Focal Motor Deficits Research Articles

Head tremor and progressive multifocal leukoencephalopathy in AIDS patients: report of two cases

Progressive multifocal leukoencephalopathy (PML) is caused by replication of JC virus in oligodendrocytes of immunocompromised patients. Common manifestations are focal motor and sensory deficits, gait abnormalities, speech and language disturbances, cognitive disorders, headache, and visual impairment. Although the occurrence of movement disorders is rare in PML, bradykinesia, rigidity, dystonia, myoclonic jerks and myoclonic ataxia have been described. Head tremor associated with PML has not been previously reported. We report two cases of PML in whom head tremor was present.

Extranodal Rosai-Dorfman Disease Involving the Meninges

Rosai-Dorfman disease is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Extranodal involvement has also been reported. We report the case of a 57-year-old woman presenting with seizures, focal motor deficits, and leptomeningeal enhancement on magnetic resonance imaging. Meningeal biopsy revealed Rosai-Dorfman disease. Sinus histiocytosis may mimic meningeal disease, and Rosai-Dorfman disease should be considered in the differential diagnosis of dural-based masses with diffuse leptomeningeal enhancement.

Extranodal Rosai-Dorfman Disease Involving the Meninges

Rosai-Dorfman disease is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Extranodal involvement has also been reported. We report the case of a 57-year-old woman presenting with seizures, focal motor deficits, and leptomeningeal enhancement on magnetic resonance imaging. Meningeal biopsy revealed Rosai-Dorfman disease. Sinus histiocytosis may mimic meningeal disease, and Rosai-Dorfman disease should be considered in the differential diagnosis of dural-based masses with diffuse leptomeningeal enhancement.

Intracranial hemangiopericytoma: study of 12 cases.

Most hemangiopericytomas (HPCs) are located in the musculoskeletal system and the skin, while the intracranial location is rare. They represent 2 to 4% in large series of meningeal tumours, thus accounting for less than 1% of all intracranial tumours. Many authors have argued about the true origin of this tumour. The current World Health Organization classification of Central Nervous System tumours distinguishes HPC as an entity of its own, and classified it into the group of "mesenchymal, non-meningothelial tumours". Radical surgery is the treatment of choice, but must be completed with postoperative radiotherapy, which has proved to be the therapy most strongly related to the final prognosis. HPCs have a relentless tendency for local recurrence and metastases outside the central nervous system which can appear even many years after diagnosis and adequate treatment of the primary tumour. Twelve patients with intracranial HPC were treated at our Unit between 1978 and 1999. There were 10 women and 2 men. Ten tumours were supratentorial and most located at frontoparietal parasagittal level. The most common manner of presentation was a focal motor deficit. All tumours were hyperdense in the basal Computed Tomography scans and most enhanced homogeneously following intravenous contrast injection. In 50% of cases, tumour margins were irregular or lobulated. Seven tumours were studied with Magnetic Resonance Imaging, being six of them iso-intense with the cortical gray matter on T1-weighted and T2-weighted images. Twenty operations were performed in the 12 patients. In 10 cases radical excision could be achieved with no operative mortality. Total recurrence rate was 33.3%. Eight patients were treated with external radiotherapy at some time through the course of their disease. Eight out of the 12 patients in this series are disease-free (Glasgow Outcome Scale categories 1 and 2) after a mean follow up of 52 months.

Lesiones cerebrales focales isquémicas o hemorrágicas en el recién nacido a término. Revisión casuística de la última década

