Facial nerve enhancement on gadolinium-DTPA in a case with neurosarcoidosis

In a case of neurosarcoidosis with bilateral facial nerve palsy and hydrocephalus, contrast-enhanced magnetic resonance imaging (MRI) study and angiotensin converting enzyme (ACE) activities in cerebrospinal fluid (CSF) were valuable for the diagnosis and the follow up. Facial nerve lesions were demonstrated on gadolinium-DTPA enhanced MRI. The disappearance of enhancement was concomitant with the amelioration of facial nerve palsy after corticosteroid therapy.     

Neurosarcoidosis

Signs and symptoms of neurosarcoidosis are variable and depend on location and size of granulomas. Clinical studies suggest a rate of 5% and autopsy results a rate of more than 25% of central nervous system (CNS) involvement in sarcoidosis. Statistical analysis of 57,789 patients admitted to the Department of Medicine in Lucerne over an 11-year period revealed 51 patients (0.9/1000) with the diagnosis of sarcoidosis. Six of these (12%) had sarcoidosis affecting the CNS. Neurosarcoidosis presented as: leptomeningeal granulomas, cranial nerve palsy, hypothalamic-pituitary syndrome, diabetes insipidus, pareses, paresthesia, pyramidal signs, dementia, urine retention, and asymptomatic granulomas. Neurosarcoidosis has predilections for the base of the brain, cranial nerves (facial nerve palsy is the most common) and meninges, but any part of the CNS may be affected. Therefore, the diagnosis of neurosarcoidosis may be extremely difficult, especially when it occurs as an isolated finding. Positive findings in transbronchial biopsy and lavage may demonstrate asymptomatic pulmonary involvement in as many as 50% of patients with neurosarcoidosis. Angiotensin-converting enzyme levels may be raised in the blood or cerebrospinal fluid in some 50% of cases. Kveim test has a low sensitivity in neurosarcoidosis and thus is of little use. Gallium uptake may demonstrate extracranial granuloma available for biopsy. All these tests, and also computed tomography and magnetic resonance imaging, may be helpful. However, when in selected cases with isolated CNS disease standard investigations are not conclusive, meningeal or cerebral biopsy may be required in order to exclude other causes such as other granulomatous disorders, tumor metastasis, lymphoma, vasculitis, Sj?gren syndrome, infection, neurologic disease such as multiple sclerosis, or systemic diseases such as Whipple's disease. CNS involvement in the acute phase of the disease has a favorable prognosis, while chronic courses respond less well to therapy. Treatment is initiated most frequently with corticosteroids (0.5-1 mg/kg body weight/day or pulses of 1 g/day of methylprednisolone in severe cases). Improvement is seen within 1-2 months. Side effects of corticosteroids, aggressive disease or frequent recurrences may require other immunosuppressive drugs (methotrexate, azathioprine, chlorambucil, cyclosporine A). Cerebral irradiation may be successful in some cases when other treatments fail.     

Acute hemorrhagic leukoencephalitis: recovery and reversal of magnetic resonance imaging findings in a child

A case of acute hemorrhagic leukoencephalitis (AHLE) in a 6-year-old girl is reported. The presentation was typical for acute hemorrhagic leukoencephalitis, with acute onset of a rapidly progressive neurologic disorder with asymmetric involvement of brain, with polymorphonuclear predominant peripheral leukocytosis and cerebrospinal fluid pleocytosis. Cerebrospinal fluid findings not previously reported included elevation of IgG and the presence of myelin basic protein. Additional previously unreported findings were striking abnormalities on magnetic resonance imaging (MRI) of the brain, in contrast to normal findings on computed tomography (CT). The child was treated with high dose intravenous steroids and made a full recovery, with a parallel disappearance of all of her cerebrospinal fluid abnormalities and almost all of her abnormalities on MRI. Detailed examination of cerebrospinal fluid and MRI of brain should facilitate early diagnosis in other cases of suspected acute hemorrhagic leukoencephalitis and high-dose steroid therapy may lead to improved clinical outcomes.     

