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Results for “Bulbar Palsy, Progressive”

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PubMed1990

Far-field and cortical somatosensory evoked potentials in motor neuron disease.

We examined median somatosensory evoked potentials (SEPs) in 26 patients with sporadic motor neuron disease (MND). SEPs were recorded with multiple scalp derivations, using both the midfront and the earlobe as references for each subject. Central conduction time (CCT) was abnormal in three patients, but only when using the midfront reference. Moreover, an exclusive alteration of the early prerolandic potentials (absent or delayed P20 and/or P22) was noted using the earlobe reference in amyotrophic lateral sclerosis and in progressive bulbar palsy (54% and 50% of patients, respectively) but not in progressive muscular atrophy. These findings correlated with clinical evidence of upper motor neuron signs and with the severity of the disease. In agreement with recent views regarding the sources of the early anterior cortical responses, neuronal loss in the motor cortex may be considered as affecting the generator sites of these potentials.

Adult↗

PubMed1994

An open-randomized clinical trial of selegiline in amyotrophic lateral sclerosis.

Based on the hypothesis that free radicals play a general role in the neurodegenerative process in motor neuron disease, we tested selegiline in a group of patients affected by amyotrophic lateral sclerosis (ALS) to examine whether it might modify the progression of the disease. Patients were admitted if they were 25-80 years old and had a confirmed diagnosis of ALS with symptoms lasting no longer than 24 months. Patients with familial ALS, pure progressive bulbar palsy, primary lateral sclerosis or progressive muscle atrophy were excluded; a total of 111 patients were recruited. Fifty-three patients were randomly assigned to receive the drug (selegiline 10 mg/day orally for 6 months) and the remaining 58 were considered ALS controls. Mortality was similar in the two groups (4 and 5 patients respectively), though the difference was not statistically significant. Among the survivors, mean MRC and Norris disability scores and forced vital capacity were fairly similar in the two groups at all times and no statistically significant difference between treated and untreated patients was found. The results did not change when the data were related to age, duration and characteristics of onset of the disease. The rate of progression was significantly more rapid in patients with bulbar symptoms in both groups. Our data do not show any significant effect of selegiline in modifying the progression of ALS.

Adult↗

PubMed1997

Phenotypic heterogeneity in motor neuron disease patients with CuZn-superoxide dismutase mutations in Scandinavia.

Four-hundred and fifty-one blood samples from Scandinavian patients with motor neuron disease were analysed for mutations in the CuZn-superoxide dismutase gene. Forty-one (9.6%) of the 427 patients with the amyotrophic lateral sclerosis (ALS) form of the disease were found to have a disease-associated mutation, and 14 of these patients were apparently sporadic cases. A mutation was found in 12 of the 51 families with recognized familial ALS. The five different mutations found (Ala4Val, Val14Gly, Asp76Tyr, Asp90Ala, Gly127insTGGG) have different genetic characteristics and are associated with very variable phenotypes spanning from rapidly progressing disease with only lower motor neuron signs to very slowly progressing disease with both the upper and lower motor neuron systems affected. The patients showed different sites of onset, though the progressive bulbar palsy form of the disease appears to be rare among patients with a CuZn-superoxide dismutase mutation. The progression of motor signs and symptoms followed the same basic pattern in patients with different mutations. Extra-motor system symptoms were frequent among patients with a CuZn-superoxide dismutase mutation. The results suggest that patients with mutations in the CuZn-superoxide dismutase gene constitute one disease entity. The Val14Gly and Asp76Tyr mutations have not been reported before, and the latter is the first mutation to be found in exon 3 of the CuZn-superoxide dismutase gene.

Adult↗

PubMed1980

Progressive bulbar paralysis associated with neural deafness. A nosological entity.

A complete autopsy verification of progressive bulbar palsy associated with neural deafness was performed. Hearing loss and speech difficulties developed in a five-year-old girl. When she was 24 years old, clinical examination demonstrated deafness and bulbopontine paralysis together with retinitis pigmentosa, peripheral amyotrophies, pyramidal signs, and ataxia. The patients died at 27 years and the autopsy disclosed degenerative changes characterized by simple atrophy and loss of neurons accompanied by gliosis and loss of myelinated fibers. The structures principally affected were the anterior horns and the motor nuclei of the brain stem together with the eighth cranial nerve nuclei. Loss of myelinated fibers was found in the spinocerebellar and pyramidal tracts and in the fasciculus gracilis. Our study suggests that progressive bulbar paralysis with neural deafness should be considered as a nosological entity.