Although focal cerebral ischemic or hemorrhagic lesions are infrequent in the term newborn, they must be considered when neurologic symptoms appear, especially when seizures are present. Possible risk factors to suffer from these pathologies have been studied, as well as their evolution, to try to give a prognosis. The term newborns with focal ischemic or hemorrhagic cerebral lesions who presented symptoms in the neonatal period have been studied for 10 years (January 1990-March 2000) in our Children s Hospital Miguel Servet of Zaragoza (Spain). The newborns have been studied in our hospital, and their evolution followed in the Neuropediatric Consulting Room. From each case data about familiar history, pregnancy, labor, clinical manifestations, physical examination, complementary studies, diagnosis and neurodevelopment evolution have been collected. RESULTS. From the nine cases found, four were infarctions of the left medial cerebral artery, and five were hemorrhages. Except in two cases in which an important birth trauma was present, any other antecedent that could be the cause was found. All except one manifested as seizures. Neuroimaging studies visualized the stroke in all of them. A slight motor deficit remains in seven children, and it is severe in one. Cerebrovascular strokes are infrequent in term newborns. Most of the times their etiology is not found. These accidents usually manifestate as focal seizures in the immediate neonatal period, and neuroimaging studies (ECO-TC) are essential for the diagnosis. The long term evolution is favorable in most of the cases, although focal motor deficit remains present frequently.

Gliomatosis cerebri: findings with computed tomography and magnetic resonance imaging

Gliomatosis cerebri (GC), is a rare neoplastic disease (less than 150 cases reported in the literature) with a diffuse, widespread proliferation of neoplastic glial cells in the brain, generally affecting both hemispheres and involving the gray and white matter [1–3]. Less commonly, the cerebellum, the brain stem and the medulla can be affected. Histologic evaluation reveals neoplastic astrocytes with varying levels of differentiation. Perineuronal and perivascular spread of tumor infiltration is observed. Demyelination can be found in the affected areas. A well-preserved underlying neuroanatomic architecture is considered characteristic [2]. Clinical signs vary and are non-specific, including changes in the mental state and headaches, followed by focal motor deficits and convulsive episodes [4]. The prognosis is poor, ranging from weeks to some years after the manifestation of the symptoms. Steroids may be useful in the short term, but chemotherapy is of little value and radiotherapy of questionable benefit. The literature was reviewed and the radiological pattern of three new cases of GC is reported. In two cases the diagnosis was achieved ante-mortem.

Normothermic cardiopulmonary bypass: effect on the incidence of persistent postoperative neurological dysfunction following coronary artery bypass graft surgery

We retrospectively reviewed the records of 250 consecutive patients undergoing coronary artery bypass graft surgery (CABG) from January 1994 through January 1996 to determine the incidence of persistent postoperative neurological dysfunction after CABG and to compare normothermic and moderate hypothermic cardiopulmonary bypass (CPB). Normothermic CPB was used in 128 patients (36°-37°C) and hypothermic CPB (27°-28°C) in 122 patients. Postoperative neurological dysfunction included focal motor deficits, delayed recovery of consciousness (>24h) after surgery, and seizures within 1 week postoperatively. Persistent neurological dysfunction was diagnosed if complete resolution had not occurred within 10 days of surgery. The incidence of persistent postoperative neurological dysfunction was 4.1% in the hypothermic CPB group and 2.3% in the normothermic CPB group. There were no statistically significant differences between the two groups (P=NS). These results suggest that normothermic CPB did not increase the incidence of persistent postoperative neurological dysfunction compared to hypothermic CPB.

Cerebral venous thrombosis in young adults. Experience in a stroke unit, 1988-1994.

We report a series of nine patients younger than 45 years with angiographically-documented cerebral venous thrombosis, consecutively referred to our Stroke Unit from 1988 to 1994. Two of them were men and seven were women. Eight patients initially complained of headache, often associated with other symptoms of increased intracranial pressure. Seven patients had focal motor deficits and four suffered from epileptic seizures. None of the patients died. Only one patient remained severely disabled, whereas the others recovered self-sufficiency within two months. The use of oral contraceptives was closely associated with the occurrence of venous thrombosis in the women. Cerebral venous thrombosis is to be kept in mind in the differential diagnosis of stroke in the young.

Exacerbation or unmasking of focal neurologic deficits by sedatives.