Pseudotumor forms of neurosarcoidosis in children. Diagnostic difficulties and therapeutic management

In systemic sarcoidosis, neurologic manifestations are possible and occurred in 5 p. 100 of cases. The diagnosis is easier when sarcoiddosis is known, but in half case neurologic symptoms are inaugural. In these cases, involvement of other sites (liver, lung, eyes or skin) by the disease is needed to confirm sarcoidosis. Main manifestations in neurosarcoidosis are cranial nerve palsy, diabetes insipidus or chronic aseptic meningitis, but sometimes sarcoidosis can present as an intracranial mass. The diagnosis of neurosarcoidosis may be difficult and could be confused with infection (such tuberculosis or mycosis), with inflammatory diseases (such multiple sclerosis) or with neoplasm, particularly in case of oedema. Brain biopsy may be necessary in isolated neurosarcoidosis if no peripheral histology evidence of noncaseating granulomas has been obtained. Treatment with steroids alone is the best choice for initial therapy but must be prolonged. It is difficult to predict accurately the response to steroids and relapse may be possible after a long time. Other immunosuppressive therapeutics or radiotherapy have been used in patient with refractory neurosarcoidosis. Sequential magnetic resonance imaging is the useful mean for the follow-up of neurosarcoidosis.     

Presaccadic omnidirectional burst activity in the basal interstitial nucleus in the monkey cerebellum

The endocrine function and pituitary imaging in Sheehan's syndrome more than 30 years after causative events were evaluated. Magnetic resonance imaging (MRI), a combined anterior pituitary test, plasma vasopressin-to-osmolality adaptation study, and antithyroid and antipituitary cell antibody measurement were performed in 6 women with Sheehan's syndrome. The interval from delivery to the onset of symptoms of hormonal deficiency ranged from 3 to 32 years. Since clinical onset, all had received glucocorticoid and thyroid replacement therapy. Cranial MRI examination showed an "empty sella" in 5 cases. Among these, 2 of 5 (40%) demonstrated panhypopituitarism and the other 3 (60%) maintained gonadotropin response. The pituitary gland was normally discernible but with a low-intensity lesion on T1-weighted images in a patient who maintained PRL and gonadotropin responses. Posterior pituitary function was abnormal in 3 of 6 (50%) despite the absence of polyuria. No antipituitary antibodies were detected in any of the cases. Thyroid peroxidase antibody was negative in all cases, but antithyroglobulin antibody was detected in 2 of 6 (33%). Thyroid-stimulating antibody was not detected, but one case had an anti-TSH antibody. Thirty years after the initial events, most patients with Sheehan's syndrome showed signs of an empty sella on MRI, all having noticeable suppression of anterior and/or posterior pituitary hormones with no related autoimmunity.     

MRI-demonstrable regression of a pituitary mass in a case of primary hypothyroidism after a week of acute thyroid hormone therapy

Although magnetic resonance imaging (MRI) characteristics of pituitary gland hyperplasia in primary hypothyroidism have been previously described, the time span necessary for the regression of the hyperplasia in response to acute thyroid hormone (TH) therapy has not been defined. A 26-yr-old woman underwent 131I ablation 11 yr before admission. Intermittent poor compliance to levothyroxine (LT4) therapy led to inappropriately high serum thyroid-stimulating hormone (TSH) for her triiodothyronine (T3) and thyroxine (T4) levels. The patient was investigated to rule out TSH-secreting pituitary adenoma or resistance to TH. On admission, the patient's clinical features and thyroid function tests, as well as thyrotropin-releasing hormone (TRH) and acute T3 suppression tests, were in favor of profound primary hypothyroidism. MRI revealed symmetrical enlargement of the pituitary gland with distinct morphological characteristics of a macroadenoma. The patient began high-dose TH therapy and was rescanned six days later. The follow-up scan revealed a dramatic shrinkage of the pituitary gland. Four weeks later, serum T4 and TSH were within the normal range, and repeat MRI scan of the pituitary at that time showed a normal gland. This case is the first to document dramatic shrinkage of pituitary hyperplasia in long-standing primary hypothyroidism within one week of acute TH therapy. MRI alone is unable to reliably differentiate between a TSH-secreting pituitary adenoma and hypothyroidism-induced pituitary hyperplasia. Dynamic endocrine testing as well as repeat pituitary MRI after a brief TH trial may provide a firm diagnosis in similar cases.     

