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Showing papers on "Amyotrophic lateral sclerosis published in 1972"


Journal ArticleDOI
TL;DR: An intensive search for amyotrophic lateral sclerosis in Mexicans revealed only 16 patients diagnosed between 1962 and 1969 in theISSSTE government health program, confirming reports of a low ALS mortality in Mexico and among Mexican-Americans in the United States based on death certificates.
Abstract: An intensive search for amyotrophic lateral sclerosis (ALS) in Mexicans revealed only 16 patients diagnosed between 1962 and 1969 in the Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado (ISSSTE) government health program. The prevalence was 0.8 and the average annual incidence and mortality were 0.40 and 0.28/100,000 population, respectively. These low rates confirm reports of a low ALS mortality in Mexico and among Mexican-Americans in the United States based on death certificates. Pathologically, neurofibrillary and granulovacuolar changes were not seen and no familial cases were observed. Clinically, the cases resembled sporadic ALS. Just as there are populations among whom ALS is unusually common (eg, Guamanian Chamorros), there may be others like the Mexicans where ALS is rare. An explanation of population differences in ALS frequency may provide clues to the cause.

62 citations



Journal ArticleDOI
TL;DR: A 32-year-old Japanese patient with amyotrophic lateral sclerosis (ALS) had schizophrenic symptoms antedating the neurological picture, and the similarities to Alzheimer's disease and to the ALS-parkinsonism-dementia complex cases on Guam is of striking interest.
Abstract: A 32-year-old Japanese patient with amyotrophic lateral sclerosis (ALS) had schizophrenic symptoms antedating the neurological picture. Neuropathologically, there was a wide distribution of Alzheimer's neurofibrillary change, mainly in the cerebral cortex but less in the basal ganglia, in addition to the classical changes of ALS. This patient came from one of the foci of ALS located in the Kii Peninsula of Japan. The similarities to Alzheimer's disease and to the ALS-parkinsonism-dementia complex cases on Guam is of striking interest.

29 citations


Journal ArticleDOI
TL;DR: Further findings concerning serum CPK activity in cases of motor neuron disease and attempts to correlate the observed enzyme activity with the clinical features of the disease process are presented.
Abstract: T h e e n z y m e A T P c r e a t i n e phosphotransferase is present in high concentration in normal human striated muscle. The presence of raised levels of this enzyme in serum has proved to be a useful indication in the diagnosis of certain muscle disorders. The diagnostic value of the estimation of such levels in the Duchenne type of progressive muscular dystro~ h y l ~ and in the detection of carriers of this has been established. Such estimations have also been used to differentiate between the Duchenne type of dystrophy and other dystrophic states in which levels are raised t o a lesser degree and, further, t o differentiate these forms of primary muscle disease from neurogenic muscle wasting.8 39 Reports of raised serum CPK activity in neurogenic muscle wasting are rare but increasingly more prevalent in recent years.'''-'* In a r e c e n t s t u d y of chronic spinal muscular atrophy, Mastaglia and Walton have found raised levels of serum creatine kinase that corresponded to the degree of myopathic change found in the muscles.' In one series of estimations in motor neuron disease (MND), elevated levels of serum CPK were found in over 50% of the cases. ' This paper presents further findings concerning serum CPK activity in cases of motor neuron disease and attempts t o correlate the observed enzyme activity with the clinical features of the disease process. The validity of CPK estimations in the differential diagnosis of primary muscle disease and muscle disease of neurogenic origin is reassessed.

27 citations


Journal ArticleDOI
TL;DR: It is of interest that the type 2 strain of HVH is markedly more neurotropic in laboratory animals than the type 1 strain, which is usually responsible for vulvovaginitis, penile lesions, and most cases of herpesvirus infection in the adult.
Abstract: Herpesvirus hominis (HVH, herpes simplex) is presently recognized as an etiologic agent in acute disease of the central nervous system (CNS), causing encephalitis and aseptic meningitis in all age groups. Several authors have also suggested the possibility that HVH may play a role in subacute or chronic diseases of the CNS in man. Herpesvirus hominis has been shown to persist as a subclinical infection in the CNS of rabbits following intracerebral inoculation of virus in previously immunized animals1 Encephalitis or paralysis can be precipitated in such animals by administering epinephrine weeks to months later in asymptomatic animals. 1 , 2 Herpes encephalitis has been reported in man in cases in which the patient had either coexistent (apparently recurrent) mouth lesions3 or a history of previous HVH infection, thus presenting the possibility of reactivation of an occult CNS i n f e ~ t i o n . ~ It is now established that at least two strains of HVH exist which differ considerably in their antigenic and biological proper tie^.^ The type 1 HVH strain produces most cases of herpetic gingivostomatitis, recurrent herpes labialis, and herpes encephalitis in the adult. The type 2 strain of HVH is usually responsible for vulvovaginitis, penile lesions, and most cases of herpesvirus infection in the n e w b ~ r n . ~ It is of interest that the type 2 strain of HVH is markedly more neurotropic in laboratory animals than the type 1 strain.2,6 However, all human cases of herpes encephalitis beyond the newborn period in which the virus strain has been determined have been associated with the type 1 strain.’ In 1964, Gudnadottir et a18 reported the isolation of a herpesvirus (“MS”) from the brain of a patient with multiple sclerosis (MS). This virus was subsequently shown to be a type 2 strain of HVH.5 Neutralization studies of sera from 53 Icelandic multiple sclerosis patients revealed that 96% had antibodies to this virus whereas less than 70% of control sera neutralized the virus.8 Subsequently, Plummer and Hackett6 found that sera from 8 patients with multiple sclerosis neutralized a type 1 strain (“L-2”) better than MS and that 2 patients had no antibodies to either type. Plummer et al. also examined 9 sera from patients with amyotrophic lateral sclerosis (ALS) and found neutralizing antibody levels to HVH that were fourfold to eightfold higher than those levels found in any of the 50 control sera from a similar age group.g These elevated antibody levels were against the type 1 strain.1° Other surveys of sera and cerebrospinal fluid for elevated complement-fixing (CF) antibody titers to herpesvirus revealed no differences between multiple sclerosis patients and controls. -1

25 citations





Journal ArticleDOI
TL;DR: The case described adds to the concept of motor neurone disease as a syndrome rather than a disease entity of undetermined aetiology.
Abstract: A case is described in which the clinical and pathological features of amyotrophic lateral sclerosis (ALS) occurred in a patient with cysticercosis of the central nervous system and associated pachymeningitis of the cervical spinal cord. This is an extremely rare finding. The patient also presented the two symptom complexes more commonly encountered in this condition: focal and generalized epilepsy, and signs of obstructive hydrocephalus. The literature relevant to this case, and to cysticercosis in Great Britain, is briefly reviewed. The case described adds to the concept of motor neurone disease as a syndrome rather than a disease entity of undetermined aetiology.

18 citations