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Aphasia after hemispherectomy in an adult with early onset epilepsy and hemiplegia.

A 55 year old left handed man with left hemisphere subcortical encephalomalacia, seizures, language impairment, and right hemiparesis from a motor vehicle accident at age five was evaluated for epilepsy surgery. The patient continued to speak and followed commands during a left intracarotid amobarbital test (IAT). Left functional hemispherectomy resulted in expressive aphasia. Based on postoperative outcome, language was bilateral. The injury after primary development of language function, the predominantly subcortical lesion, and the late timing of surgical intervention well past development and plasticity may have been factors in the emergence of postoperative aphasia.

Accidents, Traffic↗

[Hemiparetic cerebral palsy and startle epilepsy].

OBJECTIVES: We analyzed electroclinical features and evolution in nine patients with hemiparetic cerebral palsy associated with SE. PATIENTS AND METHODS: Nine patients with mean age 12.3 years and a follow up from 1 to 11 years were studied, analyzing etiology, neurological examination, psychometric evaluation, age at onset and semiology of epileptic seizures, EEGs and neuro-radiological findings, response to treatment and evolution. RESULTS: Etiologies were: porencephaly in 4 cases, hypoxic-ischemic encephalopathy in 2, bacterial meningitis in 1, herpetic encephalitis in 1 and meningo-encephalitis in the last. All cases had mental retardation. Mean age at onset of epileptic seizures was 4 years. Mean age at onset of startle seizures was 6. The startle seizures were characterized by sudden tonic contractions of the paretic hemibody, provoked by auditory stimulus in 6, somatosensory in 2 and both types of stimulus in 1. Falls were observed in 6 patients. Seizures were daily and always when awake. Unprovoked focal seizures with or without secondary generalization were found in 8 cases, and in 6 they presented previously to the startle seizures. Interictal EEGs showed unilateral spikes in 3, bilateral spikes in 3 and generalized polyspike-wave paroxysms in the other three cases. Ictal EEGs were obtained in 8 of the 9 patients and showed diffuse paroxysms of rhythms 6-11 Hz. Cerebral CT scan and/or MRI revealed extensive unilateral encephalomalacia in 5 and porencephaly in 4. The different antiepileptic schedules were unsuccessful in all cases. Surgery was performed in two patients. They are free of seizures after 1 to 4 years of follow up. CONCLUSION: SE should be considered as a distinctive epileptic syndrome or a particular electro-clinical evolution in patients with a large unilateral brain lesion associated with provoked reflex seizures usually refractory to antiepileptic drugs. Epileptic surgery should be considered.

Cerebral Palsy↗

PHACE syndrome: report of one case.

PHACE syndrome indicates a neurocutaneous syndrome including Posterior fossa malformation, large facial Hemangioma, Arterial malformation, Coarctation of the aorta and/or other Cardiac anomalies, and Eye abnormalities. We here report a female infant who presented a giant facial hemangioma. Dandy-Walker malformations with hydrocephalus was found subsequently. We treated the hemangioma with prednisolone, methylprednisolone, interferon-alpha, and local injections of corticosteroid. Encephalomalacia was found later and it was attributed to carotid artery anomalies. Brain image studies, thorough ophthalmologic and cardiac examinations are strongly recommended in infants with large, plaquelike facial hemangiomas. Carotid angiography should also be considered if carotid artery anomalies are suspected.

Abnormalities, Multiple↗

Spontaneous superficial parenchymal and leptomeningeal hemorrhage in term neonates.