Effects of oral treatment with sustained release morphine tablets on hypothalamic-pituitary-adrenal axis

A 31-year-old black male with sarcoidosis en-plaque of the dura mater, which is a rare morphological variant of neurosarcoidosis (NS), presented at our clinic. Magnetic resonance imaging (MRI) of the head with gadolinium showed non-specific enhancement of both tentorial leaves extending to the floor of right middle cranial fossa and cavernous sinus. The laboratory results were normal except for slightly increased serum angiotensin converting enzyme (SACE) (68 U/ml n = 4-56 U/ml) and cerebrospinal fluid (CSF) IgG index (0.57, n = 0.46). Biopsy of the intracranial dural lesion was consistent with sarcoidosis. Oral steroid therapy (Methylprednisolone 4 mg QID) was started and the patient became asymptomatic. However, MRI of the brain with gadolinium 2 months after biopsy showed progression and extension of the enhanced dural lesion. His SACE level was unchanged. We concluded that progression of the enhanced lesion seen in MRI could be recently formed scar tissue, new lesion or both. MRI findings should always be correlated with clinical findings for evaluation of NS during follow-up.     

Obesity, salt intake, and renal perfusion in healthy humans

OBJECTIVE: To evaluate the pathogenicity of a recently discovered arthropod-transmitted bunyavirus (Toscana virus) on the CNS in children and to provide information on the epidemiologic and clinical aspects of Toscana virus infection. STUDY DESIGN: Case-series analysis of children hospitalized with clinical and cerebrospinal fluid examination compatible with a CNS disease of viral origin. METHODS: Cerebrospinal fluid, acute, and convalescent sera were investigated for conventional neurotropic viruses and for Toscana and tickborne encephalitis viruses. A clinical-epidemiologic analysis was carried out on confirmed Toscana virus cases to clarify the profile of Toscana virus infection in children. RESULTS: The study indicates that (1) Toscana virus has been endemic in the Siena province for at least 15 years; (2) the virus is responsible for at least 80% of acute viral infections of the CNS in children throughout the summertime; (3) the clinical signs and symptoms range from aseptic meningitis to meningoencephalitis; (4) infected children resided habitually or temporarily in rural or suburban areas of the Siena province, where ecological characteristics allow arthropods to be peridomestic in human settlements. CONCLUSIONS: Toscana virus is the most common viral agent involved in acute infections of CNS in children in central Italy.     

Correlation between sexual steroid effect and activity changes and isoenzyme distribution of lactate dehydrogenase (EC 1.1.1.27) in rat anterior pituitary

Sexual steroid feed-back is a fundamental regulating factor oification of pituitary metabolism in rat. Oestrogen synthesis and/or activity of M subunif testosterone, in males testosterone treatment as well as lack of oestrogen result in characteristic pituitary LDH activity and isoenzyme distribution. Physiological doses of sexual steroids affect, directly or indirectly, pituitary LDH activity, oxydative metabolism, and imply different biochemical bases for the polypeptide hormone production in the anterior pituitary gland.     

Mechanism of action of a new component of the Ca(2+)-dependent proteolytic system in rat brain: the calpain activator

OBJECTIVE: Using single photon emission computed tomography (SPECT) we evaluated the presence and evolution of changes in brain perfusion in juvenile systemic lupus erythematosus (JSLE). METHODS: SPECT was performed in 14 patients with active JSLE divided in 2 groups: the first included 7 patients without central nervous system (CNS) involvement and the second 7 patients with minor neuropsychiatric symptoms (headache, reactive depression, cognitive impairment, mood swing). SPECT findings were compared to seroimmunological and magnetic resonance imaging (MRI) data. After 6 month followup, a second SPECT scan was performed in 12 of 14 patients. RESULTS: At baseline, SPECT showed perfusion defects in 2 patients without neuropsychiatric symptoms and in 5 patients with CNS involvement. In one of the 7 patients with altered SPECT, MRI showed focal hyperintensities. MRI alterations were observed in another patient who had a normal SPECT scan. Cortical atrophy was present in 5 of 14 patients. Correlation between neuropsychiatric manifestations and SPECT findings was not clearly evident because the major part of JSLE patients with CNS involvement and with SPECT alterations had multiple symptoms, but showed focal hypoperfusion on SPECT imaging. No significant association was found between seroimmunological data and SPECT findings. At followup, improvement of perfusion alterations was observed in 6 of 7 patients with altered SPECT and, in 3 of them, findings might be attributed to changes in steroid treatment. CONCLUSION: Perfusion abnormalities in SLE may represent reversible lesions or subclinical CNS involvement. Moreover, SPECT imaging appears to be useful in detecting and monitoring CNS involvement in SLE.     

