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Target Concepts:
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Query: UMLS:C0002736 (
amyotrophic lateral sclerosis
)
19,048
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Transcutaneous botulinum toxin injection in the salivary glands was introduced in 2000 as a new treatment for sialorrhoea in
amyotrophic lateral sclerosis
(
ALS
). We describe an
ALS
patient who developed serious complications of botulinum toxin treatment for sialorrhoea, and we review the relevant literature. A 64-yr-old woman with bulbar
ALS
for 6 mos was treated for disabling sialorrhoea. She had moderate dysphagia, but she was able to swallow. The submandibular and parotid glands were injected transcutaneously, under ultrasound guidance, with botulinum toxin (Dysport), 80 U on each side. Four days later, her bulbar function rapidly deteriorated, resulting in complete
aphagia
and anarthria on the fifth day. A PEG catheter was placed. Although according to the literature this treatment can be made safer by cautiously increasing the dosage and injecting the parotid glands first, BTX should not be the first-line treatment of sialorrhoea in
ALS
; comparative studies of BTX, amitryptiline, scopolamine, and radiation should be performed first.
...
PMID:Acute deterioration of bulbar function after botulinum toxin treatment for sialorrhoea in amyotrophic lateral sclerosis. 1830 72
Severe appetite and weight loss define the eating disorder anorexia nervosa, and can also accompany the progression of some neurodegenerative disorders such as
amyotrophic lateral sclerosis
(
ALS
). Although acute loss of hypothalamic neurons that produce appetite-stimulating neuropeptide Y (Npy) and agouti-related peptide (Agrp) in adult mice or in mice homozygous for the anorexia (
anx
) mutation causes
aphagia
, our understanding of the factors that help maintain appetite regulatory circuitry is limited. Here we identify a mutation (C19T) that converts an arginine to a tryptophan (R7W) in the TYRO3 protein tyrosine kinase 3 (
Tyro3
) gene, which resides within the
anx
critical interval, as contributing to the severity of
anx
phenotypes. Our observation that, like
Tyro3
-/-
mice,
anx/anx
mice exhibit abnormal secondary platelet aggregation suggested that the C19T
Tyro3
variant might have functional consequences.
Tyro3
is expressed in the hypothalamus and other brain regions affected by the
anx
mutation, and its mRNA localization appeared abnormal in
anx/anx
brains by postnatal day 19 (P19). The presence of wild-type
Tyro3
transgenes, but not an
R7W-Tyro3
transgene, doubled the weight and lifespans of
anx/anx
mice and near-normal numbers of hypothalamic Npy-expressing neurons were present in
Tyro3
-transgenic
anx/anx
mice at P19. Although no differences in R7W-Tyro3 signal sequence function or protein localization were discernible
in vitro
, distribution of R7W-Tyro3 protein differed from that of Tyro3 protein in the cerebellum of transgenic wild-type mice. Thus, R7W-Tyro3 protein localization deficits are only detectable
in vivo
Further analyses revealed that the C19T
Tyro3
mutation is present in a few other mouse strains, and hence is not the causative
anx
mutation, but rather an
anx
modifier. Our work shows that Tyro3 has prosurvival roles in the appetite regulatory circuitry and could also provide useful insights towards the development of interventions targeting detrimental weight loss.
...
PMID:The tyrosine kinase receptor Tyro3 enhances lifespan and neuropeptide Y (Npy) neuron survival in the mouse anorexia (
anx
) mutation. 2809 6
A percutaneous endoscopic gastrostomy (PEG) is an useful intervention for feeding of
amyotrophic lateral sclerosis
(
ALS
) patients who have lost oral intake function. The aim of this study was to investigate the risk factors for early death and the survival after PEG placement. A total of 102
ALS
patients who underwent PEG placement were enrolled in this study. Patients were divided into two groups; the poor prognosis group included patients who died or needed permanent mechanical ventilation within 30days after PEG placement, and the good prognosis group included patients who did not meet the criteria of the poor prognosis group. Clinical characteristics, respiratory function, and nutritional parameters were compared for the two groups to assess the correlations between clinical and laboratory variables and early death after PEG placement. Multivariate analysis between two groups revealed that higher arterial carbon dioxide pressure (PaCO
2
) and
aphagia
before PEG placement were significantly associated with the poor prognosis group. Multivariate analysis for survival also revealed that higher PaCO
2
and shorter duration from onset to PEG placement were significantly associated with shorter survival after PEG placement. In conclusion, respiratory and nutritional parameters are revealed to be important prognostic factors for
ALS
patients who undergo PEG placement.
...
PMID:An analysis of prognostic factors after percutaneous endoscopic gastrostomy placement in Japanese patients with amyotrophic lateral sclerosis. 2843 13
Amyotrophic lateral sclerosis
(
ALS
) is a fatal, neurodegenerative disorder leading to quadriplegia and
aphagia
. While swallowing difficulties and increased energy demand lead to malnutrition, increased lipid concentration may correlate with survival and respiratory functions.
Objective
: To analyze the frequency and type of dyslipidemias in a large population of clinically characterized
ALS
patients (PALS).
Methods
: The retrospective study included clinical and laboratory data of 650 consecutive PALS fulfilling the El Escorial criteria and 365 age- and gender-matched hospital controls.
Results
: 65% of PALS suffered from dyslipidemia independently of concomitant metabolic diseases. The most frequent lipid disorder was hypercholesterolemia (35% PALS, 25% controls), followed by mixed dyslipidemia (24.6%, 14%), with rare cases of hypertriglyceridemia and atherogenic dyslipidemia. Triacylglycerols (TAG) and LDL/HDL correlated with BMI, while LDL/HDL and total cholesterol (TCh) with disease duration. Among PALS with concomitant metabolic diseases, TCh correlated with disease duration and ALSFRS-R, while TAG with respiratory functions (FVC) in patients without metabolic diseases. The highest median concentration of TCh, LDL and LDL/HDL was found in classic
ALS
and PMA and the lowest in PBP.
Conclusion
: Dyslipidemia occurs more frequently in PALS compared to controls and independently of concomitant metabolic diseases. Similar to the general population, the most frequent lipid disturbance is hypercholesterolemia, followed by mixed dyslipidemia. Although particular lipid parameters correlate with BMI and disease duration, they do not show strong correlations with disease progression rate. There is a need of randomized control trials assessing the risk and benefits of the use of lipid lowering agents in
ALS
.
...
PMID:Dyslipidemia in patients with amyotrophic lateral sclerosis - a case control retrospective study. 3310 50