Adolescent↗

PubMedDate not supplied

Frequency of motor neuron diseases in a Mexico City referral center.

OBJECTIVE: To analyze the incidence and clinical characteristics of MND (Motor Neuron Diseases) in a Mexican institution. MATERIAL AND METHODS: It was a retrospective study, of 274 definitive MND patients seen in a neurological reference hospital of Mexico City during the period of 1965-1995 (248 as Amyotrophic Lateral Sclerosis, 15 as Progressive Bulbar Palsy, 8 as Primary Lateral Sclerosis and 3 as Progressive Spinal Atrophy). RESULTS: The frequency of MND increased gradually in our institution in the 31 years revised. The mean age of onset in our series was approximately 48 years in contrast to a higher age found in other series. The clinical features are similar to those found elsewhere. CONCLUSIONS: Our study showed that the frequency of MND is increasing in Mexico in a similar fashion to that observed in the rest of the world. This makes conceivable that the incidence of MND in Mexico may also resemble the figures reported worldwide. Prospective population studies are required to establish the incidence of MND in Mexico.

Adult↗

PubMed1990

Immunosuppressive treatment of patients with amyotrophic lateral sclerosis.

Documented treatment for amyotrophic lateral sclerosis (ALS) is not available. Several studies have suggested an immunological etiology and an effect on the course of disease, when ALS-patients were treated with immunosuppressants. The aim of the present study was to evaluate the effect of immunosuppressive therapy in ALS-patients comparing the course of disease in treated patients and in historic controls with ALS; 21 patients were included in the study, 17 men and 4 women. Median age at admission was 54 years for men and 61 years for women. 5 had progressive bulbar palsy, 7 both upper and lower motor neuron affections and 9 progressive muscular atrophy. Patients were treated with prednisolone and azathioprine for 1 year and examined regularly; 12 were treated and followed for more than a year. No definite difference between survival in treated patients and their controls was found.

Administration, Oral↗

PubMed1991

Motor neurone disease in Thailand: the clinical aspects of 77 patients.

This is the first large scale case series of motor neurone disease (MND) in Thailand. Seventy-seven patients were identified between 1978 and 1984 at Siriraj Hospital Medical School, Bangkok, Thailand. Fifty-five patients were male (71.43%) and the mean age of the patients was 51.55 (SD 14.26) years with the range of 17 to 78 years. Clinical classification of MND was categorized as progressive bulbar palsy (PBP), 26 patients (33.77%); amyotrophic lateral sclerosis (ALS), 42 patients (54.54%); and progressive spinal atrophy (PSA), 9 patients (11.69%). The mean age of PBP, ALS and PSA were in the order of 57.61 (SD 12.09), 52.81 (SD 11.18), and 28.11 (SD 9.44) years. Progressive spinal atrophy group was younger than PBP and ALS groups significantly at the P-value less than 0.05 by analysis of variance and Duncan tests. Fifty-three patients (72.60%) were resident in Bangkok and the central part of Thailand. The main presenting symptoms were wasting of the small muscles of both hands, leg weakness, and speech and/or swallowing difficulties. These symptoms were found in 62 patients (81.58%). Nearly half of the patients (48.68%) came to our care within six months of onset, 22.8 per cent presented with asymmetry of motor wasting, while limb and trunk fasciculation was seen in 73.61 per cent. Dysarthria, dysphagia and tongue fasciculation were recorded as 51.32, 48.68, 60.53 per cent respectively. Exaggerated deep tendon reflexes were noted as 65.79 and 80.26 per cent over the upper and lower limbs, while Babinski sign was elicited in only 23.3 per cent of the patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1990

Role of aluminium in sporadic motor neuron disease.

