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PubMed2001

Epidemiological survey of X-linked bulbar and spinal muscular atrophy, or Kennedy disease, in the province of Reggio Emilia, Italy.

Commencing with the work carried out during the epidemiological survey of amyotrophic lateral sclerosis in the period 1980-1992 and the pathology follow-up, we carried out a perspective incidence, prevalence and mortality survey of X-linked bulbar and spinal muscular atrophy (X-BSMA) in the province of Reggio Emilia in Northern Italy. Based on 11 patients (eight familial and three sporadic cases), the mean incidence per year for the period 1980 through 1997, as evaluated at the onset of symptoms, was 0.09 cases/100,000 for the total population and 0.19 cases/100,000 for the male population. On December 31, 1997, the prevalence rate was 1.6/100,000 for the total population and 3.3/100,000 for the male population. In the 18-year period of 1980-1997, the average yearly mortality rate was: 0.03 cases/100,000 per year for the total population and 0.06 cases/ 100,000 for the male population. The average age at onset was 44.8 +/- 10.1, and the average survival period was 27.3 +/- 2.3 years. The average age of the prevalence day was 58.9 +/- 14.9, and the average age at death was 71.3 +/- 4.7 years. Whereas the incidence rate of X-BSMA in the province of Reggio Emilia is 16 times lower that of amyotrophic lateral sclerosis (ALS), the incidence rate of progressive bulbar palsy in the male population is only slightly higher than X-BSMA; and the prevalence rate of ALS for males is two times the prevalence rate for X-BSMA, with overlapping of confidence intervals. X-BSMA is a rare disease, which is probably under-diagnosed, but due to the long survival period of this disease its frequency is not negligible. Because of the presence of sporadic cases or non-evident familial cases, it is appropriate to consider this diagnostic possibility in making a diagnosis of ALS in patients in whom lower motor neuron dysfunction or bulbar onset predominates.

Adult↗
PubMed1979

Amyotrophic lateral sclerosis with temporal lobe atrophy.

Clinical and neuropathological findings are reported on a 48-year-old man who developed progressive bulbar palsy, muscle atrophy of four extremities, and hyperreflexia. Duration of the illness was about 19 months. Moderate personality changes were observed during his hospitalization. Neuropathological examination revealed the presence of an ALS: severe loss of the large motoneurons in the spinal anterior horns, and degeneration of the corticospinal tract, more prominent on the left side. The hypoglossal, the facial, and the motor trigeminal nuclei were also involved, but the oculomotor, the trochlear and the abducens nuclei were well preserved. There was bilateral, but more pronounced on the right, atrophy of the temporal poles consistent with the lesions of the temporal types of Pick's disease. This case indicates the simultaneous occurrence of degenerative diseases of the CNS, and the correspondence of laterality between the temporal lobe and the spinal cord may suggest a common etiology of these two types of disease processes.

Amyotrophic Lateral Sclerosis↗
PubMed2006

Comparison of the growth hormone, IGF-1 and insulin in cerebrospinal fluid and serum between patients with motor neuron disease and healthy controls.

Neurotrophic effects of the growth hormone (GH), insulin-like growth factor-1 (IGF-1) and insulin on the central nervous system have become more apparent in the past decade. In this study, we measured serum and cerebrospinal fluid (CSF) concentrations of GH, IGF-1 and insulin in 35 patients with motor neuron disease (MND) [24 patients with definite amyotrophic lateral sclerosis (ALS) and 11 patients with progressive bulbar palsy] and in 40 healthy controls. Levels of serum concentrations of GH and IGF-1 did not significantly differ between the MND patient group and the healthy controls, while the level of insulin was significantly decreased (P = 0.0033) in the MND patient group. However, levels of all three examined parameters in CSF were significantly lower in the MND group than in the healthy controls with the statistical significance for IGF-1 and insulin of P < 0.001. This finding has not been reported previously, and further investigations into its association with ALS should establish whether it can be used as an early marker of the disease, or whether it merely represents a consequence of ALS development.

