ABSTRACT
Neurilemmomas of the tongue have only been sporadically reported in the literature. Vague symptoms and limited knowledge of this kind of tumor may result in long delays before diagnosis and treatment. The rarity of the lesion in this area is the main reason why we report this case of neurilemmoma. After observation of a mass in the tongue, magnetic resonance imaging showed a large tumor with central necrosis localized to the base of the tongue. Needle electromyography disclosed no nerve lesion. Fine-needle aspiration cytology was negative, whereas biopsy revealed a neurilemmoma. The tumor was surgically removed, and histopathology, including immunohistochemistry, of the resected specimen disclosed the typical neurilemmoma. The postoperative course was uneventful.
Subject(s)
Neurilemmoma/diagnosis , Tongue Neoplasms/diagnosis , Adult , Biopsy , Biopsy, Needle , Cell Nucleus/ultrastructure , Connective Tissue/pathology , Contrast Media , Electromyography/methods , Humans , Immunohistochemistry , Magnetic Resonance Imaging , Male , Neurilemmoma/pathology , S100 Proteins/analysis , Tongue Neoplasms/pathologyABSTRACT
We describe 36 patients (six were apparently sporadic cases and 30 were cases from nine families) with amyotrophic lateral sclerosis (ALS) characterized by a distinct phenotype associated with homozygosity for an Asp90Ala mutation in the CuZn-superoxide dismutase gene. The presenting motor manifestation in all patients was paresis in the legs, with slow progression to the upper extremities and finally to the bulbar muscles. The age of ALS onset varied from 20 to 94 years, with a mean of 44 years. Mean survival time was 13 years for the 11 deceased patients. However, this is probably biased and untypical (low) when compared with the disease duration in the surviving patients, and when considering other medical complications in the deceased patients. The rate of progression was highly variable, even within families. All patients showed signs of involvement of both upper and lower motor neurons. Other neurological features included painful muscle spasms and paraesthesiae in the lower extremities. Two-thirds of patients experienced difficulty with micturition. Electrophysiological studies confirmed the slow progression and spatial distribution of clinical symptoms in the peripheral motor system. Furthermore, [corrected] potentials evoked by transcranial magnetic stimulation (MEP) were compared with those evoked by cervical or lumbosacral electrical stimulation and often revealed marked slowing of transmission in central motor pathways. In Sweden and Finland ALS patients homozygous for the Asp90Ala mutation constitute a phenotypically characteristic subset of motor neuron disease.
Subject(s)
Amyotrophic Lateral Sclerosis/genetics , Superoxide Dismutase/genetics , Adult , Age Distribution , Aged , Amyotrophic Lateral Sclerosis/physiopathology , Female , Humans , Male , Middle Aged , Motor Neuron Disease/genetics , PrognosisABSTRACT
In a population-based prospective study of epileptic seizures in adult s aged > 17 years, we identified 563 patients with possible seizures in a period of 34 months. Seizures were unprovoked in 160 patients, an incidence of 56 in 100,000 person-years. There was no difference in incidence between sexes. Age-specific incidences of unprovoked seizures increased sharply in men from age 60 years and in women from age 70 years. The incidence of unprovoked seizures in those aged > 65 years was 139 (men 166, women 116). The cumulative incidence of unprovoked seizures between the ages of 17 and 84 years was 4.6%. The proportion with an identified presumptive cause for unprovoked seizures increased with advancing age. A presumed etiology was identified in 77% of persons aged > 60 years. Stroke was the most common etiology, detected in 30% (incidence 16) and in 45% at ages > 60 years. Tumors were detected in 11% (incidence 6) and Alzheimer's disease was detected in 7% (incidence 4). Eighteen percent of patients were demented. Unprovoked seizures were partial in 68% of cases (incidence 38), and generalized in 16% (incidence 9). Another 13% of patients had generalized seizures, but seizure onset was not witnessed (incidence 7). In 16%, there was a delay of > 1 year from the first unprovoked seizure to initial diagnosis.