Inducible expression of the 2-5A synthetase/RNase L system results in inhibition of vaccinia virus replication

Clinically apparent involvement of the nervous system occurs in a relatively small number of patients with sarcoidosis. The diagnosis of neurosarcoidosis is often difficult and particularly so in patients who lack either pulmonary or systemic manifestations of sarcoidosis. Furthermore, clinical features of neurosarcoidosis are extremely variable. In this series of 37 patients, seen during the last 30 years, cranial nerve palsies occurred in 52%, polyneuritis or polyneuropathy in 24%, meningeal involvement in 24%, muscle disease in 8%, and Guillain-Barré syndrome in 5% of the patients. Other presentations included seizures, brain mass, pituitary/hypothalamic syndrome, and memory loss associated with confusion. The chest radiograph was abnormal in 8 of every 10 patients with neurosarcoidosis. In 18 (85%) of 21 patients, gallium uptake was consistent with the diagnosis of active sarcoidosis. Serum angiotensin-converting enzyme levels were raised in about half of the patients. Cerebrospinal fluid features, including lymphocyte pleocytosis, raised protein levels, and decreased glucose concentration, were of little help. MRI with gadolinium enhancement was the most sensitive diagnostic tool, particularly in patients with meningeal involvement. The ultimate arbiter of the diagnosis of neurosarcoidosis, the presence of noncaseating granulomas in the involved tissue, was not always available. Although corticosteroids are the mainstay of therapy, in this series, 12 patients received chloroquine or hydroxychloroquine. Prognosis of chronic neurosarcoidosis is poor. Six (18%) of 37 patients died of complications related to sarcoidosis.     

Cancer of the nasal cavity and paranasal sinuses and exposure to environmental tobacco smoke in pet dogs

PURPOSE: To evaluate dynamic MR imaging of the pituitary gland. MATERIAL AND METHODS: 19 patients with suspected mass lesions of the pituitary gland were examined at 1.5 Tesla with dynamic and standard MRI using a Turbo-FLASH sequence (1 image/s for 40 s). RESULTS: In 13/19 patients microadenomas were detected. One of the 13 microadenomas was detected using dynamic imaging and was not seen on standard MRI. The remaining 12 microadenomas were diagnosed with standard MRI. CONCLUSION: Dynamic imaging of the pituitary gland is a time-consuming and costly diagnostic technique. If laboratory results suggest the presence of a microadenoma and conventional MRI is unable to localise it, dynamic imaging should be performed.     

Detection and identification of carcinogen-peptide adducts by nanoelectrospray tandem mass spectrometry

Leukoencephalopathy syndromes encompass a variety of neurologic abnormalities that affect cerebral white matter. Known etiologies include malignant hypertension, eclampsia, renal failure, CNS infection, and drug therapy with cyclosporine, tacrolimus (FK506) and interferon-alpha. Symptoms vary according to sites of involvement; they include altered mentation, visual disturbances, focal neurologic signs, and seizures. Characteristic radiologic findings are hypodense areas without contrast enhancement on CT and an increased T2 signal on MRI. The hypodense areas are often symmetric. The clinical symptoms and neuroimaging abnormalities are often reversible with treatment of the underlying condition or removal of the offending drug. We describe a patient with non-Hodgkin's lymphoma treated with conventional cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy and high-dose steroids who developed a rapidly progressive fatal leukoencephalopathy. Her neurologic symptoms and findings on CT are consistent with reported cases of leukoencephalopathy; however, in this instance, the syndrome was not reversible and was ultimately fatal. None of the previously described etiologies could be demonstrated in association with the patient's illness. There are no prior reports of fatal leukoencephalopathy in adult patients treated with standard-dose CHOP. We believe the concurrent immunosuppression from chemotherapy and high-dose steroids resulted in this patient's fatal complication.     