BACKGROUND AND PURPOSE: Intracranial hemorrhage in term neonates often results from asphyxia, obvious birth trauma, blood dyscrasia, or vascular malformation but may occur without an obvious inciting event. In this study, we review the clinical and neuroimaging features of healthy term neonates presenting with spontaneous superficial parenchymal and leptomeningeal (ie, subpial or subarachnoid) hemorrhage. METHODS: The clinical records and neuroimaging studies of seven term neonates with spontaneous superficial parenchymal and leptomeningeal hemorrhage were retrospectively reviewed. All underwent diffusion-weighted MR imaging and 6 underwent CT within 72 hours of birth. Magnetic susceptibility-weighted imaging was performed in five, MR angiography in two, and MR venography in two. Follow-up MR imaging was performed in one infant. Clinical follow-up was done in four patients. RESULTS: All neonates had normal birth weights and high 5-minute APGAR scores. All were delivered vaginally (one with forceps assistance, and one with vacuum assistance). No blood dyscrasias were noted. Within 36 hours after delivery, all neonates presented with apnea or seizures or both. Neuroimaging subsequently revealed superficial parenchymal and leptomeningeal hemorrhage. Four occurred in the anterior-inferior-lateral temporal lobe adjacent to the pterion. The remaining three were located in the parietal lobe, frontal lobe, and lateral temporal lobe under the squamosal suture. Decreased diffusion in parenchyma adjacent to the hemorrhage and overlying subcutaneous soft-tissue swelling were apparent in five patients. Susceptibility-weighted imaging showed no additional lesions. MR angiography and MR venography findings were normal. Follow-up MR imaging in one patient demonstrated encephalomalacia. Four patients with short-term clinical follow-up were neurologically normal. CONCLUSION: Spontaneous superficial parenchymal and leptomeningeal hemorrhage occurs in otherwise healthy term neonates. The hemorrhage is most often in the temporal lobe and in proximity to sutures, accompanied by overlying soft-tissue swelling and adjacent decreased diffusion. This pattern leads us to implicate local trauma with contusion or venous compression or occlusion in the development of these hemorrhages.

Brain↗

[Clinical features and surgical treatment of posttraumatic epilepsy].

OBJECTIVE: To study the clinical features of posttraumatic epilepsy and evaluate the surgical procedures for anti-epileptic treatment and their therapeutic effects. METHODS: Neurological and scalp EEGs were performed in 35 patients with posttraumatic epilepsy, who also underwent CT, MRI examination, single photon emission computerized tomography (SPECT) and positron emission tomography (PET) prior to intracranial surgery, with intraoperative monitoring of the cortical EEG. RESULTS: With the cortical EEG monitoring, 16 patients underwent surgical resection of the epileptogenic foci identified by intraoperative EEG, 7 had multiple subpial transection and 12 received stereotactic radiosurgery for the epileptogenic foci localized by PET. In most of the cases, the epileptogenic foci were located around the lesions of encephalomalacia. During the follow-up of 32 patients varying from 1 to 4 years, 18 patients became seizure free, 10 had obvious reduction in the frequency of seizure while 4 failed to respond favorably to the treatment. CONCLUSIONS: Seizures of posttraumatic epilepsy may aggravate the brain dysfunction due to primary trauma, and surgical treatment often yields good effect and lessens the toxic and adverse effect of antiepileptic drugs. Stereotactic radiosurgery guided by PET is safe and effective in the treatment of patients with posttraumatic epilepsy.

Adolescent↗

[Neurological symptoms of cytomegalovirus infection in children].

Cytomegalovirus is considered as one of the most common causes of congenital infections. Clinical signs of infection are present in newborns whose mothers were infected during pregnancy. Disease is most severe if mother were infected during the first trimester. Congenital cytomegalovirus infection causes damage of CNS (central nervous system) and the liver, atrophy of optical nerve and hearing retardation up to complete loss. The most severe and frequent damage is of CNS. Under our observation were 20 children of different ages, among them 8 newborns. Verification of cytomegalovirus infection was done by ELISA. Polymerase Chain Reaction (PCR) was also used for diagnostic reasons. Examination of CNS was performed by neurosonography, electroencephalography and computed tomography. Four from eight newborns died at the age of 6-8 weeks (in these cases the generalized form of infection with severe damage of CNS was present). In the rest patients treatment with specific anticytomegalovirus immunoglobulin together with syndrome therapy yielded positive effect. Lately moderate retardation of phychomotor development became apparent in all cases. 12 patients, who come to our clinic in the period from 2 months to 1.5 years of age, psychomotor retardation, micro or hydrocephaly, hyperkinesias, absentia (epileptica), convulsive syndrome and malignant hypertension were present. On tomograms intracranial calcificats and cysts, also atrophy of cerebrum and encephalomalacia was visible. Sensory hearing loss was apparent in two patients. The study showed the presence of severe and in most cases irreversible CNS damages in the cases of congenital cytomegalovirus infection.