Biomarkers↗
PubMed2005

Clinical and epidemiological features of motor neuron disease in south-western Greece.

OBJECTIVE: To evaluate the epidemiological and clinical features of motor neuron disease (MND) in a region (835,000 inhabitants) of south-western Greece. PATIENTS AND METHODS: The medical records of all patients diagnosed with adult-onset MND at the Department of Neurology of the University Hospital of Patras from 1990 to 2003 were reviewed. RESULTS: Overall 133 patients were identified, corresponding to a mean annual incidence rate of 1.13/100,000 population with male preponderance. Eighty-five of them were males (63.9%) and 48 (36.1%) females with a mean age of 61.4 +/- 13.3 years. The most common type of MND was amyotrophic lateral sclerosis (ALS) being identified in 111 (83.5%) patients, whereas 19 cases (14.3%) were classified as progressive spinal muscular atrophy (PSMA) and three (2.2%) cases as progressive bulbar palsy (PBP). The mean age at onset was 60.3 +/- 13.5 years, while the mean delay between age at onset and age at diagnosis was 1.3 +/- 1.1 years. The symptoms at onset involved the lower limbs in 76 (57.2%) cases, upper limbs in 32 (24%) cases, bulbar region in 22 (16.5%) cases and respiratory muscles in three (2.3%) cases. The mean survival time after onset of disease was 20.4 +/- 8.3 months for ALS patients, 15.3 +/- 4.5 months for PBP and 38.1 +/- 26.4 months for PSMA patients. CONCLUSIONS: There was no statistically significant difference in the results of the considered epidemiological parameters of our study to those reported by other similar studies. The study of the patients with MND showed a predominance of ALS patients. No potentially causative clinical associations were found and no relation between socioeconomic factors, occupational exposure and the disease was noted.

Adolescent↗
PubMed2006

[Clinical characteristics of amyotrophic lateral sclerosis subsets].

Amyotrophic lateral sclerosis (ALS) is characterized by significant clinical variability. Different subsets are classically individualized: bulbar onset and limb onset ALS, sporadic and familial ALS, ALS-plus syndromes (characterized by the presence of atypical clinical features, e.g. extrapyramidal signs or dementia, in association with the classical phenotype of ALS) and Western Pacific ALS. In addition, ALS-related syndromes include progressive muscular atrophy, primary lateral sclerosis and progressive bulbar palsy. The recognition of ALS subsets and ALS-related syndromes is important in clinical practice since the prognosis may vary depending on the clinical presentation. The prognosis of bulbar-onset ALS is poor compared with the spinal-onset type. Primary lateral sclerosis, defined by pure upper motor neuron findings, has a more benign course than classical ALS. It has also important implications for therapeutic trials to ensure the homogeneity of clinical material since inclusion of atypical forms with different prognoses can skew the outcome analysis. This paper reviews the clinical characteristics of the ALS subsets and ALS-related syndromes.

Amyotrophic Lateral Sclerosis↗
PubMed2006

[Differential diagnosis and atypical subsets of amyotrophic lateral sclerosis].

Amyotrophic lateral sclerosis (ALS) is a progressive degeneration of upper and lower motor neurons. In the absence of any validated biological marker, the diagnosis of ALS depends upon recognition of characteristic symptoms and signs together with supportive electrophysiological findings. The diagnosis of ALS is easy to recognize in its fully developed form but during the early stages both false positive and false negative diagnoses are common. In clinical practice, diagnostic difficulties mostly arise with patients who present either with only upper motor neuron, or with only lower motor neuron signs. It may be difficult to distinguish ALS with clinically predominant lower motor neuron involvement from alternative diagnoses including spinal atrophies of adult onset, Kennedy's disease, inclusion body myositis and motor neuropathies with conduction blocks. The diagnosis of ALS related syndromes (progressive muscular atrophy, primary lateral sclerosis and progressive bulbar palsy) requires the elimination of alternate diagnoses. This paper reviews the main characteristics of diseases mimicking ALS and the atypical subsets of ALS.

