ABSTRACT
An asymptomatic child with antenatally diagnosed globoid cell leukodystrophy was found to have electrodiagnostic evidence of peripheral neuropathy at 7 weeks of age. The earliest prior such evidence in the literature was in a 6-month-old-child. Thus, peripheral neuropathy can be both the initial manifestation and a very early finding in Krabbe's disease.
Subject(s)
Leukodystrophy, Globoid Cell/diagnosis , Peripheral Nervous System Diseases/diagnosis , Electrodiagnosis , Female , Humans , Infant , Pregnancy , Prenatal DiagnosisABSTRACT
A male patient who is now 15 years old has experienced a seizure disorder since age 9 years. The seizures were expressed as episodes of excruciating pain localized to the genital region. Appropriate anticonvulsant medication has controlled both pain and seizures.
Subject(s)
Epilepsies, Partial/complications , Pain/etiology , Testicular Diseases/etiology , Cerebral Cortex/physiopathology , Child , Electroencephalography , Epilepsies, Partial/physiopathology , Evoked Potentials , Humans , MaleABSTRACT
Menstrually related temporary remissions of myasthenic symptoms are reported to occur in 25 to 50% of female patients. Even though this has been attributed to hormonal changes associated with the menstrual cycle the underlying mechanism of this hormonal influence has remained elusive. The present study demonstrated a cyclical variation in the activity of red cell acetylcholinesterase (EC 3.1.1.7) enzyme (AChE) with a marked reduction at the time of menstrual remission of symptoms of myasthenia. These cyclical changes were abolished by thymectomy. It appears, therefore, that menstrual remission in myasthenia is at least partly due to hormone-induced changes in AChE activity. This process seems to be under the control of the thymus gland.
Subject(s)
Acetylcholinesterase/metabolism , Erythrocytes/enzymology , Menstruation , Myasthenia Gravis/enzymology , Adolescent , Adult , Autoantibodies , Estrogens/blood , Female , Humans , Middle Aged , Myasthenia Gravis/blood , Myasthenia Gravis/immunology , Neuromuscular Junction/enzymology , Progesterone/blood , Remission, Spontaneous , ThymectomySubject(s)
Nervous System Diseases/etiology , Nervous System Physiological Phenomena , Thiamine Deficiency/complications , Thiamine/physiology , Action Potentials , Animals , Brain Diseases/etiology , Central Nervous System/pathology , Central Nervous System/physiopathology , Humans , Membranes , Nervous System Diseases/physiopathology , Peripheral Nerves/pathology , Permeability , Polyneuropathies/etiology , Wernicke Encephalopathy/metabolismABSTRACT
Five patients developed posttraumatic vascular headaches associated with autonomic dysfunction. The precipitating injury affected the anterior triangle of the neck, presumably involving the region of the carotid artery sheath. Disturbance of sympathetic function, characterized by excessive sweating and pupillary dilation associated with headache, was noted. Appropriate pharmacologic studies revealed evidence of partial sympathetic devervation. While headache was resistant to ergotamine preparations, prompt relief was obtained with propranolol hydrochloride, and adrenergic beta-receptor blocking agent.
Subject(s)
Autonomic Nervous System , Headache/etiology , Neck Injuries , Wounds, Nonpenetrating/complications , Adult , Carotid Artery Injuries , Female , Headache/drug therapy , Humans , Male , Middle Aged , Nervous System Diseases/drug therapy , Nervous System Diseases/etiology , Propranolol/therapeutic useSubject(s)
Brain/enzymology , Neurologic Manifestations , Thiamine Deficiency/enzymology , Transketolase/metabolism , Animals , Body Weight , Brain/growth & development , Brain/metabolism , Brain/pathology , Brain Stem/enzymology , Cerebellum/enzymology , DNA/metabolism , Diencephalon/enzymology , Female , Maternal-Fetal Exchange , Organ Size , Pregnancy , RNA/metabolism , Rats , Rats, Inbred Strains , Sciatic Nerve/enzymology , Thiamine Deficiency/diagnosis , Thiamine Deficiency/metabolism , Thiamine Deficiency/pathologySubject(s)
Cerebellar Ataxia/etiology , Chickenpox/complications , Acute Disease , Age Factors , Cerebellar Ataxia/microbiology , Child , Humans , MaleSubject(s)
Nervous System Diseases/metabolism , Erythrocytes/enzymology , Glycerophosphates , Heptoses , Humans , Peripheral Nerves/enzymology , Peripheral Nervous System Diseases/enzymology , Peripheral Nervous System Diseases/metabolism , Thiamine/metabolism , Transferases/metabolism , Uremia/enzymology , Uremia/metabolismSubject(s)
Brain Diseases/diagnosis , Cryptococcosis/diagnosis , Granuloma/diagnosis , Adult , Brain/pathology , Brain Diseases/diagnostic imaging , Brain Diseases/pathology , Cerebral Angiography , Cerebral Ventriculography , Cryptococcosis/complications , Cryptococcosis/diagnostic imaging , Cryptococcosis/pathology , Granuloma/diagnostic imaging , Granuloma/etiology , Granuloma/pathology , Humans , MaleABSTRACT
Spinal epidural abscess accompanies blood-borne infection, vertebral osteomyelitis, or an overlying cutaneous source of infection. This report documents the development of non-infective epidural abscess where the inflammatory response was induced by the highly irritant contents (keratin and cholesterol) of an underlying epidermoid. This was associated with aseptic meningitis.