Central Nervous System↗

Acute and chronic brain infarcts on MR imaging in a 20-year-old woman with acute posterior multifocal placoid pigment epitheliopathy.

A 20-year-old woman recently diagnosed with acute posterior multifocal placoid pigment epitheliopathy developed headaches, weakness, and paresthesias. MR imaging of the brain revealed an acute infarct (demonstrated by diffusion-weighted images) in the head of the right caudate nucleus, a chronic infarct with encephalomalacia in the body of the corpus callosum, and multiple foci of abnormal signal intensity in the white matter of the centrum semiovale.

Acute Disease↗

[Transient ischemic attacks (TIA) from the viewpoint of the clinical neurologist].

A transient ischaemic attack (TIA) is a cerebrovascular attack the clinical symptomatology of which is restored to normal within 24 hours. Minor haemorrhages, epilepsy, hypoglycaemia, migraine and even peripheral neuropathies can be mistaken for TIA. CT examination indicates in a considerable percentage of examined subjects encephalomalacia. The term TIA should be reserved for ischaemic attacks, where after regression of the clinical symptomatology within 24 hours no structural changes are found.

Humans↗

Occurrence of runting and stunting syndrome in broiler chickens in the Sudan.

The first outbreak of reovirus infection in broiler chickens was observed in the Sudan in the largest poultry industry of the country. The disease was characterized clinically by growth retardation, lameness, poor feathering, shank depigmentation and occasionally retraction of the head. Grossly there was enlargement of the proventriculus, atrophy of the pancreas and bone abnormalities. Histopathologic changes included hepatitis, nephritis, myocarditis, pericarditis, catarrhal enteritis, pancreatic necrosis, encephalomalacia and ricketic changes. Details were described. Reovirus was isolated from affected birds and reisolated from experimentally infected chicks. Infection was confirmed by immunoelectrophoresis and agar gel precipitation tests.

Animals↗

The origin and pathogenicity of Basidiobolus species in northern Australia.

Basidiobolus haptosporus Drechsler causes human and animal disease in the tropics. This paper reports the isolation of this organism from natural substrates and describes the pathogenicity of the isolates. Basidiobolus was recovered from faecal samples of amphibians, reptiles and macropods, from woodlice, and from granulomatous skin lesions of horses. Some isolates were heat-tolerant. Almost all of the heat-tolerant isolates were pathogenic to suckling mice and had smooth or undulate, or smooth plus undulate zygospore walls. When inoculated intracerebrally into suckling mice they caused encephalomalacia, necrosis, meningoencephalitis, congestion, haemorrhage and a granulomatous reaction in the central nervous tissue, hydrocephalus and nervous signs. There was a variety of animal sources of pathogenic strains of the organism which were widely distributed in the sampling area, thus the potential hazard of infection could be more serious than previously considered.

Animals↗

Immunoreactive A4 and gamma-trace peptide colocalization in amyloidotic arteriolar lesions in brains of patients with Alzheimer's disease.