Transient focal neurologic deficits have been observed in patients emerging from brain tumor or carotid surgery, and a pharmacologic effect of anesthetic agents has been proposed as the cause of such neurologic dysfunction. Therefore, the effect of sedation with midazolam or fentanyl on motor neurologic function was studied prospectively and preoperatively in patients with carotid disease or mass lesions of the brain. Fifty-four unpremedicated adult patients with carotid disease or a brain tumor were given small intravenous doses of either 2.8 +/- 1.3 mg midazolam or 170 +/- 60 micrograms fentanyl in the preoperative period. A thorough motor examination was performed at baseline and after sedation by an individual who was unaware of the details of the patient's disease or symptoms. A mental status examination also was performed to control for the effects of inattentiveness or lack of cooperation during the neurologic examination. Patients were sedated mildly but were fully cooperative. Focal motor deterioration occurred after sedation in 30% of patients, and the incidence was similar in patients in the fentanyl and midazolam groups. Among patients with a focal motor abnormality on baseline examination or a resolved prior motor deficit, 73% had exacerbation or unmasking of these signs by sedation, whereas no patient without a prior history of motor dysfunction had a sedative-induced change. Sedative-induced changes in neurologic function ranged from unilateral mild weakness to complete plegia, but appeared to be transient in nature. Sedation with midazolam or fentanyl can transiently exacerbate or unmask focal motor deficits in patients with prior motor dysfunction.

Computed tomography--but not magnetic resonance imaging--identified periventricular white-matter lesions predict symptomatic cerebrovascular disease in probable Alzheimer's disease.

To examine the clinical consequences of periventricular white-matter lesions on computed tomography (CT) and magnetic resonance imaging (MRI) scans in probable Alzheimer's disease. Case series, 12-month follow-up. Multidisciplinary behavioral neurology research clinic. We longitudinally evaluated the clinical characteristics of 27 patients with probable AD for whom both CT and MRI scans had been performed at baseline. None. The presence of abnormal neurological signs was examined at baseline and at a 12-month examination. Periventricular white-matter lesions were observed with CT in 12 patients (44%) and with MRI in 21 patients (78%). Computed tomography did not detect lesions of 1 to 3 mm, as were seen on MRI scans, and CT also did not detect lesions of 4 to 10 mm when they occurred in the deep subcortical white matter and were not part of a greater confluent lesion. There was no relationship between the severity of periventricular white-matter lesions with either neuroimaging method and the presence of abnormal neurological signs. However, there was a greater frequency of periventricular white-matter lesions shown on CT scans than on MRI scans at baseline in patients in whom abnormal neurological signs (eg, abnormal gait, asymmetric deep tendon reflexes, focal motor deficits, abnormal plantar response) developed at 12-month follow-up. Although MRI may be more sensitive in detecting periventricular white-matter lesions, CT is more specific in predicting subsequent symptomatic cerebrovascular disease.

Clinical predictors of computed tomographic abnormalities following pediatric traumatic brain injury.

Children commonly seek attention in emergency departments following head injury. Head computed tomography (CT) is often used to decide subsequent disposition. Clinical criteria predicting CT abnormalities would allow effective and timely treatment and minimize unnecessary procedures depleting overburdened medical resources. We prospectively compared presenting clinical features with subsequent emergent head CT in 300 children less than 19 years old over a nine-month period. The disposition of patients following imaging was also recorded. Only suspected abuse was more than 50% positively predictive in children below age two and those above age two. Two signs were more than 67% positively predictive in both age groups: focal motor deficit and pupillary asymmetry. Patients with abnormal CTs were the only children to undergo emergent neurosurgery (30%) and were nearly five times as likely to be intensively monitored. Children with normal CTs were nearly five times as likely to be observed in a routine department or at home. We conclude that no single clinical feature can predict with certainty an abnormality on immediate head CT. However, children suspected of being abused, and those with focal motor deficits or pupillary asymmetry, should be imaged. Finally, emergent CT when judiciously ordered likely reduces unforeseen morbidity and minimizes costly intensive care observation.

