Unexplained Neurological Signs Research Articles

Cognitive dysfunction, urinary retention, and a lesion in the thalamus—Beware of possible toxocariasis of the central nervous system

Toxocariasis of the central nervous system is usually characterized by an eosinophilic meningitis, encephalitis or myelitis. We here report a patient with an at least 7 years history of unexplained neurologic signs and symptoms. MRI showed a cystic lesion in the left thalamus, compatible with a parasitic infection. Blood and CSF analyses were positive for Toxocara canis IgG Western-blot, but were otherwise unremarkable. The case report raises the question whether there are chronic or late variants of this disease.

Craniovertebral junction database analysis: incidence, classification, presentation, and treatment algorithms

The initial treatment of bony lesions at the craniocervical junction consisted of posterior decompression with enlargement of the foramen and removal of the posterior arch of the atlas and axis vertebra. This was regardless of the site of compression. Needless to say, the results were poor when an irreducible ventral lesion at the craniocervical junction caused compression of the cervicomedullary area. It was predictable that an adverse outcome would occur in approximately 35-40% of patients treated with dorsal decompression with a fixed anterior abnormality at the craniovertebral junction. Thus, it became essential to identify the causes of failure and recognize the pathology as well as attempt to improve the treatment. The need to identify natural history led to a prospective database. This database now lists 5,300 patients who were analyzed. Of these, 2,000 were children and this has been reviewed here. Review of symptoms showed that children with craniovertebral abnormalities present with failure to thrive, weakness, basilar migraine, dysphagia, sleep apnea, scoliosis, and the cervical central cord syndrome. Torticollis was seen in the very young. New imaging techniques were utilized as they became available. Unexplained neurological symptoms and signs associated with craniovertebral abnormalities in children required angiography which was performed with dynamic motion studies. All children with reducible craniocervical abnormalities underwent stabilization. In irreducible abnormalities, decompression was paramount with a fusion to be done immediately. The decompression was accomplished in the direction in which encroachment occurred. A treatment algorithm was formulated as a result of this database and a classification of the craniovertebral abnormalities; that holds good for the entire spectrum.

Cerebral venous thrombosis in adult nephrotic syndrome due to systemic amyloidosis

Although venous thrombosis is one of the common complications in nephrotic patients, cerebral venous thrombosis (CVT) is rarely reported. CVT is so difficult to be detected by conventional diagnostic methods that it is sometimes overlooked despite its potential severity. We report a 79-year-old female with nephrotic syndrome due to systemic amyloidosis who suddenly altered mental status during her hospitalization. The underlying etiology had been not identified by physical examinations, various laboratory data, and repeated computed tomography, and finally she died. The post-mortem examination showed a massive thrombus impacted in intracranial left-sided transverse and sigmoid sinus. This case suggests that CVT can occur in a nephrotic patient who presents unexplained neurological signs and symptoms, which might not be detected only through conventional diagnostic tests.

The role of cranial MRI in identifying patients suffering from child abuse and presenting with unexplained neurological findings

Objective: The aim of this study was to demonstrate the usefulness of cerebral MRI to detect possible child abuse in children with unexplained neurologic findings. Method: Between 1990 and 1997, 208 children were referred for suspected physical child abuse to the Child Protection Clinic of Ste-Justine Hospital, a tertiary care pediatric hospital. Among them, 39 children presented initially with neurological findings. For 27 of them, the CT Scan results prompted the diagnosis of child abuse. However, in 12 children, even if a CT-Scan was performed, the diagnosis and/or the mechanisms of the neurologic distress remained obscure. Investigation was completed with MRI study in those 12 cases. Results: MRI findings were diagnostic for physical abuse in eight cases. A diagnosis of child abuse was made in two more cases by a combination of MRI and skeletal survey findings. In one case, MRI was suggestive but the diagnosis of child abuse could not be confirmed. One case was misinterpreted as normal. Conclusions: MRI is the test of choice to rule out child abuse when faced with a child presenting unexplained neurologic signs lasting for few days. The fact that MRI can better differentiate collections of different ages makes this imaging test particularly useful in identifying cases of child abuse. These results, however, always have to be integrated in a well conducted multidisciplinary clinical approach. French abstract not available at time of publication.

Intradural extramedullary spinal canal secondary neoplasms: MR findings in 30 patients.

