Acute Neurological Dysfunction Research Articles

Stroke-like episodes in autosomal recessive cytochrome oxidase deficiency

Stroke-like episodes, defined as periods of acute localized neurological dysfunction during which brain imagery suggests cerebral ischemia but vascular anatomy is normal, occurred in 3 patients with autosomal recessive Saguenay-Lac St-Jean (SLSJ) cytochrome oxidase (COX) deficiency. The patients developed focal neurological deterioration and frontal hypodensities on cerebral computerized tomography (CT). Arteriography, performed in 1 patient during an acute episode, showed normal vascular anatomy. Nevertheless, capillary shunting was evident both in regions that appeared abnormal on the initial cerebral CT study and in regions that appeared normal but subsequently developed Leigh disease. Stroke-like episodes did not exacerbate systemic acidosis, and acidotic decompensations occurred independently of stroke-like episodes. In conclusion, stroke-like episodes occur in autosomal recessively inherited congenital lactic acidoses as well as in those caused by mitochondrial DNA mutations. In some cases, acute localized neurovascular changes occur in regions that subsequently develop Leigh disease.

Index of Suspicion

Index of Suspicion

Acute neurologic dysfunction associated with high-dose chemotherapy and autologous bone marrow rescue for primary malignant brain tumors.

Acute neurologic complications occurred 103 times in 50 (54%) of 92 patients (primarily children) treated with high-dose chemotherapy and autologous bone marrow rescue for primary central nervous tumors. Different types of neurologic compromise occurred during the chemotherapy infusion as compared to the first 100 days after the chemotherapy and the greater-than-100-day time period. The causes of the neurologic compromise were also time sensitive. Results of treatment for children with primary brain tumors using high-dose chemotherapy with autologous marrow rescue (ABMR) have been encouraging. However, the neurotoxicity associated with this technique remains a major concern. We reviewed the records of 92 patients who underwent ABMR for malignant brain tumors between 1986 and 1992 for the occurrence and timing of acute neurologic dysfunction (AND). Individual investigators at the participating institutions retrospectively completed standardized forms on each patient. The manner in which the distribution of AND versus time of treatment emerged led to the establishment of distinct time periods for data analysis and discussion. The pre-ABMR period included those events that occurred during the chemotherapy infusion, the early posttreatment period included the first 100 days following bone marrow rescue, and the late posttreatment period was greater than 100 days following bone marrow rescue. Fifty patients (54%) had 103 episodes of AND. AND included encephalopathies with or without hallucinations or coma (32), seizures (23), headaches (9), ataxia-tremor-dysarthria syndrome (7), anorexia and nausea syndrome (7) and others (25). During the chemotherapy infusion, encephalopathies and seizures were most common. Hallucinations occurred primarily related to drug infusion, while encephalopathies without hallucinations were usually due to demonstrable dysmetabolic states. In the 100 days following ABMR, dysmetabolic states and iatrogenic factors caused 45% and progressive disease caused 33% of AND. Greater than 100 days from ABMR, progressive disease caused 55% of AND; 7 patients were noted to develop chronic anorexia and nausea of unclear etiology. The occurrence of neurologic compromise was not related to the chemotherapy regimens, tumor histology, tumor location, patient age, prior treatment, or the amount of tumor at time of treatment. Dexamethasone use was the only clinical factor associated with AND (p < 0.004). The cause of AND was definable for 95% of instances that occurred within 100 days of ABMR. Early AND was often iatrogenic and reversible. The greater the time from ABMR the more likely AND was due to progressive disease. Clinical factors could not predict the occurrence of AND as only the concurrent use of dexamethasone at the time of treatment proved significant. Although frequent, AND should not be considered a limiting toxicity of this approach or preclude the use of this technique.

