scholarly journals Adult Presentation of Dyke-Davidoff-Masson Syndrome: A Case Report

2016 ◽  
Vol 8 (1) ◽  
pp. 20-26 ◽  
Author(s):  
Ujjawal Roy ◽  
Ajay Panwar ◽  
Adreesh Mukherjee ◽  
Debsadhan Biswas
Keyword(s):  
Mental Retardation ◽  
Rare Disease ◽  
Medical Literature ◽  
Facial Asymmetry ◽  
Rare Entity ◽  
Radiological Findings ◽  
Cerebral Hemiatrophy ◽  
Long Duration ◽  
Frontal Sinuses ◽  
Adult Presentation

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.

scholarly journals MR and CT imaging in the Dyke-Davidoff-Masson syndrome: report of three cases and contribution to pathogenesis and differential diagnosis

1998 ◽  
Vol 56 (4) ◽  
pp. 803-807 ◽  
Author(s):  
PAULO HENRIQUE AGUIAR ◽  
WEI LIU CHING ◽  
HELIO LEITÃO ◽  
F. ISSA ◽  
GUILHERME LEPSKI ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Mental Retardation ◽  
Brain Injury ◽  
Paranasal Sinuses ◽  
Facial Asymmetry ◽  
Cerebral Injury ◽  
Radiological Features ◽  
Cerebral Hemiatrophy ◽  
The Brain ◽  
Syndrome Report

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.

scholarly journals Dyke–Davidoff–Masson syndrome with crossed cerebellar atrophy

2017 ◽  
Vol 21 (1) ◽  
Author(s):  
Sanjay M. Khaladkar ◽  
Shishir Chauhan ◽  
Abhijit M. Patil ◽  
Siddappa G. Gandage ◽  
Surbhi Chauhan Kalra
Keyword(s):  
Mental Retardation ◽  
Middle Cerebral Artery ◽  
Cerebellar Atrophy ◽  
Facial Asymmetry ◽  
Rare Condition ◽  
Female Child ◽  
Left Middle Cerebral Artery ◽  
Cerebral Hemiatrophy ◽  
Radiological Changes ◽  
Left Middle

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.

scholarly journals Bilateral breast tuberculosis � A rare entity

2015 ◽  
Vol 1 (3) ◽  
pp. 173
Author(s):  
Nikhil S Shetty ◽  
Vijay P Agrawal ◽  
Ashwin Narasimhaprasad
Keyword(s):  
Developing Countries ◽  
Rare Disease ◽  
Immunocompetent Patient ◽  
Rare Entity ◽  
Radiological Findings ◽  
Western Countries ◽  
Radiologic Findings ◽  
Breast Tuberculosis ◽  
Malignant Lesions

Tuberculosis of the breast is an extremely rare disease. In developing countries, where tuberculosis is endemic, the incidence is 0.25 - 4.5% in contrast to western countries where it is less than 0.1%. The diagnosis is difficult because of nonspecific clinical and radiologic findings. It is usually suspected in a multiparous, lactating woman who may be immunocompromised. Radiological findings simulate malignant lesions of breast. We report an immunocompetent patient, who had no issue and had bilateral breast involvement which is very rare.

scholarly journals Dyke-Davidoff-Masson Syndrome: A Delayed Diagnosis of an Acquired Variant

2018 ◽  
Vol 2 (2) ◽  
pp. e000121
Author(s):  
T Seetam Kumar ◽  
Abhijit Vipul ◽  
Rohtas Yadav
Keyword(s):  
Mental Retardation ◽  
Early Diagnosis ◽  
Female Patient ◽  
Clinical Presentation ◽  
Delayed Diagnosis ◽  
Intractable Epilepsy ◽  
Drug Resistant ◽  
Cerebral Hemiatrophy ◽  
Frontal Sinuses

Dyk-Davidof-Masson Syndrome (DDMS) is an important cause of intractable and drug-resistant seizures. It has varied clinical presentation and history with distinct neuroimaging features. Here, we describe a female patient presented with recurrent intractable convulsion, mental retardation, hemiparesis, and characteristic neuroimaging features of cerebral hemiatrophy, calvarial thickening, and ipsilateral hyperpneumatization of the frontal sinuses which is suggestive of DDMS. Early institution of neuroimaging in patients with intractable epilepsy will make early diagnosis and better outcome.

scholarly journals Dyke-Davidoff-Masson Syndrome

2012 ◽  
Vol 1 (2) ◽  
pp. 84-86 ◽  
Author(s):  
Naba Raj Koirala ◽  
Roshana Khadka ◽  
Manoj Bhattarai ◽  
Dibya Tulachan ◽  
Ajay Kumar Das ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Upper Limb ◽  
Facial Asymmetry ◽  
Young Girl ◽  
Severe Mental Retardation ◽  
Medical College ◽  
Cerebral Hemiatrophy ◽  
Ct Brain ◽  
Radiologic Features

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

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
Keyword(s):  
Mental Retardation ◽  
Abdominal Pain ◽  
Facial Asymmetry ◽  
Severe Abdominal Pain ◽  
Medical Sciences ◽  
Rare Presentation ◽  
Radiological Features ◽  
Cerebral Hemiatrophy ◽  
Right Cerebral Hemisphere ◽  
Ct And Mri

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

scholarly journals Agenesis of the posterior arch of the atlas

2002 ◽  
Vol 57 (2) ◽  
pp. 73-76 ◽  
Author(s):  
Martin Torriani ◽  
José Leonardo Goes Lourenço
Keyword(s):  
Computerized Tomography ◽  
Congenital Abnormality ◽  
Structural Instability ◽  
Neurological Deficits ◽  
Atlantoaxial Instability ◽  
Posterior Arch ◽  
Rare Entity ◽  
Radiological Findings ◽  
Plain Films ◽  
Rare Congenital Abnormality