Central nervous system infection with Listeria monocytogenes. 33 years' experience at a general hospital and review of 776 episodes from the literature

We reviewed 776 previously reported and 44 new cases of CNS listeriosis outside of pregnancy and the neonatal period, and evaluated the epidemiologic, diagnostic, and therapeutic characteristics of this infection. Among patients with Listeria meningitis/meningoencephalitis, hematologic malignancy and kidney transplantation were the leading predisposing factors, but 36% of patients had no underlying diseases recognized. The infection occurred throughout life, with a higher incidence before the age of 3 and after the age of 45-50 years. Fever, altered sensorium, and headache were the most common symptoms, but 42% of patients had no meningeal signs on admission. Compared with patients with acute meningitis due to other bacterial pathogens, patients with Listeria infection had a significantly lower incidence of meningeal signs, and the CSF profile was significantly less likely to have a high WBC count or a high protein concentration. Gram stain of CSF was negative in two-thirds of cases of CNS listeriosis. One-third of patients had focal neurologic findings, and approximately one-fourth developed seizures over their course. Mortality was 26% overall, and was higher among patients with seizures and those older than 65 years of age. Relapse occurred in 7% of episodes. Ampicillin for a minimum of 15-21 days (with an aminoglycoside for at least the first 7-10 days) remains the treatment of choice. Cerebritis/abscess due to L. monocytogenes, without meningeal involvement, is less common but may be diagnosed by blood cultures and CNS imaging, or by stereotactic biopsy. Longer antibiotic therapy (at least 5-6 weeks) is needed in the presence of localized CNS involvement.     

Cleveland Clinic experience with adrenal Cushing's syndrome

PURPOSE: Cushing's syndrome due to adrenal adenoma or adrenocortical carcinoma is rare. To understand better the clinical and biochemical presentation of this disorder, as well as therapy efficacy and patient survival, we conducted a retrospective review. MATERIALS AND METHODS: Between August 1971 and April 1994, 40 patients presented to our institution with adrenal Cushing's syndrome (27 adenomas and 13 carcinomas). These groups were analyzed with respect to clinical signs and symptoms preoperatively and postoperatively, biochemical analysis, length of postoperative steroid replacement therapy, disease recurrence and patient survival. Followup was obtained by chart review and telephone interviews and averaged 59.6 +/- 66.4 and 47.6 +/- 56.2 months for adenoma and carcinoma patients, respectively. RESULTS: Women predominated in both groups (26 of 27 adenomas, 11 of 13 carcinomas), and tumors affected the left adrenal gland more frequently (19 of 27 adenomas, 9 of 13 carcinomas). Adenoma patients were younger than carcinoma patients (39.6 +/- 14.4 versus 51.5 +/- 16.6 years, p = 0.026) and presented with smaller tumors (3.3 +/- 1.0 versus 8.6 +/- 4.5 cm., p = 0.001). There was a trend toward increased incidence of glucose intolerance among carcinoma patients but no significant differences in clinical signs or symptoms between adenoma and carcinoma patients could be made. Similarly, while there was no significant difference in biochemical evaluation of adenoma versus carcinoma patients, 24-hour urinary free cortisol and serum lactate dehydrogenase levels tended to be higher among carcinoma patients. In addition 17-ketosteroid and dehydroepiandrosterone sulfate levels were more elevated in carcinoma than in adenoma patients, and several adenoma patients actually had subnormal levels. Among adenoma patients mean length of steroid replacement therapy was 16.8 +/- 9.1 months. However, 7 adenoma patients (25.9%) required greater than 24 months of exogenous steroids, and only 1 of these patients was subsequently weaned off steroid replacement. There were no recurrences among adenoma patients, although there was 1 perioperative death due to hypoglycemia. Ten (76.9%) carcinoma patients had recurrences at a mean followup of 33 months. The 3 and 5-year survival rates were 41.5 and 31.2%, respectively. CONCLUSIONS: While presenting signs and symptoms and hormonal analysis may suggest benign or malignant disease, only tumor size and patient age are reliable preoperative indicators of adrenal adenoma versus adrenocortical carcinoma among patients with adrenal Cushing's syndrome. Surgery is curative for adenoma patients, but lifelong steroid replacement may be required. Survival remains poor among carcinoma patients.     