Valve replacement in patients with endocarditis and acute neurologic deficit

Acute neurologic deficits occur in up to 40% of patients with left heart endocarditis. Appropriate evaluation and management of patients with acute neurologic dysfunction who require valve operations for endocarditis remain controversial. This retrospective review was undertaken to develop recommendations for the evaluation and treatment of these challenging patients. From 1983 to 1995, 247 patients underwent operations for left heart native valve endocarditis at the Johns Hopkins Hospital. From a review of medical and pathology records, 34 patients (14%) with preoperative neurologic deficits were identified. Data on these 34 patients were recorded and analyzed. Causes of neurologic dysfunction included embolic cerebrovascular accident (n = 23, 68%), embolic cerebrovascular accident with hemorrhage (n = 4, 12%), ruptured mycotic aneurysm (n = 3, 9%), transient ischemic attack (n = 2, 6%), and meningitis (n = 2, 6%). Preoperative diagnostic studies included computed tomography (32 patients), magnetic resonance imaging (11 patients), cerebral angiogram (14 patients), and lumbar puncture (2 patients). Computed tomography demonstrated structural lesions in 29 of 32 patients; in only 1 patient did magnetic resonance imaging reveal a lesion not already seen on computed tomography. Of 14 patients having cerebral angiograms, 7 had a mycotic aneurysm. Three mycotic aneurysms had ruptured, and these were clipped before cardiac operations. The mean interval from onset of neurologic deficit to cardiac operation was 22.2 +/- 2.8 days for all patients and 22.1 +/- 3.0 days for those with embolic cerebrovascular accident. The hospital mortality rate was 6%. New or worse neurologic deficits occurred in 2 patients (6%). Neurologic deficits are common in patients with endocarditis referred for cardiac operations. Despite substantial preoperative morbidity, most of these patients do well if the operation can be delayed for 2 to 3 weeks. Computed tomography scan is the preoperative imaging technique of choice, as routine magnetic resonance imaging and cerebral angiogram are unrewarding. Cerebral angiogram is indicated only if computed tomography reveals hemorrhage.

Central nervous system disease in a child with primary Sjögren syndrome

A 9-year-old girl had hemiparesis, and a diagnosis of primary Sjögren syndrome was made. The neurologic dysfunction was multifocal, involving both the brain and spinal cord, and was recurrent; the findings mimicked multiple sclerosis. Corticosteroid treatment during episodes of acute neurologic dysfunction appeared to be beneficial. (J P EDIATR 1995;127:961-3)

Bilateral, Reversible, Selective Thalamic Involvement Demonstrated by Brain MR and Acute Severe Neurological Dysfunction with Favorable Outcome*

We report on two children who presented acute, severe, neurological dysfunction with bilateral, reversible, selective thalamic lesions demonstrated by brain MRI. In both children neurological symptoms appeared two weeks after a febrile respiratory illness. Clinical conditions worsened in a few days to a stuporous state and tetraplegia in one and to coma with decerebrate posturing in the other. Three weeks after the onset, both children improved and recovered within one month. During the acute phase, brain MRI showed in both children bilateral hyperintense areas on T2-weighted sequences limited to both thalamic regions. During the follow-up, repeated brain MRI showed complete disappearance of abnormalities in one patient and a small residual left thalamic lesion in the other. In both patients hematological routine exams were normal. Bacterial and viral studies of serum and CSF were negative. CSF findings showed elevated white blood cell count and protein levels, with no oligoclonal IgG bands. Urine and CSF organic acids by GC/MS and plasma as well as CSF amino acids were normal. We believe that the benign evolution of this disorder and CSF findings strongly suggest a postinfectious process of the central nervous system.

Beauty Parlor Stroke: When a Beautician Becomes a Physician

In Reply. —I wish to thank Drs Beringer and Golden for this opportunity to expand on my article. Beringer wants more information. Patients 1 and 5 developed acute neurological dysfunction during the shampoo procedure, bringing it to a halt. In each case, the patient was immediately hospitalized. Patients 2,3, and 4 noted symptoms of brainstem dysfunction at the conclusion of the shampooing procedure when they stood up. They subsequently sought medical attention. The biomechanics of the shampooing procedure require the head to be placed in a recessed sink with the assumption of the hanging neck and head position. Reliable data about the extent and duration of forces applied by different beauticians, as well as the specific angulation achieved and the ischemic threshold of the individual, are not known nor have they ever been standardized. Despite these theoretical drawbacks and lack of quantification, the occurrence of stroke during this procedure suggests

Bilateral striatal lesions in childhood

From 1983 to 1991, 13 patients were identified with a clinical radiologic association characterized by acute or persistent neurologic dysfunction and bilateral lesions in the basal ganglia region demonstrated by ultrasound, computed tomography, or magnetic resonance imaging. Initial clinical manifestations of this group of patients were characterized by extrapyramidal signs (i.e., dystonia 9, hypotonia 2, athetosis 1, rigidity 1), altered state of consciousness in 5, and seizures in 3. The outcomes of most of these patients were poor: 10 had motor sequelae, 9 cognitive impairment, and 4 died. The outcomes of 2 patients, however, were much better than what was expected from the initial presentation. Based on current and previous reports, the diagnostic approach and classification of patients with neurologic dysfunction and bilateral striatal lesions are presented.