Severe neurological complications following orthotopic liver transplantation in patients receiving FK 506 and prednisone

The objective of this study was to define the severe neurological complications that occur in recipients of an orthotopic liver transplantation, receiving FK 506 as their primary immunosuppressive agent. To accomplish this, 100 consecutive orthotopic liver transplantation patients were followed prospectively from the time of their transplant until the date of their initial post-orthotopic liver transplantation discharge from hospital. All major neurological complications occurring during this period were recorded and assessed. The frequency of severe neurological complications occurring in these severely ill transplant recipients was 34%. Delirium was noted in 16, coma in 9, seizures in 4, and 5 developed focal motor deficits associated with the finding of a brain abscess, transient ischemic attack or central pontine myelinolysis. At the time at which a major neurologic complication was noted, the blood level of FK 506 was recorded. No direct relationship between FK 506 blood levels and the presence or absence of major neurologic complications of orthotopic liver transplantation could be demonstrated. Based upon this series, it can be concluded that although FK 506 may contribute to the pathogenesis of minor neurological complications seen after orthotopic liver transplantation such as tremors and headaches, the pathogenesis of most of the major neurologic complications occurring after orthotopic liver transplantation is multifactorial and cannot be ascribed solely to FK 506 toxicity.

Central Nervous System Complications After Cardiac Surgery

Central nervous system (CNS) complications (disturbance of consciousness, focal motor deficits, and seizures) after coronary artery bypass grafting (CABG) and cardiac valve surgery were studied retrospectively. The incidence of CNS complications was significantly more frequent in CABG (11%, 71/638) than in valve surgery (7%, 24/345). Major contributory factors of CNS complications were preexisting cerebrovascular disease and cardiopulmonary bypass time. In comparison to previous reports, older age, hypertension, diabetes mellitus, and cerebrovascular disease were more common in the patients undergoing CABG. The preexisting cerebrovascular disease and prolonged cardiopulmonary bypass time probably increase the risk of cerebral embolism and/or cerebral hypoperfusion. We conclude that patients undergoing CABG surgery are at greater risk for neurological damage in comparison to those undergoing valve surgery.

Neurological complications after liver retransplantation.

Postoperative neurological complications in 185 patients who underwent two or more orthotopic liver transplantations were reviewed. The most common neurological complications were alteration of mental status (84%), seizures (33%) and focal motor deficits (15%). The frequency of neurological complications after a second orthotopic liver transplantation was significantly greater than that after a single orthotopic liver transplantation. However, neurological complications were more frequent after a second orthotopic liver transplantation than after a third transplant. Significantly more neurological complications occurred in patients who did not survive a year than in those who did, regardless of the number of transplants they underwent. These findings indicate that the risk of neurological complications among patients with multiple orthotopic liver transplantations is greater in those who require a second transplant; this risk appears to diminish after a third transplant. Importantly, the presence of neurological complications is associated with increased post-orthotopic liver transplantation mortality rate.

Neurologic sequelae of cardiac surgery in children.

Major advances in surgical and cardiopulmonary bypass technology have occurred in the past 30 years. Total correction of previously inoperable congenital cardiac defects is being performed with increasing frequency and in children at progressively younger ages. While the majority of children undergoing cardiac surgery survive without incident, increasing concern is being raised about neurologic sequelae seen in some survivors. Complications such as embolization, hypoxia, inadequate cerebral perfusion, and biochemical disturbances may all lead to brain damage following cardiac surgery. Acute postoperative neurologic problems include seizures, impaired levels of consciousness, focal motor deficits, and movement disorders. Long-term sequelae include language and learning disorders, mental retardation, seizures, and cerebral palsy. Intraoperative cerebral monitoring techniques are as yet imperfect, but their use in combination with meticulous intraoperative and postoperative care currently provides the best means of reducing neurologic morbidity. Future studies should explore other methods of preserving neurologic integrity in children undergoing open heart surgery.

Progressive Expanding Congenital Porencephalies: A Treatable Cause of Progressive Encephalopathy

In congenital porencephalies, diverticulation of the lateral ventricle is a dynamic process producing compression and stretching of the brain tissue bordering the diverticulum, bulging of the overlying skull, macrocephaly, and occasionally progessive neurologic signs (hemiplegia, raised intracranial pressure), even when the rest of the ventricular system is not dilated and the CSF pressure is normal. Ventriculoperitoneal shunting can result in remarkable improvement of focal motor deficits and may apparently also play a beneficial role on further mental development. Successive computed tomography scans demonstrate that the brain parenchyma, which had been stretched by the porencephalic pouch, is capable of regaining near normal thickness. Congenital porencephalies are initiated by a limited destructive brain lesion, but the gradual expansion of the ventricular herniation may imply a mechanism identical to that which has been postulated in normal pressure hydrocephalus. Nine cases of unilateral "expanding" congenital porencephalies are presented and the treatment of this condition is discussed.