Cerebral amyloid angiopathy (CAA) defines a biochemically heterogeneous entity that manifests as effacement of cerebral microvessel walls by a fibrillar material with characteristic tinctorial properties. In biochemical terms, the amyloid that infiltrates blood vessels in CAA is composed of the A4 or beta peptide of Alzheimer's disease (AD), a molecule related to gamma trace or cystatin C (seen in patients with hereditary cerebral hemorrhage with amyloidosis in Iceland, HCHWA-I), or the PrP characteristic of spongiform encephalopathy and scrapie. Using antibodies to synthetic peptides representing portions of the 4.2-kd Alzheimer A4 peptide and the gamma-trace peptide, we immunostained sections of brain from patients with AD, senile dementia of Alzheimer's type, and CAA with associated leukoencephalopathy. Immunohistochemical studies demonstrated colocalization of the A4 and gamma-trace peptides within arteriolar walls, but only rarely in A4 amyloidotic capillaries or senile plaque cores of amyloid. When gamma-tracelike reactivity was noted in capillary walls, it was sometimes noted within the cytoplasm of pericytes. Immunostaining was always more intense when the anti-A4 antibody was used as the primary antibody. Gamma-trace immunostaining was more prominent on the adventitial component of arteriolar walls, whereas A4 staining was usually seen more diffusely throughout the blood vessel wall, especially in the media. Rarely individual pericytelike cells showed prominent gamma-trace immunoreactivity. These findings suggest that A4 and gamma-tracelike molecules may colocalize within arteriolar walls within the brains of patients with AD, and highlight the fact that CAA identified with AD and HCHWA-I are not as biochemically distinct as was assumed previously. Furthermore these findings suggest that other peptidases or protease inhibitors may be found within amyloidotic microvessel walls and may contribute to senile brain change and CAA-related strokes, including hemorrhage and encephalomalacia.

Aged↗

[Cerebral toxoplasmosis in subjects with acquired immunodeficiency syndrome].

Cerebral toxoplasmosis is the most common cause of focal CNS disease complicating AIDS and may afflict 10% to 33% of such patients. We present the clinical, neuroradiological and immunological findings in 5 cases with cerebral toxoplasmosis complicating the acquired immune deficiency syndrome. All patients had focal neurological signs and symptoms; CT scan findings included single or multiple lesions with ring contrast enhancement, mass effect and oedema. CSF analysis showed increased protein levels, decreased glucose levels and pleocytosis; CSF IgG antitoxoplasma antibodies were positive in 4 patients; serum IgG antitoxoplasma antibodies were positive in all patients, while IgM resulted always negative in serum and in CSF. After therapy, in 2 cases CT scans showed small areas of encephalomalacia replacing the abscesses. We think that non-invasive techniques (CT scans, CSF and serum immunochemical tests) together with the finding of a favourable response to therapy may be useful for the diagnosis of cerebral toxoplasmosis, allowing to avoid invasive technique such as cerebral biopsy.

Acquired Immunodeficiency Syndrome↗

[Transcranial Doppler sonography combined with other ultrasonic methods in the diagnosis of stroke].

The use of transcranial Doppler sonography (TDS) in combined ultrasound diagnosis was the first to allow a noninvasive assessment of cerebral circulation. Altogether 50 patients in acute stroke period were examined. The results obtained were verified at surgery, cerebral angiography or autopsy. Changes in the velocity of the blood flow, its reversal, different Doppler sonogram pattern appeared controversial for cerebral and subarachnoid haemorrhages caused by the occlusion of the internal carotid artery or other factors of encephalomalacia. TDS employment promotes the improvement in the diagnosis of the cause underlying the acute impairment of cerebral circulation and in the prognosis of cerebral coma. The findings are correlated with routine echo-pulsography and Doppler sonography.

Carotid Artery Thrombosis↗

Experimental approach of treatment of cerebral ischemia by a combination of raubasine and almitrine.

Post-oligemic syndrome in rat results from transient reduction of cerebral blood flow obtained by bilateral carotid ligation for 60 min and mild systemic hypotension induced by s.c. injection of sodium nitroprusside. Over an acute phase of 3 days the syndrome consisted in a marked neurological deficit evidenced by a decrease in scores obtained by rats in "visual placing" test, a loss in learning ability substantiated by a decrease in the ability of rats to integrate and remember a passive avoidance reflex; biochemical disorders revealed by an increase in cerebral water, calcium and free fatty acids (FFA) contents associated with a decrease in cerebral potassium content. From 2 weeks until 4 weeks after ischemic insult a process of malacia of the cerebral tissue occurred. Treatment of rats with a combination of raubasine and almitrine (5023 SE, Duxil) by oral route (16.7 to 66.6 mg kg-1) twice daily from 1 h post-oligemia to sacrifice, in the acute phase, led to 1. an improvement of visual placing response, 2. an increase in learning ability, 3. a decrease in water and calcium cerebral contents associated with an increase in potassium cerebral contents. Enhancement of FFA contents was reduced when preventive treatment was associated with curative treatment. Incidence and extent of encephalomalacia was reduced by the drug in the chronic phase. It is concluded that in a rat model of post-oligemic syndrome and under these experimental conditions 5023 SE caused, 3 days after the ischemic insult, a significant reduction of cerebral disorders that may explain the therapeutic effect of the drug evidenced 4 weeks later.