Video-assisted thoracoscopic surgery for spontaneous hemopneumothorax

PURPOSE: This study examined the usefulness of magnetic resonance imaging (MRI) in assessing whether parotid tumors were located in the superficial or deep lobe. PATIENTS AND METHODS: Eight patients with parotid gland tumors underwent MRI using a spin echo pulse sequence. T1- and T2-weighted images were obtained. To assess tumor localization in the parotid gland, two anatomic landmarks were used: 1) a line connecting the lateral surface of posterior belly of digastric muscle and lateral surface of the cortical bone of ascending ramus (facial nerve [FN] line) and 2) the relationship to the retromandibular vein (RV). Results of all MRI examinations were compared with the surgical findings. RESULTS: Seven of eight cases were correctly diagnosed using the FN line criterion. Five of eight cases were correctly diagnosed using the RV criterion. CONCLUSION: MRI is an excellent modality to show tumor localization in the parotid gland.     

Studies on the method of the analysis of sapogenin. III. Coulometric titration of diosgenin (author''s transl)

Fifteen of 146 (10%) adult patients with non-Hodgkin's lymphoma showed clinical and pathologic evidence of involvement of the central nervous system (CNS): in 6 patients, the CNS lymphoma was present at the onset of disease, in 3 of them it was the only sign detected. In the remaining 9 cases, CNS involvement appeared during the course of systemic disease. In all cases symptoms related to infiltration of the CNS were associated with advanced disease (stage IV); bone marrow or bone involvement was found in 9 patients (60%). The histologic subtypes were mostly of high-grade malignancy according to the Kiel classification: immunoblastic (3), centroblastic (3), Burkitt type (2), lymphoblastic (1), LP immunocytoma in polymorphic variant (3), unclassifiable (3). The prominent signs and symptoms of CNS lymphoma are listed: the cranial nerve palsies are the most common finding. The principal means of detecting CNS involvement are discussed: cerebrospinal fluid cytology, brain scan and CAT scan were the most useful diagnostic procedures. The reported data allow identification of patients at high risk of CNS lymphoma: this includes histologies of high-grade malignancy, advanced stage of disease, and bone marrow or bone infiltration. Therefore, either intensive systemic chemotherapy or CNS prophylaxis are recommended for patients with high risk of CNS disease.     

Hormonal regulation of the menstrual cycle (author's transl)

The menstrual cycle is regulated by a complex hormonal system with positive and negative feedback mechanisms and changes in sensitivity of peripheral tissues. Four concepts appear to be fundamental: -- regular, pulsatile secretion of LHR is necessary to the functioning of the system; -- regulation is to a great extent effected by the pituitary gland in response to changes in ovarian steroid levels; -- changes in ovarian steroid levels are due to regulatory changes in receptivity to pituitary hormones, as well as to variations in enzyme activities; -- at the periphery, changes in hormonal impacts are accompanied by modifications of receptivity to steroid hormones.     

Murine typhus presenting as subacute meningoencephalitis

Murine typhus is a febrile systemic illness, presenting with headache and undulating fever. Neurological involvement is considered a rare complication. During 1994 and 1995, 34 patients admitted to our hospital were diagnosed as having murine typhus. Five of these patients presented with a syndrome of subacute "aseptic" meningitis or meningoencephalitis. Three had bilateral papilloedema and 2 had focal neurological signs. None had a rash or other systemic findings suggestive of rickettsial disease. The diagnosis was based on serum and cerebrospinal fluid serology and on prompt response to doxycycline therapy. These cases suggest that neurological involvement in murine typhus is more common than previously suspected and that murine typhus should be included in the differential diagnosis of subacute meningitis in endemic areas.