In an attempt to ascertain the role of aluminium in sporadic motor neuron disease (MND), trace metals were estimated in blood, plasma and cerebrospinal fluid (CSF) of 38 patients of sporadic MND and 30 controls by direct current plasma emission spectrophotometry. CSF aluminium levels (20.76 +/- 4.38 micrograms/dl) were significantly higher (P less than 0.05) in those patients of MND who presented as progressive bulbar palsy (PBP) as compared to the other subtypes of MND (amyotrophic lateral sclerosis = 7.71 +/- 2.01 micrograms/dl; progressive muscular atrophy = 10.01 +/- 2.41 micrograms/dl) and controls (11.63 +/- 2.82 micrograms/dl). Aluminium may be important in the etiopathogenesis of a subgroup of sporadic MND.

Adolescent↗

PubMed1992

Inadvertent intrathecal injection of daunorubicin with fatal outcome.

We report the first known case of daunorubicin administered directly into the human central nervous system. A 3 1/2-year-old female with pneumonia and otitis media was diagnosed with acute lymphoblastic leukemia and was admitted for antibiotics and chemotherapy. On the first day she inadvertently received a 17 mg intrathecal (IT) injection of daunorubicin. When the error was recognized about 1 hour later, her cerebrospinal fluid (CSF) was exchanged with sterile saline by barbotage, IT hydrocortisone was given, a subarachnoid catheter was inserted, and the CSF was allowed to drain for 36 hours. Only 5.6 mg (33%) of the dose was recovered from CSF, 2.7 mg as daunorubicin and 2.9 mg as the metabolite, daunorubicinol. Initially she was asymptomatic and induction therapy continued with vincristine, 1-asparaginase, prednisone, and IT methotrexate. One week after the daunorubicin injection she developed headache and irritability; CSF protein was 3.2 gm/dl. On the 12th day, she developed fungal sepsis and worsening pneumonia. On the 15th day, she became comatose with a flacid paraparesis, areflexia, and an ascending progressive bulbar palsy. A series of computerized tomography scans over 6 weeks showed increasing diffuse cerebral atrophy. Nerve conduction velocity studies were consistent with an axonal neuropathy. Despite her multiple concurrent medical problems, the timing and characteristics of neurologic damage suggest that IT daunorubicin caused progressive destruction of the nervous system.

Central Nervous System Diseases↗

PubMed1996

Pharmacologic treatment of emotional lability.

Emotional lability may be a part of the syndrome of pseudobulbar palsy. Here we report our experience with fluvoxamine, a selective serotonin reuptake inhibitor, used to treat 10 patients with emotional incontinence. Over a 7-month period, we studied and treated 10 consecutive patients (mean age, 61 +/- 8 years) attending our department: four had amyotrophic lateral sclerosis (progressive bulbar palsy form), four had clinically definite multiple sclerosis, and two had had strokes. They were given a single evening dose (100 mg) of fluvoxamine. All 10 patients had > 30 affective outbursts daily. It was observed that in 2 to 6 days, all the patients improved, the number of emotional outbursts dropping to none to five per day. This result suggests that the serotoninergic system may be implicated in emotional lability. The short latency of improvement we observed in our patients suggests that the mechanism of fluvoxamine for treating emotional lability differs from its mechanism for treating affective disorders.

Affective Symptoms↗

PubMed2000

Rapidly progressive polymyositis with elevated antiacetylcholine receptor antibody activity.

We report a 51-year-old woman with polymyositis accompanied by a high titer of antiacetylcholine receptor antibody. The patient presented with weakness of grip strength followed by rapidly progressive dyspnea, which required mechanical ventilation. She was treated with a glucocorticoid and came off the respirator one week later. Antiacetylcholine receptor antibody activity was elevated in the acute phase and decreased during recovery, although other signs of myasthenia gravis were negative. This patient suggested that in cases of rapidly progressive bulbar palsy and limb muscle weakness, it is necessary to include polymyositis associated with elevated antiacetylcholine receptor antibody activity in the differential diagnosis.

Anti-Inflammatory Agents↗

PubMed2004

A mutation in the vesicle-trafficking protein VAPB causes late-onset spinal muscular atrophy and amyotrophic lateral sclerosis.

