Intestinal pseudoobstruction as a feature of myotonic muscular dystrophy. 1995

K Füger, and J Barnert, and W Höpfner, and M Wienbeck
III. Medizinische Klinik, Zentralklinik Augsburg, Germany.

We report two cases of intestinal pseudoobstruction caused by visceral smooth muscle involvement due to myotonic muscular dystrophy. Two patients with myotonic muscular dystrophy presented with abdominal pain, distention, constipation, and vomiting. The exclusion of mechanical obstruction by plain abdominal radiography, contrast studies, and colonoscopy led to the diagnosis of intestinal pseudoobstruction. Diagnosis was confirmed by manometric and cineradiographic findings of abnormal intestinal motility. Conservative management including laxatives and cisapride led to the resolution of the pseudoobstruction syndrome and long-term remission without relapses during a two year follow-up. In patients with known myotonic dystrophy the occurrence of intestinal pseudoobstruction should be considered in order to avoid unnecessary laparotomies.

UI MeSH Term Description Entries
D007418 Intestinal Pseudo-Obstruction A type of ILEUS, a functional not mechanical obstruction of the INTESTINES. This syndrome is caused by a large number of disorders involving the smooth muscles (MUSCLE, SMOOTH) or the NERVOUS SYSTEM. CIPO,Chronic Idiopathic Intestinal Pseudo-Obstruction,Paralytic Ileus,Pseudo-Obstruction, Intestinal,Visceral Myopathy,Congenital Short Bowel Syndrome,Enteric Neuropathy,Intestinal Pseudo-Obstruction, Idiopathic,Intestinal Pseudoobstruction,Pseudointestinal Obstruction Syndrome,Pseudoobstructive Syndrome,Chronic Idiopathic Intestinal Pseudo Obstruction,Enteric Neuropathies,Idiopathic Intestinal Pseudo-Obstruction,Idiopathic Intestinal Pseudo-Obstructions,Intestinal Pseudo Obstruction,Intestinal Pseudo Obstruction, Idiopathic,Intestinal Pseudo-Obstructions,Intestinal Pseudo-Obstructions, Idiopathic,Intestinal Pseudoobstructions,Myopathy, Visceral,Neuropathy, Enteric,Obstruction Syndrome, Pseudointestinal,Pseudo Obstruction, Intestinal,Pseudointestinal Obstruction Syndromes,Pseudoobstruction, Intestinal,Pseudoobstructive Syndromes,Syndrome, Pseudointestinal Obstruction,Syndrome, Pseudoobstructive,Visceral Myopathies
D008297 Male Males
D008365 Manometry Measurement of the pressure or tension of liquids or gases with a manometer. Tonometry,Manometries
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009130 Muscle, Smooth Unstriated and unstriped muscle, one of the muscles of the internal organs, blood vessels, hair follicles, etc. Contractile elements are elongated, usually spindle-shaped cells with centrally located nuclei. Smooth muscle fibers are bound together into sheets or bundles by reticular fibers and frequently elastic nets are also abundant. (From Stedman, 25th ed) Muscle, Involuntary,Smooth Muscle,Involuntary Muscle,Involuntary Muscles,Muscles, Involuntary,Muscles, Smooth,Smooth Muscles
D009223 Myotonic Dystrophy Neuromuscular disorder characterized by PROGRESSIVE MUSCULAR ATROPHY; MYOTONIA, and various multisystem atrophies. Mild INTELLECTUAL DISABILITY may also occur. Abnormal TRINUCLEOTIDE REPEAT EXPANSION in the 3' UNTRANSLATED REGIONS of DMPK PROTEIN gene is associated with Myotonic Dystrophy 1. DNA REPEAT EXPANSION of zinc finger protein-9 gene intron is associated with Myotonic Dystrophy 2. Dystrophia Myotonica,Myotonic Dystrophy, Congenital,Myotonic Myopathy, Proximal,Steinert Disease,Congenital Myotonic Dystrophy,Dystrophia Myotonica 1,Dystrophia Myotonica 2,Myotonia Atrophica,Myotonia Dystrophica,Myotonic Dystrophy 1,Myotonic Dystrophy 2,PROMM (Proximal Myotonic Myopathy),Proximal Myotonic Myopathy,Ricker Syndrome,Steinert Myotonic Dystrophy,Steinert's Disease,Atrophica, Myotonia,Atrophicas, Myotonia,Congenital Myotonic Dystrophies,Disease, Steinert,Disease, Steinert's,Dystrophia Myotonica 2s,Dystrophia Myotonicas,Dystrophica, Myotonia,Dystrophicas, Myotonia,Dystrophies, Congenital Myotonic,Dystrophies, Myotonic,Dystrophy, Congenital Myotonic,Dystrophy, Myotonic,Dystrophy, Steinert Myotonic,Myopathies, Proximal Myotonic,Myopathy, Proximal Myotonic,Myotonia Atrophicas,Myotonia Dystrophicas,Myotonic Dystrophies,Myotonic Dystrophies, Congenital,Myotonic Dystrophy, Steinert,Myotonic Myopathies, Proximal,Myotonica, Dystrophia,Myotonicas, Dystrophia,PROMMs (Proximal Myotonic Myopathy),Proximal Myotonic Myopathies,Steinerts Disease,Syndrome, Ricker
D010880 Piperidines A family of hexahydropyridines.
D011859 Radiography Examination of any part of the body for diagnostic purposes by means of X-RAYS or GAMMA RAYS, recording the image on a sensitized surface (such as photographic film). Radiology, Diagnostic X-Ray,Roentgenography,X-Ray, Diagnostic,Diagnostic X-Ray,Diagnostic X-Ray Radiology,X-Ray Radiology, Diagnostic,Diagnostic X Ray,Diagnostic X Ray Radiology,Diagnostic X-Rays,Radiology, Diagnostic X Ray,X Ray Radiology, Diagnostic,X Ray, Diagnostic,X-Rays, Diagnostic
D002400 Cathartics Agents that are used to stimulate evacuation of the bowels. Bowel Evacuant,Bowel Preparation Solution,Cathartic,Purgative,Purgatives,Bowel Evacuants,Bowel Preparation Solutions,Evacuant, Bowel,Evacuants, Bowel,Preparation Solution, Bowel,Preparation Solutions, Bowel,Solution, Bowel Preparation,Solutions, Bowel Preparation
D005260 Female Females

Related Publications

K Füger, and J Barnert, and W Höpfner, and M Wienbeck
February 1986, Gastroenterology,
K Füger, and J Barnert, and W Höpfner, and M Wienbeck
September 2001, Journal of clinical gastroenterology,
K Füger, and J Barnert, and W Höpfner, and M Wienbeck
March 2007, The Journal of the Association of Physicians of India,
K Füger, and J Barnert, and W Höpfner, and M Wienbeck
February 1989, Neurologic clinics,
K Füger, and J Barnert, and W Höpfner, and M Wienbeck
June 2015, Clinical case reports,
K Füger, and J Barnert, and W Höpfner, and M Wienbeck
March 1948, Revista chilena de neuro-psiquiatria,
K Füger, and J Barnert, and W Höpfner, and M Wienbeck
January 1996, International journal of colorectal disease,
K Füger, and J Barnert, and W Höpfner, and M Wienbeck
January 2011, Revista de investigacion clinica; organo del Hospital de Enfermedades de la Nutricion,
K Füger, and J Barnert, and W Höpfner, and M Wienbeck
June 1979, Psychosomatics,
K Füger, and J Barnert, and W Höpfner, and M Wienbeck
November 1964, Archives of internal medicine,
Copied contents to your clipboard!