scholarly journals Dyke Davidoff Masson Syndrome with Abdominal Epilepsy-Rare Presentation

2013 ◽  
Vol 3 (2) ◽  
pp. 25-28
Author(s):  
Shitanshu Srivastava ◽  
Rajat Pratap Singh ◽  
KL Srivastava

Dyke Davidoff Masson Syndrome (DDMS) with abdominal epilepsy, is a rare clinical condition. It is characterized by severe abdominal pain with seizures, facial asymmetry, contralateral hemiparesis, and mental retardation. Diagnosis is made clinically coupled with radiological features which include cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses along with abnormal EEG and response to antiepileptic drugs. Here we report a case of DDMS with abdominal epilepsy in a 10 year old male child who presented with recurrent episodes of severe abdominal pain followed by seizures, hemiparesis and mental retardation. CT and MRI showed hemiatrophy of right cerebral hemisphere. DOI: http://dx.doi.org/10.3126/ajms.v3i2.6172 Asian Journal of Medical Sciences 3(2012) 25-28

1998 ◽  
Vol 56 (4) ◽  
pp. 803-807 ◽  
Author(s):  
PAULO HENRIQUE AGUIAR ◽  
WEI LIU CHING ◽  
HELIO LEITÃO ◽  
F. ISSA ◽  
GUILHERME LEPSKI ◽  
...  

Cerebral hemiatrophy or Dyke-Davidoff-Masson syndrome is a condition characterized by seizures, facial asymmetry, contralateral hemiplegia or hemiparesis, and mental retardation. These findings are due to cerebral injury that may occur early in life or in utero. The radiological features are unilateral loss of cerebral volume and associated compensatory bone alterations in the calvarium, like thickening, hyperpneumatization of the paranasal sinuses and mastoid cells and elevation of the petrous ridge. The authors describe three cases. Classical findings of the syndrome are present in variable degrees according to the extent of the brain injury. Pathogenesis is commented.


2017 ◽  
Vol 21 (1) ◽  
Author(s):  
Sanjay M. Khaladkar ◽  
Shishir Chauhan ◽  
Abhijit M. Patil ◽  
Siddappa G. Gandage ◽  
Surbhi Chauhan Kalra

Dyke–Davidoff–Masson syndrome is a rare condition with classical, clinical and radiological changes – mental retardation, hemiparesis, facial asymmetry, seizures and cerebral hemiatrophy with calvarial changes. Contralateral cerebellar atrophy is rare and occurs if insult occurs after 1 month of age. We report a case of a 6-year-old female child presenting with right-sided hemiparesis, convulsions and left cerebral hemiatrophy with an old infarct in left middle cerebral artery (MCA) territory, ipsilateral calvarial thickening and right (crossed) cerebellar atrophy.


Cureus ◽  
2018 ◽  
Author(s):  
Abdul Rehman ◽  
Abdul Majeed Maliyakkal ◽  
Vamanjore A Naushad ◽  
Hisham Allam ◽  
Ahmed M Suliman

2012 ◽  
Vol 1 (2) ◽  
pp. 84-86 ◽  
Author(s):  
Naba Raj Koirala ◽  
Roshana Khadka ◽  
Manoj Bhattarai ◽  
Dibya Tulachan ◽  
Ajay Kumar Das ◽  
...  

Dyke-Davidoff-Masson Syndrome (DDMS) is characterized by seizures, facial asymmetry, contralateral hemiplegia and mental retardation. The characteristic radiologic features are cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. Here we report a case of a 16 years young girl who presented with seizures severe mental retardation and weakness of left upper-limb and on CT brain was diagnosed to have DDMS.DOI: http://dx.doi.org/10.3126/jonmc.v1i2.7306 Journal of Nobel Medical College (2012), Vol.1 No.2 p.84-86


2016 ◽  
Vol 8 (1) ◽  
pp. 20-26 ◽  
Author(s):  
Ujjawal Roy ◽  
Ajay Panwar ◽  
Adreesh Mukherjee ◽  
Debsadhan Biswas

Dyke-Davidoff-Masson syndrome (DDMS) is a rare disease which is clinically characterized by hemiparesis, seizures, facial asymmetry, and mental retardation. The classical radiological findings are cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. This disease is a rare entity, and it mainly presents in childhood. Adult presentation of DDMS is unusual and has been rarely reported in the medical literature. Key Messages: DDMS is a rare disease of childhood. However, it should be kept in mind as a diagnostic possibility in an adult who presents with a long duration of progressive hemiparesis with seizures and mental retardation. Cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses are diagnostic for this illness on brain imaging.


