ABSTRACT
Dyke-Davidoff-Masson syndrome ( DDMS) is a rare epilepsy syndrome which is characterized by cerebral hemiatrophy with ipsilateral compensatory skull changes and contralateral hemiplegia recurrent .Here we reported a case of which the curative effect turned out to be unsatisfied after conservative treatment , physical examination re-vealed mental retardation , facial asymmetry , mild right hemiparesis , MRI scan of the head showed left cerebral hemiatrophy , calvaria thickening , PET-CT showed less functional left cerebral hemisphere .The patient finally un-derwent multi-lobe disconnection to relieve recurrent seizure , whose seizure was well controlled in the following up of one year .
ABSTRACT
DDMS is a rare syndrome 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. Dyke-Davidoff- Masson Syndrome (DDMS) is one among the syndromes associated with refractory epilepsy. We report a case of DDMS in an 18 year old female who presented with seizures, hemiparesis of the right side and mental retardation. Computed tomography on this patient assisted in making a diagnosis of DDMS.
ABSTRACT
A newborn presented at 14 hrs of age with right sided clonic seizures and shrill cry. Magnetic Resonance Imaging of the brain showed left cerebral hemiatrophy with cystic changes in left fronto-parietal lobe and parasylvian region. The Magnetic Resonance Angiography revealed hypoplasia of left supraclinoid Internal Carotid Artery and hypoplasia and irregularity of vessel wall affecting the left Middle Cerebral Artery. Such an early presentation of this rare disorder has not been reported previously. Recognition of this anomaly has important implications during carotid and trans-sphenoidal surgery, in thromboembolic disease, and in the surveillance and detection of associated cerebral aneurysms.
Subject(s)
Apgar Score , Carotid Artery, Internal/abnormalities , Carotid Artery, Internal/pathology , Cerebral Angiography/methods , Follow-Up Studies , Humans , Imaging, Three-Dimensional/methods , Infant, Newborn , Magnetic Resonance Angiography/methods , Male , Phenobarbital/therapeutic use , Recurrence , Risk Assessment , Seizures/congenital , Seizures/diagnosis , Seizures/drug therapy , Seizures/etiology , Treatment Outcome , Vascular Malformations/complications , Vascular Malformations/diagnosis , Term BirthABSTRACT
We present a case of cerebral germinoma that occurred in the basal ganglia with progressive hemiparesis. A 10-year-old boy was referred to our hospital complaining of progressive right hemiparesis and decreased mental functions. Brain MRI showed left cerebral hemiatrophy and high signal intensity in left thalamus, basal ganglia, cerebral peduncle, and pons. However, no mass or enhancement was observed. 11C-Methionine positron emission tomography showed discernable hot uptake in the left basal ganglia and thalamus. Stereotactic biopsy, with a guide of 11C-Methionine positron emission tomography, was performed and the diagnosis of pure germ cell tumor was determined. 11C-Methionine positron emission tomography is useful for localization of a suspicious tumor in patients with cerebral hemiatrophy without overt mass or enhancing lesions in brain MRI.
Subject(s)
Child , Humans , Male , Basal Ganglia , Biopsy , Brain , Diagnosis , Electrons , Germinoma , Magnetic Resonance Imaging , Neoplasms, Germ Cell and Embryonal , Paresis , Pons , Positron-Emission Tomography , Tegmentum Mesencephali , ThalamusABSTRACT
Incontinentia pigmenti is an uncommon genodermatosis presented by characteristic cutaneous stages and frequent association with one or more ectodermal or mesodermal anomalies. A new-born girl, 2 days old, had had crops of erythematous papules and vesicles on the trunk and extremities. At 2 years of age, she had the whorled hyperpigmentation. There were no anomalous features in teeth, eyes and hair, but she had a neurologic disorder. Left hemiplegia, related to upper motor neuron weakness, showed brisk left DTR, spastic Achilles tendon with walking disorders, decreased grasping power of left hand and weakness of left upper extremity. Brain MRI revealed marked loss of volume in the white matter of the right lateral ventricle in T1 weighted images. We report herein an explicit case of incontinentia pigmenti associated with right cerebral hemiatrophy.
Subject(s)
Female , Humans , Achilles Tendon , Brain , Ectoderm , Extremities , Hair , Hand , Hand Strength , Hemiplegia , Hyperpigmentation , Incontinentia Pigmenti , Lateral Ventricles , Magnetic Resonance Imaging , Mesoderm , Motor Neurons , Muscle Spasticity , Nervous System Diseases , Tooth , Upper Extremity , WalkingABSTRACT
A 20-years old man with a history of complex partial seizure, was admitted due to continuous focal clonic movements in the left upper and lower extremities lasting for about 24 hours. In emergency room, focal clonic movements were stopped with an emergent intravenous lorazepam injection. Two months later, left-sided hemiparesis remained and cere-bral hemiatrophy was noted. We report a case of a 20-years old man who showed rapid development of cerebral hemiat-rophy after epilepsia partialis continua.
Subject(s)
Humans , Young Adult , Emergency Service, Hospital , Epilepsia Partialis Continua , Lorazepam , Lower Extremity , Paresis , Seizures , Status EpilepticusABSTRACT
Dyke-Davidoff-Masson syndrome is a rare disorder which developed hemiatrophy of brain, enlargement of ventricles and prominent pneumatization of petrous pyramid of the temporal bone. It manifests hemiparesis, varying type seizure, and mental retardation. We experienced two cases of Dyke-avidoff-Masson Syndrome, in a 6/12 year-old boy and a 1, 10/12 year-old girl who were admitted to our department of pediatrics, because of convulsion and hemiparesis The brain CT scan showed marked dilatation of lateral ventricle, prominent sylvian fissure and cortical sulci. So we report two cases of Dyke-Davidoff-Masson syndrome with review of literature.