Motor neuron diseases (MNDs) are a group of neurodegenerative disorders with involvement of upper and/or lower motor neurons, such as amyotrophic lateral sclerosis (ALS), spinal muscular atrophy (SMA), progressive bulbar palsy, and primary lateral sclerosis. Recently, we have mapped a new locus for an atypical form of ALS/MND (atypical amyotrophic lateral sclerosis [ALS8]) at 20q13.3 in a large white Brazilian family. Here, we report the finding of a novel missense mutation in the vesicle-associated membrane protein/synaptobrevin-associated membrane protein B (VAPB) gene in patients from this family. Subsequently, the same mutation was identified in patients from six additional kindreds but with different clinical courses, such as ALS8, late-onset SMA, and typical severe ALS with rapid progression. Although it was not possible to link all these families, haplotype analysis suggests a founder effect. Members of the vesicle-associated proteins are intracellular membrane proteins that can associate with microtubules and that have been shown to have a function in membrane transport. These data suggest that clinically variable MNDs may be caused by a dysfunction in intracellular membrane trafficking.

Adult↗

PubMed1986

Degeneration of Betz cells in motor neuron disease. A Golgi study.

A morphological study using a modified Golgi impregnation technique was carried out on Betz cells in the motor area of eight patients with motor neuron disease (MND) and age-matched controls without abnormal neuropathological findings during routine examination. Betz cells were categorized as normal (type I), or those with moderate (type II) or marked (type III) degeneration of dendrites. Types II and III Betz cells tended to be dominant in the cases of MND, whereas type I Betz cells constituted the highest percentage in most of the control cases. The frequently observed morphological alterations of Betz cells in MND were accumulation of lipofuscin, degeneration or loss of dendrites, and reactive astrocytic gliosis around the Betz cell soma. It was suggested that Betz cell changes in MND are not only due to aging but also to evident pathological processes, since the above-mentioned findings were more extensive in the MND cases than in the control cases. Also, the so-called lower motor neuron disease belongs to one basic disease termed "motor neuron disease", because the pathological changes of Betz cells were observed even in cases of spinal progressive muscular atrophy alone or associated with progressive bulbar palsy, which are devoid of pyramidal tract degeneration and signs of upper motor neuron lesion.

Aged↗

PubMed1993

The prognosis of adult-onset motor neuron disease: a prospective study based on the Scottish Motor Neuron Disease Register.

The Scottish Motor Neuron Disease Register (SMNDR) is a prospective, collaborative, population-based project which has been collecting data on incident patients since 1989. In this report we present the clinical features of 229 patients with motor neuron disease (218 sporadic and 11 familial) diagnosed in 1989 and 1990 and compare their prognosis with previous studies of survival. The overall 50% survival from symptom onset was 2.5 years (95% CI, 2.2-3.0) and 5-year survival 28% (95% CI, 20-36%). The presence of progressive bulbar palsy (PBP), either at presentation or developing during the course of the illness, significantly reduced survival and was the most important prognostic indicator. Patients who survived longer than 5 years from symptom onset did not have PBP as part of their presenting illness. The prognosis was worse for women, and this was in part related to the higher frequency of PBP in older women, but age was also an independent adverse risk factor. Differences in survival between this and previous series can probably be explained on the basis of variation in case definition and ascertainment methods.

Adolescent↗

PubMed1993

Onset, natural history and outcome in idiopathic adult motor neuron disease.

Cases of adult-onset idiopathic motor neuron disease (MND) identified from January 1970 through December 1986 were studied in a defined area of California. The patients were followed prospectively throughout the illness in 99% of cases. Among 708 cases aged 25-74 years at onset, the most common type (86%) was typical, sporadic amyotrophic lateral sclerosis (SporALS). The risk of bulbar onset and shorter survival times increased with age in both men and women. About 4%, mainly younger men, experienced unusually long courses with milder paralysis, but could not be identified early in the illness. They probably represent one extreme of the ALS spectrum rather than a distinct subtype. Familial ALS (FamALS) was diagnosed in 7%. It developed earlier in life but ran a slightly longer course, which suggests a different disease process. Overall there was a statistically significant predominance of males, especially in 17 cases (2%) of progressive muscular atrophy (PMA). There were 26 cases (4%) classified as primary lateral sclerosis (PLS). Progressive bulbar palsy was not found; that diagnosis usually denotes merely the bulbar onset of ALS.