Almitrine↗

The first two decades of vitamin E.

This presentation reviews highlights of the first 20 years (1922-1942) of vitamin E. It begins with background information leading to identification of an antisterility factor for rats of both sexes and its acceptance into the vitamin family as vitamin E (1925). Research of the next 12 years revealed a multiplicity of deficiency manifestations: embryonic mortality, testis degeneration, encephalomalacia and exudative diathesis in the chick, and nutritional muscular dystrophy in avian and mammalian species. Toward the close of this period came the isolation of vitamin E from natural sources, determination of its empirical formula, and introduction of the designation alpha-tocopherol for vitamin E (1936). Within the next two years the structural formula of alpha-tocopherol was elucidated, its chemical synthesis accomplished, and its production from natural plant oils by molecular distillation was well established. The existence of other tocopherols with lesser degrees of biological activity became recognized. Also, the concurrent development of a chemical method for determining the vitamin E content of alpha-tocopherol in foods, body tissues and body fluids, which replaced the very laborious bioassay procedure, greatly facilitated later advances in knowledge of the distribution and nature of vitamin E.

Animals↗

[Successful treatment of a brain abscess with antibiotics and drainage puncture in an 11-year-old boy with a complex cyanotic heart defect].

A Blalock-Taussig-anastomosis was performed at the age of 2 years in a boy with transposition of the great arteries, ventricular septal defect, and pulmonary atresia. Nine years later he developed a transient aphasia. Cranial computed tomography (CT-scan) revealed a structure compatible with brain abscess. The boy was conscious and no neurological deficit was found. Initial therapy consisted of Ampicillin, Tobramycin, and Metronidazole, 12 days later an acute hemiplegia developed. 30 ml of pus were aspirated from the brain abscess, and the boy's condition dramatically improved. Streptococcus milleri was found bacteriologically and antibiotic therapy was continued over six weeks. Serial CT-scans during and after therapy demonstrated disappearance of the brain abscess. The presented case shows that after a shunt procedure in cyanotic heart disease right-to-left-shunting and therefore chronic oxygen desaturation and polycythemia are still present as facilitating factors for focal encephalomalacia, cerebritis, and brain abscess. In case of short duration of neurological symptoms and a size of abscess less than 4 cm in diameter antibiotic therapy without total excision may eliminate the infection.

Anti-Bacterial Agents↗

[Quantitative determination of cells in sterile skin exudate in acute cerebrovascular diseases].

Rebuck's test was done in 126 subjects (40 healthy controls and 86 patients with acute cerebrovascular disease) on both upper extremities. In the control group no statistically significant quantitative difference was found in the number of cells in the exudate on both sides. In the patients, however, the number of cells in the exudate was greater on the paralysed extremity. The reaction dependent on the degree of paralysis, coexistence of sensory and autonomic disturbances, but was independent of the form of acute cerebrovascular disease (haemorrhage, encephalomalacia) and the time after the onset of the disease.

Acute Disease↗

CT of neonatal herpes encephalitis.

Seven neonates with proven herpes (HSV type 2) encephalitis exhibited a characteristic sequence of findings on cranial computed tomography (CT). The initial CT abnormalities in all infants were fingerlike areas of cortical increased attenuation noted on unenhanced scans obtained 2-30 days after presentation. These usually were accentuated by increased white-matter lucency. Subsequently (more than 30 days after presentation), extensive cerebral destruction, multicystic encephalomalacia, and calcification were seen on follow-up CT scans obtained in five infants.

Brain↗