scholarly journals Dyke-Davidoff-Masson Syndrome with crossed cerebellar atrophy

2021 ◽  
Vol 18 (3) ◽  
pp. 73-75
Author(s):  
Rohit Kumar ◽  
Deepak Kumar ◽  
Himanshu Mishra ◽  
Sanjay Kumar Suman ◽  
Umakant Prasad
Keyword(s):  
Early Childhood ◽  
Mental Retardation ◽  
Frontal Sinus ◽  
Cerebellar Atrophy ◽  
Cerebral Atrophy ◽  
Facial Asymmetry ◽  
Mri Findings ◽  
Neurological Condition ◽  
Mri Brain ◽  
Cerebral Insult

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare neurological condition characterised clinically by recurrent seizures, facial asymmetry, hemiplegia and mental retardation likely due to foetal or early childhood cerebral insult. We describe the MRI findings of DDMS in a 10-year-old male child. MRI brain revealed right cerebral atrophy, ipsilateral thickening of calvarium, right lateral ventricular dilatation, hyper-pneumatisation of frontal sinus, and contralateral cerebellar atrophy which are consistent with DDMS.

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 Clinical characteristics and neuroimaging findings of seven patients with Dyke Davidoff Masson syndrome

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Bing Wang ◽  
Wentao Jiang ◽  
Weiqiang Yan ◽  
Jianhong Tian ◽  
Jianxing Xu ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Behavioral Disorders ◽  
Clinical Characteristics ◽  
Psychiatric Symptoms ◽  
Neuropsychiatric Symptoms ◽  
Clinical Manifestations ◽  
Cerebral Atrophy ◽  
Facial Asymmetry ◽  
Compensatory Hypertrophy ◽  
Neuropsychiatric Manifestations

Abstract Background DDMS is a rare disease diagnosed by clinical and radiological characteristics. But the complexity of radiological and clinical manifestations of DDMS has become a challenge diagnostically. To date, the reported cases with DDMS had highly varied clinical manifestations including seizures, contralateral hemiplegia/hemiparesis, facial asymmetry, mental retardation, etc. In addition to typical clinical findings, some new characteristics have been recently added to the spectrum of DDMS. However, few cases have been reported to be associated with neuropsychiatric symptoms according to the literature. This study aimed to investigate the neuropsychiatric manifestations associated with Dyke-Davidoff-Masson syndrome (DDMS) and related imaging findings. Methods This study included 7 patients diagnosed with DDMS between 2014 and 2020. The clinical characteristics, neuropsychiatric manifestations, and radiological results were retrospectively evaluated. Results Seven patients (five males and two females) with a mean age of 28.0 ± 9.73 (range 15.0–41.0) years were included. Five patients were admitted to the psychiatric unit due to psychological and behavioral disorders. Two patients were referred to the neurology unit mainly due to epilepsy. Six patients had epileptic seizures, 4 had hemiplegia, 3 had mental retardation, 2 patients had external ear deformities, and 2 had facial asymmetry. Neuropsychiatric symptoms were presented in 6 (85.7 %) cases. Cases 2–6 developed affective disorders. Deficits in verbal communication, impairment of social interaction, lack of insight, adulia and hypobulia appeared in cases 1–4. Schizophrenia with apathy, and epileptic schizoid psychosis were observed in cases 4 and 5 respectively. Case 6 had behavioral disorders, hyperactivity, tic disorder, mental retardation, anxiety, catatonic symptoms and suicidal tendency. Case 7 had seizures and mental retardation, and no psychiatric symptoms were presented. Radiological examinations showed unilateral cerebral atrophy, enlarged lateral ventricles, and various compensatory hypertrophy of the skull in all cases. The midline structure has shifted to the affected side in 5(71.4 %) cases. Atrophy of the basal ganglia or brain stem was observed in 4(57.1 %) cases. Conclusions The hallmark imaging manifestations of DDMS facilitated the diagnosis in most cases. This study illustrated that a variety of psychoneurotic disorders and ear abnormalities were correlated with DDMS.

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.

Coprophagia in an Elderly Man: A Case Report and Review of the Literature

2005 ◽  
Vol 35 (4) ◽  
pp. 417-427 ◽  
Author(s):  
David A. Beck ◽  
Nora R. Frohberg
Keyword(s):  
Mental Retardation ◽  
Behavioral Interventions ◽  
Selective Serotonin Reuptake Inhibitors ◽  
Cerebral Atrophy ◽  
Laboratory Evaluation ◽  
Psychiatric Evaluation ◽  
Obsessive Compulsive ◽  
Supportive Psychotherapy ◽  
Compulsive Disorder ◽  
Medical Disorders