Adult↗

PubMed1995

Transcranial magnetic stimulation as a diagnostic and prognostic test in amyotrophic lateral sclerosis.

Corticospinal stimulus conduction was investigated after transcranial magnetic stimulation of the motor cortex in 63 patients (20 female, 43 male, 59 +/- 12 years) with amyotrophic lateral sclerosis (ALS) and progressive bulbar palsy. Recordings were made bilaterally from the Abductor digiti minimi muscle (ADM) in the hand and the Tibialis anterior muscle (TA) in the leg. Thirteen patients were re-examined after 250 +/- 125 days. Eight patients were examined a third time after 552 +/- 165 days. At the first investigation central motor conduction time was abnormal to one or more target muscles in 51% (n = 32) of all patients. No significant delay in CMCT developed during follow-up. The average time of survival of patients with normal CMCT at the first investigation was 16.5 +/- 7.5 months, and 14.7 +/- 8.8 months in patients with abnormal CMCT. This is not a significant difference. It is therefore concluded that transcranial magnetic stimulation is not a sensitive tool in the diagnosis of ALS. Furthermore, CMCT does not provide significant prognostic information.

Adolescent↗

PubMed2000

Immunocytochemical and ultrastructural study of the motor cortex in patients with lower motor neuron disease.

This report conveys the results of an immunocytochemical and ultrastructural study of the motor cortices of six patients with clinically and pathologically-diagnosed lower motor neuron disease (LMND) such as progressive spinal muscular atrophy, progressive bulbar palsy, or both. These patients showed neither upper motor neuron signs nor upper motor neuron system involvement including the corticospinal tract in postmortem tissues after conventional stainings. Specimens from 12 age-matched normal individuals served as controls. All patients showed loss of brainstem motor neurons and anterior horn cells. Betz cells in LMND patients were significantly reduced in number as compared to controls (P<0.01). However, there was no significant difference in the density of phosphorylated neurofilament (PNF) (200 kDa)-positive Betz cells between LMND patients and controls. The pyramidal cells of layer III were immunostained for PNF in four of six LMND patients, but there was no significant difference in the density of PNF-positive pyramidal cells between LMND patients and controls. The number of astrocytes immunostained for glial fibrillary acidic protein increased in layer III and at the transition between white matter and motor cortex in three out of six patients and one of 12 controls. Ultrastructural examination revealed that the Betz cells of five of six LMND patients had Bunina bodies, Lewy body-like inclusions or skein-like inclusions, all of which are characteristic of amyotrophic lateral sclerosis (ALS). These findings suggest that most patients with clinically and pathologically-diagnosed LMND should be classified into the category of ALS.

Adult↗

PubMed1999

Motor neuron disease: usefulness of transcranial magnetic stimulation in improving the diagnosis.

Clinical upper motor neuron (UMN) involvement is sometimes difficult to detect in motor neuron disease (MND). For this reason we performed transcranial magnetic stimulation (TMS) to find out whether this technique may be useful in revealing signs of pyramidal tract impairment. Fifty-five MND patients, clinically divided into 22 amyotrophic lateral sclerosis (ALS), 18 ALS with probable UMN signs (ALS-PUMNS), 10 pure lower motor neuron syndrome (LMNS), and 5 progressive bulbar palsy (PBP), underwent standard TMS, recording from abductor digiti minimi and flexor allucis muscles. Prolongation of cortical motor evoked potential (MEP) latency and central conduction time (CCT) and absent MEP were considered as pathologic. ALS-PUMNS and LMNS patients were clinically reclassified after 1 year. TMS was abnormal in 95.4% of ALS, 72.2% of ALS-PUMNS, 50% of LMNS and 20% of PBP. Correlations between TMS parameters and both clinical signs of UMN involvement and disease severity were highly significant. TMS showed a high sensitivity, but lacked specificity. After 1 year, 11 patients among the ALS-PUMNS group were clinically reclassified as definite ALS: all of them had shown TMS abnormalities at the first examination. In conclusion, TMS provides important diagnostic information for an early prediction of ALS in those MND patients presenting with clinically equivocal UMN impairment.

Adult↗