The purpose of this study was to describe magnetic resonance findings of intradural spinal canal secondaries and to select the best way of investigating this condition. Thirty patients with a known malignancy [breast carcinoma (n = 14), lung carcinoma (n = 10), other sites (n = 6)] and unexplained neurologic signs were studied with pre- and post-contrast T1-weighted images and T2-weighted images. Cerebrospinal fluid cytology was available in 16 patients and positive in 11 patients. In all the patients, post-contrast T1-weighted images demonstrated abnormal enhanced lesions. Most of them were nodular, located on the conus medullaris and the cauda equina. Few lesions appeared at the thoracic or cervical levels, as nodular or thin areas of enhancement. Pre-contrast T1-weighted sequences failed or were equivocal to detect the lesions. Eighteen of 30 patients had cerebral metastases. Fourteen had osseous metastases. In conclusion, post-contrast T1-weighted sequence is the optimal modality for the diagnostic of intradural spinal canal metastases. Axial and coronal images may be a useful adjunct to precise anatomic changes. T1-weighted and T2-weighted sequences remain necessary when further information is expected on vertebra or soft tissue.

Three cases of hindbrain herniation in adults with comments on some diagnostic difficulties

In discussing the clinical features and surgical treatment of adult patients with Arnold Chiari deformity type I the authors focus on the differential diagnosis from demyelinating disease and present three illustrative cases. Out of 118 patients initially diagnosed as having demyelinating disease and admitted to our hospital between December 1991 and April 1993, three were subsequently found to have the Arnold Chiari deformity on MRI. The delay until correct diagnosis amounted to 10, 16 and 32 years, respectively. Our study stresses the diagnostic importance of MRI of the craniocervical area in cases of unexplained neurological symptoms and external signs of dysplasia. Accurate examination, early diagnosis and selection of proper surgical management can alleviate severe symptoms in many instances and prevent neurological complications.

Spinal cord pial metastases: MR imaging with gadopentetate dimeglumine.

The purpose of this investigation was to describe gadopentetate-dimeglumine-enhanced MR findings in metastatic disease to the pial lining of the spinal cord. Correlation was made with clinical data, other radiologic studies, and pathologic findings. Eighty-six patients with a known malignancy and unexplained neurologic signs or symptoms were studied with pre- and postcontrast T1-weighted images. In seven of these patients, abnormal enhancement of the pial lining of the cord was seen on the sagittal postcontrast T1-weighted images. This appeared as a thin rim of enhancement along the surface of the cord in six patients and as a focal, thick rim of enhancement in addition to the thin rim of enhancement in the seventh patient. Axial images confirmed the location along the pial lining in each case. Precontrast T1-weighted images in all seven cases and precontrast T2-weighted images in five cases failed to detect any focal abnormalities of the pial lining of the cord. Pathologic confirmation was available in five of the seven patients. Primary malignancies in these patients included breast carcinomas (two), lymphoma (one), leukemia (one), adenocarcinoma of the lung (one), prostate carcinoma (one), and malignant melanoma (one). Three of seven patients had metastatic disease evident only within the CNS, while four patients had widespread disease outside the CNS. We conclude that contrast-enhanced MR imaging is useful in the diagnosis of pial spread of metastatic disease in patients with a known primary malignancy and unexplained neurologic signs or symptoms.

Multiple sclerosis in twins.

We studied 30 sets of twins in whom one or both was suspected of having multiple sclerosis (MS). In 24 pairs, a firm clinical diagnosis was made on each twin. Among these 24 pairs, 6 of 12 monozygotic twins were concordant for clinical MS, compared with 2 of 12 dizygotic twins. Of those over the age of 50, two of three monozygotic pairs were concordant, but neither of the two dizygotic twin pairs were concordant. Because ascertainment was primarily through public announcement, this series may be biased in favor of twins concordant for MS. The individuals within monozygotic concordant twin pairs exhibited wide differences in severity and age at onset of disease; the more recently affected twin tended to have a lower cerebrospinal fluid (CSF) IgG and a higher IgM level. Although the frequency of HLA-B7 and Dw2 in this twin population was high, the HLA makeup did not differ appreciably between concordant and discordant MS twins. Furthermore, the two DZ-concordant twins were HLA-nonidentical. Unexplained neurologic signs were found in three asymptomatic twins, and a high proportion of clinically normal twins had abnormalities of CSF immunoglobulins. These latter findings suggest a high incidence of subclinical MS in this population.

Death from fat embolism as a complication of intraosseous phlebography.

Abstract A woman aged 52 with reticulum-cell sarcoma died of fat embolism 8 hours after an intraosseous pertrochanteric phlebogram was performed to demonstrate the cause of iliac-vein and inferior-vena-caval obstruction. The cause of death was confirmed at necropsy. No other record of this complication could be found in published reports. The possibility of fat embolism should be considered in all patients who die unexpectedly or in whom unexplained pulmonary, circulatory, and neurological signs develop after intraosseous phlebography.