Pathology ◽  
1993 ◽  
Vol 25 (1) ◽  
pp. 24-26 ◽  
Author(s):  
Pek-Yoon Chong ◽  
Thiow-Kong Ti

2018 ◽  
Vol 69 (8) ◽  
Author(s):  
Doina Vesa ◽  
Cristian Martu ◽  
Razvan Leata ◽  
Ludmila Lozneanu ◽  
luminita Radulescu ◽  
...  

Paranasal mucoceles are a type of cysts that evolve slowly and are asymptomatic; this poses a difficulty in diagnosing the patient because the symptoms can go unnoticed. The mucocele evolves unpredictably. On the one hand, it can become infected turning into pyoceles and on the other hand, it can invade important regions such as the orbital, cranial or genian regions, creating facial asymmetry. This is a retrospective case study of 37 patients diagnosed with sinus mucoceles, followed up by clinical examination and paraclinical tests such as CT and MRI scans. The biochemical components of the liquid from within the mucocele were analyzed and the following criteria were recorded: NaCl-, Cl-, Na+ and cholesterine as well as cellular components such as mastocytes, macrophages, hematocytes and leucocytes. In all cases, the treatment option was surgery with favorable post-operative and follow-up evaluation. The mucoceles that appeared post-operatively (maxillary and ethmoid sinuses) evolved more rapidly than the mucoceles that were induced byan external injury. Longer follow-up of operated patients permitted a more timely diagnosis of recurrences.


2020 ◽  
Vol 13 (12) ◽  
pp. e236412
Author(s):  
Alfonsa C Taiello ◽  
Vincenzo La Bella ◽  
Rossella Spataro

Thoracic radiculopathy is a rare cause of thoracic-abdominal or abdominal pain in subjects with poorly controlled diabetes. We present a case of a young woman with type I diabetes and a severe abdominal pain in both lower quadrants. An extensive diagnostic gastroenterological and gynaecological workup did not disclose abnormalities. Electromyography revealed an initial polyneuropathy and significant neurogenic abnormalities in the T10-T12 paravertebral muscles. Following the hypothesis that the radiculopathy-related abdominal pain might have an immuno-mediated pathogenesis, the patient underwent a complex trial of immunotherapy, which was accompanied by a sustained improvement over months to full recovery. This report would support the hypothesis that immune-mediated mechanisms are still active even months after onset of symptoms.


2016 ◽  
Vol 68 (5) ◽  
pp. 544-552
Author(s):  
Aaron Lewandowski ◽  
Steven Dorsey

Author(s):  
Daisuke Honda ◽  
Isao Ohsawa ◽  
Keiichi Iwanami ◽  
Hisaki Rinno ◽  
Yasuhiko Tomino ◽  
...  

AbstractHereditary angioedema due to C1-inhibitor deficiency (HAE-C1-INH) is a rare disease, which induces an acute attack of angioedema mediated by bradykinin. HAE-C1-INH can cause serious abdominal pain when severe edema develops in the gastrointestinal tract. However, because it takes a long time, 13.8 years on average in Japan, from the occurrence of the initial symptom to the diagnosis due to low awareness of the disease, undiagnosed HAE-C1-INH patients sometimes undergo unnecessary surgical procedures for severe abdominal pain. We herein present a 56-year-old patient with HAE-C1-INH, who underwent numerous abdominal operations. He frequently needed hospitalization with the administration of opioid due to severe abdominal pain. However, after he was accurately diagnosed with HAE-C1-INH at 55 years of age, he could start self-administration for an acute attack with icatibant, a selective bradykinin B2 receptor antagonist. Consequently, he did not need hospitalizing for ten months after the beginning of the treatment. A series of an accurate diagnosis and appropriate treatment for HAE-C1-INH improved his quality of life. Thus, HAE-C1-INH should be considered, when we meet patients with unidentified recurrent abdominal pain. This case highlights significance of an early diagnosis and appropriate treatment for HAE-C1-INH.


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