Objective: Coprophagia or the ingestion of feces has long been associated with psychiatric illness. It is considered to be a variant of pica. This behavior requires an extensive medical and psychiatric differential diagnosis. Medical disorders associated with coprophagia include seizure disorders, cerebral atrophy, and tumors. Psychiatric disorders associated with coprophagia include mental retardation, alcoholism, depression, obsessive compulsive disorder, schizophrenia, fetishes, delirium, and dementia. In animals, coprophagia is associated with boredom, thiamine deficiency, and lesions of the amygdala. Methods: A case of coprophagia in an elderly man is reported here. A 77-year-old man with mild mental retardation was referred for urgent psychiatric evaluation due to coprophagia. The case is discussed and the literature reviewed. Results: Psychiatric evaluation revealed cognitive dysfunction and depression. Physical examination and laboratory evaluation were noncontributory. He was started on sertraline 25 mg daily with resolution of his coprophagia. Coprophagia has been treated using behavioral interventions, supportive psychotherapy, elemental diets, tricyclic antidepressants, carbamazepine, haloperidol, and electroconvulsive therapy. Conclusions: Use of Selective Serotonin Reuptake Inhibitors (SSRIs) may also be an effective treatment for coprophagia, particularly in the setting of depression or anxiety.

scholarly journals Dementia with Leukoencephalopathy in Systemic Lupus Erythematosus

1991 ◽  
Vol 18 (3) ◽  
pp. 344-348 ◽  
Author(s):  
Andrew Kirk ◽  
Andrew Kertesz ◽  
Marsha J. Polk
Keyword(s):  
White Matter ◽  
Lupus Erythematosus ◽  
Memory Loss ◽  
Cerebral Atrophy ◽  
Hepatic Function ◽  
Subcortical White Matter ◽  
Oligoclonal Bands ◽  
Mri Findings ◽  
Recent Onset ◽  
Blood Brain Barrier Disruption

ABSTRACT:Neurologic manifestations, afflicting up to 70% of SLE patients, include psychosis, seizures, chorea, neuropathies, and stroke. MRI is useful in evaluation of lupus patients and several reports have documented cerebral atrophy or focal hyperintensities. We report an unusual MRI appearance in a 56-year-old woman with SLE, diagnosed on the basis of pleuritis, lymphopenia, anti-DNA antibodies, and neurologic involvement. She reported recent onset of Raynaud's phenomenon and generalized macular rash. She presented after two months of gradual deterioration with memory loss, flattened affect, dysphagia, dysarthria, anomia, and somnolence, without focal neurologic signs. Investigations included elevated ESR, reduced complement, normal CSF without oligoclonal bands, negative viral serology, normal hormone and vitamin levels, normal renal and hepatic function. Neuropsychologic testing showed widespread impairment (WAIS-R: FSIQ-63; WMS-69; DRS-98; RCPM-14; WAB AQ-78.8). CT was normal but MRI showed strikingly symmetric, confluent hyperintensities extensively involving cerebral and cerebellar white matter on Tl and T2 weighted scans. Basal ganglia and subependymal and subcortical white matter were spared. Treated with prednisone, the patient made a gradual, but incomplete, recovery. These MRI findings may reflect widespread vasculopathy or direct immunologic brain insult with or without imunologic blood-brain barrier disruption.

scholarly journals Early childhood MRI findings in complex partial seizures and hippocampal sclerosis

1999 ◽  
Vol 10 (1) ◽  
pp. 93-96
Author(s):  
J�rn H. Risse ◽  
Christian Menzel ◽  
Frank Gr�nwald ◽  
Dierk Brechtelsbauer ◽  
Burkhard Ostertun ◽  
...  
Keyword(s):  
Early Childhood ◽  
Hippocampal Sclerosis ◽  
Partial Seizures ◽  
Mri Findings ◽  
Complex Partial Seizures

Cerebellar atrophy: Relationship to aging and cerebral atrophy

Neurology ◽  
1981 ◽  
Vol 31 (11) ◽  
pp. 1486-1486 ◽  
Author(s):  
W. C. Koller ◽  
S. L. Glatt ◽  
J. H. Fox ◽  
A. W. Kaszniak ◽  
R. S. Wilson ◽  
...  
Keyword(s):  
Cerebellar Atrophy ◽  
Cerebral Atrophy

scholarly journals Encephalopathy and Neuropathy due to Glue, Paint Thinner, and Gasoline Sniffing in Trinidad and Tobago-MRI Findings

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Kanterpersad Ramcharan ◽  
Amrit Ramesar ◽  
Moshanti Ramdath ◽  
Joel Teelucksingh ◽  
Maria Gosein
Keyword(s):  
Clinical Evidence ◽  
Cerebellar Atrophy ◽  
Cerebral White Matter ◽  
Radiological Findings ◽  
Mri Findings ◽  
Petrol Station ◽  
Sensorimotor Polyneuropathy ◽  
History Of ◽  
Cortex Cérébral ◽  
Magnetic Resonance Brain Imaging

A 29-year-old male petrol station pump attendant was admitted with ataxia and clinical evidence of a sensorimotor polyneuropathy which developed over the preceding 3 months. He had cognitive dysfunction, hearing loss, and cerebellar clinical abnormalities that came on slowly over the three years. He had a fifteen-year history of sniffing mostly glue, occasionally paint thinners, and, in the recent two years, gasoline. Magnetic resonance brain imaging showed abnormalities of the cerebral cortex, cerebral white matter, corpus callosum, hippocampus, brainstem and cerebellar atrophy, hypointensities of basal ganglia, red nuclei, and substantia nigra as previously described in toluene sniffing. Abstinence for six months led to partial clinical improvement. Clinicians need to be aware of this preventable entity which has peculiar radiological findings which are being increasingly accepted as typical.

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