Amyotrophic Lateral Sclerosis↗
PubMed1982

Epidemiology of amyotrophic lateral sclerosis.

Motor neuron disease (MND) is used in this paper as the generic label, encompassing the clinical variants of amyotrophic lateral sclerosis (ALS), progressive myelopathic muscular atrophy (PMMA), and progressive bulbar palsy (PBP). ALS is limited to instances of anterior horn cell plus pyramidal tract involvement. When only anterior horn cell lesions are inferred, either PMMA or PBP is used, depending on the levels of involvement; when both cord and brain stem are affected. PBP is the designation. Mortality data on MND have been available for a number of countries since 1949. The coding used under international rules has varied considerably over this interval. Before 1969, hereditary muscular atrophies were included. Since 1979, no subdivision by type of MND is possible. International death rates for MND have all been rather close to 1 per 100,000 population per year, though perhaps nearer to 1.4 on the average in recent years. There has been an increasing proportion of MND deaths coded to ALS between 1949 and 1977. There is no notable geographic variation among countries, nor within countries such as the U.S. and Denmark. A slight upward trend in death rates over time in the U.S. is matched by a slight decrease in Denmark. Death rates from all sources indicate a male preponderance for ALS or MND as a whole, at about 1.5 to 1, male to female. There is also a consistent predilection by age, with few deaths under age 50 or so and a clear maximum in age-specific death rates at about age 70. This holds for both sexes. In the U.S., there is also a white-nonwhite difference, with a ratio of about 1.6:1 but with age and sex differences similar to whites. Average annual incidence rates from among white occidental populations range mostly between 0.6 and 1.8 per 100,000 population for MND and about 0.8 and 1.5 per 100,000 for ALS. Again a male predilection is seen. There is a clear maximum in age-specific incidence rates at about age 65 in all surveys except that of Rochester, Minnesota, where the age-specific rate for those 75+ years of age is apparently higher than that for those age 65 to 74. Incidence rates, then, are quite similar one land to another. A reported deficit in Mexico may reflect case-selection bias. An excess among Filipinos on Hawaii seems more a function of population age-distributions than a true racial or ethnic difference. Prevalence rates from outside the Orient range from about 1 to 7 per 100,000 population for MND and about 2 to 7 for ALS. Those surveys more likely to be reasonably complete provide ALS prevalence rates of about 4 to 6, and an overall estimate of ALS prevalence of some 5 per 100,000 population is a reasonable figure. In the Orient, most of the MND prevalence rates fall within the same range as in the occident, except for two areas of the Kii peninsula of southern Honshu, Japan, where the reported prevalence rates are some 100 to 200 per 100,000 population. These cases are similar to the Guamanian ALS, both clinically and pathologically...

Adolescent↗
PubMed1994

[Analysis of dysarthria in amyotrophic lateral sclerosis--MRI of the tongue and formant analysis of vowels].

To evaluate dysarthria in patients with ALS, we used MRI (gradient rephasing echo method) and compared it with the computed acoustic analysis. Five ALS male patients of progressive bulbar palsy type and five normal male were asked to phonate the five Japanese vowels, /a/./i/./u/./e/./o/. MRI of the sagittal tongue and vocal tract was obtained by the gradient rephasing echo method (0.2 Tesla, TR:30 ms, TE:10 ms, FA 25 degrees C, Hitachi). We could clearly visualize the change of tongue shape and the narrow site of the vocal tract for each vowel phonation. In normal subjects, the tongue shape and the narrow site of the vocal tract were distinguishable between each vowel, but unclear in ALS. Acoustic analysis showed that the first formant frequency of /i/./u/ in ALS was higher than normal and the second formant frequency of /i/./e/ in ALS was significantly lower than normal. The discrepancy from the normal first, second and third formant frequency for each vowel of ALS was most seen in /i/./e/. It was speculated that /i/ and /e/ were the most disturbed vowels in ALS. The first and second formant frequency of vowel depends on the tongue shape and the width of the oral cavity. Therefore the results of the acoustic analysis in ALS indicated poor movement of tongue in /i/./u/./e/ and were compatible with the findings of the sagittal tongue MRI. The sagittal view of the tongue in the gradient rephasing echo MRI and the acoustic analysis are useful in evaluation dysarthria in ALS.

