ABSTRACT
The development of neurologic deficits confined to the corticospinal tracts has been referred as Primary Lateral Sclerosis (PLS). Through the years, this diagnosis has remained uncertain. In this study we describe seven patients with chronic involvement of the pyramidal system. Two of these patients had serologic evidence of human T-lymphotrophic virus type I infection, one patient had multiple sclerosis and in four patients the clinical diagnosis of PLS was made. The clinical characteristics and diagnostic studies of these patients are presented. A review of the literature with emphasis in the differential diagnosis and a proposed workup for patients with chronic spastic paraparesis are made. This study provides supporting evidence in favor of the clinical entity of PLS.
Subject(s)
Amyotrophic Lateral Sclerosis/diagnosis , Paraparesis, Tropical Spastic/complications , Adult , Aged , Amyotrophic Lateral Sclerosis/complications , Chronic Disease , Diagnosis, Differential , Humans , Middle AgedABSTRACT
La aparición de signos neurológicos confinados al tracto piramidal se conoce como esclerosis lateral primaria. A través de los años, este diagnóstico ha sido cuestionado. En este estudio se describen siete pacientes con daño crónico del sistema piramidal. Dos de estos pacientes tenían evidencia serológica de infección con el virus humano T-linfotrópico tipo I. Un paciente tenía esclerosis múltiple y en cuatro pacientes el diagnóstico de esclerosis lateral primaria fue hecho. Las características clínicas y estudios diagnósticos de estos pacientes son presentados, incluyendo una revisión de la literatura con énfasis en el diagnóstico diferencial de paraparesis espática crónica. Este estudio provee evidencia a favor del diagnóstico de esclerosis lateral primaria como una entidad clínica
Subject(s)
Humans , Adult , Middle Aged , Amyotrophic Lateral Sclerosis/diagnosis , Paraparesis, Tropical Spastic/complications , Chronic Disease , Diagnosis, Differential , Amyotrophic Lateral Sclerosis/complicationsABSTRACT
The clinical, laboratory and EEG findings of 4 uremic patients on hemodialysis who accidently developed acute hypercalcemia were reviewed. An acute central nervous system syndrome developed, associated with the clinical changes of disorientation, dysarthria, seizures, myoclonic jerks, hallucinations, irritability, confusion, memory and judgment defects plus bizarre behavior. The EEG findings demonstrated diffusely severe slow background activity in all tracings. In addition, the EEG abnormalities as well as the clinical findings disappeared when serum calcium returned to normal. Hypercalcemia, a reversible condition, seems to have been the cause of this clinical syndrome which should be differentiated from dialysis dementia, a condition known to be irreversible and fatal.
Subject(s)
Dementia/etiology , Hypercalcemia/etiology , Renal Dialysis/adverse effects , Acute Disease , Central Nervous System/physiopathology , Electroencephalography , Humans , Uremia/complications , Uremia/therapyABSTRACT
A study of nine patients, aged 23 to 67 years, showed a remarkable sequence of EEG findings in progressive uremic encephalopathy. The initial characteristics suggested a disorder of subcortical gray matter, followed by involvement of cortical gray matter and finally white matter. The same EEG findings tended to persist in the early stages of the disease and were present throughout the night and during dialysis. During dialysis, the EEG background and clinical picture between paroxysms sometimes showed deterioration. Seizures indicated a grave prognosis. Five of six patients with seizures died. In some patients, progressive uremic encephalopathy may develop without hemodialysis. Routine EEGs in dialysis units or in patients prior to admission can help uncover progressive uremic encephalopathy before the clinical emergence of this disorder.
Subject(s)
Brain Diseases, Metabolic/physiopathology , Electroencephalography , Uremia/complications , Brain Diseases, Metabolic/etiology , Humans , Male , Middle Aged , Renal DialysisABSTRACT
One hundred and eight patients with independent, multifocal, spike discharges in the EEG were studied. The abnormality occurred at all ages but was more frequent between 4 and 7 years. The basic EEG activity in wake tracing was invariably slow for the age of the patient. The majority of the patients with independent multifocal spike discharges had extensive bilateral cerebral lesions that occurred in the prenatal, perinatal, or postnatal periods. Clinical seizures were of different types, and patients had varying degrees of intellectural and neurologic deficits. A significant number had had previous tracings with either hypsarrhythmic or slow spike-wave patterns, suggesting a close relationship of these three EEG abnormalities.