Congenital Porencephaly

Congenital porencephaly originates early in fet al development as a component of diffuse cerebral malformation. In infancy the patients have clinical manifestations of local or generalized cerebral disease. Convulsions, delaved growth and development and focal motor deficits are the most common clinical findings. Skull films, transillumination and brain scanning are useful methods of examination. Intracranial pneumography is the definitive means of diagbnosis. Roentgen studies in unilateral cases demonstrate characteristic changes. This pattern consists of ipsilateral calvarial enlargement and thinning, bilateral ventricular dilatation and contralateral shift of midline structures. Other anomalies of the cerebrum and meninges are often demonstrated on roentgen studies. The prognosis in these patients is guarded. Significant mental and motor retardation occurred in nearly all cases.

Epilepsy in the adolescent.

Onset of puberty may exacerbate a previously well-controlled convulsive disorder. Focal seizures and major motor deficits may take a different form in late childhood or early adolescence. True petit mal is primarily a disease of childhood, but it may present de novo in adolescence, with atypical aspects. The area in which physicians most often fail their epileptic patients is not in seizure control but in providing vocational and family counseling to integrate the patient into a self-sufficient role in society.

Meningioma and intermittent aphasia of 44 years' duration. Case report.

The patient, a 74-year-old right-handed woman, was admitted with a 1-day history of fever, cough, and shortness of breath. Two days before admission she had been found on the floor of her home in a confused state but conscious. The patient had been in good health until age 30, when she developed recurrent episodes of confusion during which she became dazed and experienced feelings of depersonalization in which she felt that her heart was beating outside her body. These and similar bizarre aura were followed by paresthesia and weakness of the right hand which spread to the face and right arm and were either accompanied or followed by global aphasia. There was no loss of consciousness. Occasionally the paresthesia would spread to the left arm and legs but convulsive movements did not occur. Aphasia would persist usually for 1 or 2 hours and rarely for as long as 24 hours. Recovery of motor and sensory function was always complete, and her speech remained unaffected during the interictal periods. Until age 47 her attacks were infrequent, averaging about one per month. At that time she experienced a marked increase in the frequency of seizures to three or four times a week. Treatment with phenobarbital, 60 mg daily, achieved some reduction in the number of attacks but complete control was not possible. When she was 59, an electroencephalogram revealed frequent sharp waves in the left temporal and frontal areas in addition to bursts of slow waves. Dilantin, 100 mg daily, was added to her treatment without appreciable benefit. Frequent attacks of aphasia continued in association with focal motor and sensory symptoms, but at age 65 the patient for the first time experienced two generalized major tonic-clonic convulsions with loss of consciousness. Over the next few years she had a slight decrease in the frequency of her aphasic attacks to three or four per month until the time of her hospitalization. Examination. On admission the patient, a thin, elderly woman, was dyspneic and cyanotic. She was conscious but did not answer questions. Blood pressure was 106/60, pulse 100, temperature 104. Bilateral rales were heard on auscultation of the chest. Fundoscopie examination demonstrated a small area of old chorioretinitis in the right eye but no papilledema. Visual fields were full, and the remainder of the cranial nerves were intact. Generalized weakness was present, but there were no focal motor deficits. A

Corneal thermal patterns in anterior uveitis.

<b>Objective: </b> To describe a more limited and less malignant form of Rasmussen encephalitis (RE). <b>Methods: </b> Three subjects (all women; 37, 31, and 32 years of age) developed childhood or late onset chronic focal encephalitis, with a relatively nonprogressive form of the disorder. <b>Results: </b> In our patients, clinical features were dominated by partial seizures without marked focal motor deficit and in two with choreo-dystonic movements. The diagnosis of RE was supported by histologic examination and anatomic and functional MRI. <b>Conclusions: </b> These cases extend the phenotypic presentations of Rasmussen encephalitis and confirm Theodore Rasmussen9s suggestion that there may be mild and nonprogressive forms of the disease.