Aged , Miller Fisher Syndrome/blood , Muscle, Skeletal/radiation effects , Neural Conduction/physiology , Peripheral Nerves/radiation effectsABSTRACT
BACKGROUND: Hereditary inclusion body myopathy (h-IBM) is an autosomal-recessive or autosomal-dominant hereditary disease characterized by peculiar findings in muscle biopsies which resemble those occurring in inclusion body myositis (IBM). The absence of an inflammatory infiltrate in myofibers in h-IBM is a relevant differential criterion between the two pathologies. Motor neuron diseases (MND) represent a group of disorders involving both upper and lower motor neurons, characterized by fasciculations, progressive muscle weakness, and muscle atrophy. The most common form and prototype of MND is the amyotrophic lateral sclerosis (ALS) or Charcot's Disease, a progressive and fatal neurodegenerative disorder occurring in late adulthood. The pathogenesis of ALS remains still unknown, a variety of hypotheses having been proposed to account for the disease. The association of both pathologies is not common and offers new hypotheses about the pathogenic mechanisms in skeletal muscle and nervous system degeneration. PATIENTS AND METHODS: Described are three case reports in which we observed the clinical, laboratory and histopathological association of IBM and MND. In one case, dementia was also present. Muscle data was obtained by muscle biopsies and immunohistochemistry, while diagnosis of MND was supported by common neurophysiological techniques. RESULTS: The accumulation ofphosphorylated neurofilaments with a hereditary IBM-like pattern in skeletal muscle fibers without accumulation of amyloid-beta protein was observed. CONCLUSIONS: A better knowledge of the mechanisms in cellular death cascade could explain the pathogenesis of these different degenerative disorders.