Aged↗
PubMed1993

[Two cases with acquired immunodeficiency syndrome in our hospital].

We report two cases of AIDS whom we have recently experienced. One patient was a 54-year-old man who admitted our hospital due to third degree burn. In this case, we did not know whether or not he was suffered from AIDS, when he was delivered by the ambulance. In autopsy, pneumocystis carinii pneumonia and renal tuberculosis were found in addition to marked decrease of T cells in lymph nodes. The other patient was a 40-year-old man with remarkable symptoms of central nervous system. Route of infection of HIV is unknown. He had dementia, left hemiplegia, bulbar palsy, progressed to rigid decorticate posture and died of respiratory arrest due to involvement of the brain stem, despite of treatment including use of 3'-azido-2',3'-dideoxythymidine (AZT). Magnetic resonance (MR) images showed progressive cerebral atrophy and a diffuse high signal intensity area of cerebral white matter on T2-weighted MR images, suggesting the diagnosis of HIV-induced encephalopathy.

Acquired Immunodeficiency Syndrome↗
PubMed1984

Autosomal recessive late onset multisystem disorder with cerebellar cortical atrophy at necropsy: report of a family.

A sister and brother developed a progressive syndrome comprising bulbar palsy, supranuclear ophthalmoplegia, facial impassivity, and cerebellar ataxia together with a mixed pyramidal and extrapyramidal deficit in the limbs, in the fourth decade of life. Their parents were unaffected and inheritance was probably autosomal recessive. The female patient, who presented initially with bulbar and extrapyramidal dysfunction, was found to have cerebellar cortical atrophy at necropsy. The basal ganglia and brainstem were normal.

Adult↗
PubMed1992

Congenital oculo-bulbar palsy.

A girl developed progressive weakness of bulbar and ocular muscles starting before the age of two years. Electromyography revealed a widespread subclinical myopathy. An intercostal muscle biopsy showed complex abnormalities including occasional neurofilamentous accumulations and honeycomb-like membranous material in terminal axons. Endplates were small and some secondary synaptic clefts were abnormally deep. Acetylcholine receptors extended unusually deeply into the clefts of the junctional folds. Muscle fibres showed subsarcolemmal vacuolation at some places. This form of congenital oculo-bulbar palsy does not appear to have been described previously.

Axons↗
PubMed1999

[Acute vincristine neurotoxicity in a non-Hodgkin's lymphoma patient with Charcot-Marie-Tooth disease].

A 44-year-old, previously healthy man with a diagnosis of non-Hodgkin's lymphoma (NHL, diffuse large B-cell type, stage IIA) was treated with combination chemotherapy including vincristine (VCR). After receiving a cumulative dose of VCR, he experienced rapid and marked weakening which progressed to quadriplegia and bulbar palsy. Prior to this therapy, the patient had no neurological problems, and his siblings were asymptomatic. Physical examination identified pes cavus (hollow foot), and electrodiagnostic studies showed markedly slower nerve conduction velocity of myelinated fibers, with abundant "onion bulb" formations. Chromosomal analysis detected 17p11.2-12 duplication, thus yielding a diagnosis of Charcot-Marie-Tooth (CMT) 1A. CMT disease is a familial neuromuscular disorder, and the incidence is approximately 1 in 2,500. We concluded that if CMT disease is diagnosed, vincristine should be avoided due to the potential severity of neurotoxicity to small doses.

Adult↗