Acute neurologic dysfunction associated with destructive lesions of the basal ganglia: A benign form of infantile bilateral striatal necrosis

In 1982 Gouti6res and Aicardi ~ described three patients with a syndrome characterized by (1) an abrupt clinical onset after an infectious illness, (2) impaired consciousness, axial hypotonia, motor rigidity, and abnormal movements and postures, (3) sustained improvement of neurologic status, and (4) bilateral low-density lesions of the basal ganglia on the computed tomographie scan. Three similar patients also have been repor ted) 4 It is likely that some other patients described before the advent of modern neuroimaging techniques had this same syndrome) Huntington chorea, Wilson disease, Hallervorden-Spatz syndrome, mitochondrial eneephalomyopathies including Leigh syndrome, familial striatal necrosis, head trauma, hypoxia-ischemia, and various intoxications can occur in childhood with acute or subaeute neurologie dysfunction associated with bilateral low-density lesions in the basal ganglia. The outcome of all of them, however, is poorer than that of acute neurologic dysfunction associated with destructive lesions of the basal ganglia, as described by Gouti6res and Aicardi. 1 We describe two patients with A D B G who had a complete clinical recovery. The CT scan findings in the acute phase of the disease were identical in both patients and supported the diagnosis. Bilateral, symmetric, necrotic lesions of the basal ganglia were found in the magnetic resonance imaging studies from the early stages of the disease and persisted in our second patient up to 5 years later.

Hypoxic-ischaemic encephalopathy after near miss sudden infant death syndrome.

Between 1982 and 1985, 14 infants aged 3-26 weeks presented with severe hypoxic episodes as a result of the 'near miss' sudden infant death syndrome (SIDS). They all had metabolic acidosis, cardiovascular instability, acute renal failure, ischaemic colitis, or acute neurological dysfunction. Investigation of the cause excluded infection and trauma, or a primary metabolic, pulmonary, cardiac, or seizure disorder. Seven infants were deeply comatose on admission, never regained consciousness, and died within 60 hours. A characteristic evolution of hypoxic-ischaemic encephalopathy not previously clearly described after near miss SIDS was seen in the seven who lived. Five of the seven were conscious within one hour of resuscitation and showed a striking interval of near normality before neurological deterioration that was characterised by status epilepticus, deep coma, and brain stem dysfunction from 36-96 hours after the event. A biphasic course was not apparent in the remaining two, each of whom was comatose on admission, though refractory seizures did develop. Computed tomograms of the brain more than a week after the event showed cortical infarction or cerebral atrophy. Six of the survivors, followed up from 16-55 months, have serious residual deficits including spastic quadriplegia, delayed development, cortical blindness, or infantile spasms.

Sepsis: A cause of aluminum release from tissue stores associated with acute neurological dysfunction and mortality, by A Davenport, PS Williams, NB Roberts, and JM Bone. Clin Nephrol 30:48–51, 1988

Sepsis: A cause of aluminum release from tissue stores associated with acute neurological dysfunction and mortality, by A Davenport, PS Williams, NB Roberts, and JM Bone. <i>Clin Nephrol</i> 30:48–51, 1988

Acute neurologic dysfunction after high-dose etoposide therapy for malignant glioma.

Etoposide (VP-16-213) has been used in the treatment of many solid tumors and hematologic malignancies. When used in high doses and in conjunction with autologous bone marrow transplantation, this agent has activity against several treatment-resistant cancers including malignant glioma. In six of eight patients (75%) who we treated for recurrent or resistant glioma, sudden severe neurologic deterioration occurred. This developed a median of 9 days after initiation of high-dose etoposide therapy. Significant clinical manifestations have included confusion, papilledema, somnolence, exacerbation of motor deficits, and sharp increase in seizure activity. These abnormalities resolved rapidly after initiation of high-dose intravenous dexamethasone therapy. In all patients, computerized tomographic (CT) brain scans demonstrated stability in tumor size and peritumor edema when compared with pretransplant scans. This complication appears to represent a significant new toxicity of high-dose etoposide therapy for malignant glioma.

Primary Sjögren's syndrome with central nervous system disease mimicking multiple sclerosis.

Central nervous system involvement has occurred in approximately 20% of patients with primary Sjögren's syndrome evaluated at our institution. Characteristically, the neurologic dysfunction is multifocal, involving both the brain and spinal cord, and is recurrent over time. We present the features of 20 patients with primary Sjögren's syndrome and central nervous system involvement whose neurologic findings, evoked potential abnormalities, and cerebrospinal fluid profiles (elevated IgG indices, oligoclonal bands on agarose gel electrophoresis, and mild pleocytosis with reactive lymphoid cells) closely resembled those of multiple sclerosis. In fact, multiple sclerosis was considered the most likely diagnosis in each of these patients before diagnosis of Sjögren's syndrome, and each patient met criteria for definite multiple sclerosis. The clinical effects of corticosteroid treatment during episodes of acute neurologic dysfunction appeared to be beneficial in these patients.