PURPOSE: To illustrate the radiological findings and review the current literature concerning a rare congenital abnormality of the posterior arch of the atlas. CASE REPORT: An adult female without neurological symptoms presented with an absent posterior arch of the atlas, examined with plain films and helical computerized tomography. Complete agenesis of the posterior arch of the atlas is a rare entity that can be easily identified by means of plain films. Although it is generally asymptomatic, atlantoaxial instability and neurological deficits may occur because of structural instability. Computerized tomography provides a means of assessing the extent of this abnormality and can help evaluate the integrity of neural structures. Although considered to be rare entities, defects of the posterior arch of the atlas may be discovered as incidental asymptomatic findings in routine cervical radiographs. Familiarity with this abnormality may aid medical professionals in the correct management of these cases.

scholarly journals Intestinal lymphangiectasia: case report

2019 ◽  
Vol 6 (5) ◽  
pp. 1678
Author(s):  
Reham Mohmmad Aljohnei ◽  
Hawazen Yousef Abdullah Alani
Keyword(s):  
Case Report ◽  
Rare Disease ◽  
Present Report ◽  
Abdominal Distention ◽  
Intestinal Lymphangiectasia ◽  
Peripheral Edema ◽  
Long Duration ◽  
White Spots ◽  
Intestinal Lymphatics ◽  
History Of

Primary intestinal lymphangiectasia (PIL) was first described by Waldmann et al, in 1961. PIL is a rare disease with several hundred reported cases. It is rarely reported in adults because it is presumably a congenital disorder and when present in adults it typically produces a long duration of manifestation such as diarrhea, abdominal distention from ascites, and peripheral edema. This disorder is characterized by markedly dilated intestinal lymphatics, hypoproteinemia, generalized edema, lymphocytopenia hypogammaglobinemia, and immunologic anomalies. The loss of protein into the from dilated intestinal lymphatics leads to the development of hypoproteinemia in these patients and its demonstration is important in the diagnosis of intestinal lymphangiectasia. The disease can be secondary to congenital, secondary or idiopathic defects in the formation of the lymphatic ducts. In the present report, we describe a case of 15 years old female presented to our hospital with history of generalized edema, bilateral hand spasm, and diarrhea. Endoscopy of the patient revealed White spots (dilated lacteals), white nodules, and submucosal elevations were observed. Changes suggestive of the disease includes White villi and/or spots (dilated lacteals), white nodules, and submucosal elevations are observed. Xanthomata’s plaques are often visualized, there are no specific treatments for patients with PIL. treatment of patients with primary intestinal lymphangiectasia involves control of symptoms with the use of dietary, pharmaceutical, and behavioral modifications.

scholarly journals Dyke-Davidoff-Masson-Syndrome Manifested by seizures and learning difficulties: A Case Report

2013 ◽  
Vol 5 (2) ◽  
pp. 111-113
Author(s):  
Mohammad Ibrahim Khalil ◽  
Maliha Hakim ◽  
Afzal Momin ◽  
Abu Nayeem ◽  
Md. Mohitul Islam ◽  
...  
Keyword(s):  
Case Report ◽  
Learning Difficulties ◽  
Facial Asymmetry ◽  
Behavioral Changes ◽  
Clinical Feature ◽  
Present Case Report ◽  
Cerebral Hemiatrophy ◽  
Generalized Seizures ◽  
Spastic Hemiplegia ◽  
Magnetic Resonance Imaging Mri

Dyke-Davidoff-Masson Syndrome (DDMS), also known as cerebral hemiatrophy, is a rare clinical condition characterized by seizures, facial asymmetry, contralateral spastic hemiplegia or hemiparesis, with learning difficulties and behavioral changes. It is commonly diagnosed in presence of clinical feature associated radiological findings which include cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. In this present case report a 12 year old teenager female patient was diagnosed as DDMS with recurrent generalized seizures, learning difficulties and right sided hemiparesis. Magnetic resonance imaging (MRI) of the brain showed hemiatrophy involving the left cerebral hemisphere and EEG showed generalized epileptiform discharged. An X-ray of the paranasal air sinuses was normal.DOI: http://dx.doi.org/10.3329/jssmc.v5i2.20768J Shaheed Suhrawardy Med Coll 2013;5(2):111-113

scholarly journals A rare cause of right iliac fossa lump: contained fish bone perforation of caecum

2020 ◽  
Vol 7 (10) ◽  
pp. 3476
Author(s):  
Washim F. Khan ◽  
Sandeep Jain ◽  
Yashwant S. Rathore ◽  
Sunil Chumber
Keyword(s):  
Foreign Body ◽  
Foreign Bodies ◽  
Iliac Fossa ◽  
Exploratory Laparotomy ◽  
Right Hemicolectomy ◽  
Fish Bone ◽  
Rare Entity ◽  
Radiological Findings ◽  
Generalized Peritonitis ◽  
High Index Of Suspicion

Ingested foreign bodies usually pass uneventfully through the gastrointestinal tract but few of them can cause symptoms. They can get stuck at acute angulations or narrow part of intestine and can perforate leading to localized to generalized peritonitis, collection or abscess formation. We describe a case of 59 year old gentleman who presented with pain in right iliac fossa with fever and a hard, tender lump. Initial investigation revealed a mass in right iliac fossa adherent to anterior abdominal was in right iliac fossa region with a foreign body inside. Patient was managed with exploratory laparotomy, removal of a fish bone from cacecum and limited right hemicolectomy. Fishbone perforation of caecum is a rare entity. Careful corroboration between patient’s presentation and radiological findings with a high index of suspicion is needed for pre-operative diagnosis.

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