scholarly journals Diagnosis, treatment, and misdiagnosis analysis of 28 cases of central nervous system echinococcosis

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Guojia Du ◽  
Yandong Li ◽  
Pan Wu ◽  
Xin Wang ◽  
Riqing Su ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Clinical Features ◽  
Multiple Organ ◽  
Pathological Examination ◽  
Surgical Removal ◽  
Surgical Treatments

Abstract Background To explore central nervous system (CNS) involvement in this disease, from the perspectives of diagnosis, treatment, and misdiagnosis Methods Twenty-eight patients with CNS echinococcosis were included in this retrospective study, including 18 males (64.3%) and 10 (35.7%) females. The average age of all the patients were 23.5 years (ranged 4–60 years). Twenty-three (23) patients (82.1%) received the first surgical resection in our hospital. Five (5) patients (17.9%) gave up surgical treatment for multiple-organ hydatidosis and previous surgery history at other hospitals, and albendazole was applied for a long-term (3–6 months) adjunct therapy for the 5 patients. The average follow-up time was 8 years. Results For the 28 patients, 23 cases received surgical treatments, and the diagnosis was confirmed by pathological examinations. The diagnosis of 4 cases of brain echinococcosis and 2 cases of spinal cord echinococcosis could not be confirmed, resulting in a misdiagnosis rate of 21.4% (6/28). For the pathological examination, a total of 17 cases were infected with Echinococcus granulosus (including 2 cases of spinal cord echinococcosis), and 6 cases were infected with Echinococcus alveolaris. Conclusion The diagnosis should be specifically considered in endemic regions. The clinical features of CNS hydatidosis were intracranial space-occupying lesions. For the treatment, the surgical removal of cysts should be necessary. In addition, the adjuvant therapy with drug and intraoperative prophylaxis is also suggested. The misdiagnosis may have resulted from atypical clinical features and radiographic manifestations, as well as the accuracy of hydatid immunologic test.

Congenital Brain Anomalies Associated With the Hypoplastic Left Heart Syndrome

PEDIATRICS ◽  
1990 ◽  
Vol 85 (6) ◽  
pp. 984-990 ◽  
Author(s):  
Tracy A. Glauser ◽  
Lucy B. Rorke ◽  
Paul M. Weinberg ◽  
Robert R. Clancy
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hypoplastic Left Heart Syndrome ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Brain Anomalies ◽  
Physical Features ◽  
Left Heart Syndrome

This study details the type, frequency, and clinical presentation of developmental brain anomalies in 41 infants with the hypoplastic left heart syndrome encountered during a 52-month interval. Overall, 29% of the infants had either a major or minor central nervous system abnormality. Overt central nervous system malformations, including 3 cases of agenesis of the corpus callosum and 1 case of holoprosencephaly, were seen in 4 infants (10%). Micrencephaly (brain weight at autopsy more than 2 SDs below the mean for age) was found in 27% of the infants. An immature cortical mantle was seen in 21% of the study group. Seven infants (17%) had specific recognizable patterns of malformation. The absence of dysmorphic physical features did not preclude overt or subtle central nervous system malformations. Conversely, the presence of dysmorphic features did not reliably indicate an underlying brain anomaly. Infants who had hypoplastic left heart syndrome as one of multiple nonneurologic malformations were more likely to have micrencephaly than those infants with hypoplastic left heart syndrome as an isolated abnormality. Occurrence of developmental neuropathology was elevated in those infants with hypoplastic left heart syndrome who did not have a recognizable pattern of malformation but who were small for gestational age, microcephalic, or had ocular abnormalities. Infants with hypoplastic left heart syndrome deserve careful genetic, ophthalmologic, and neurologic evaluations, imaging of their intracranial anatomy, and long-term neurologic follow-up.

Cognitive Outcomes and Long-term Follow-up Results after Enhanced Chemotherapy Delivery for Primary Central Nervous System Lymphoma

Neurosurgery ◽  
2000 ◽  
pp. 51-61 ◽  
Author(s):  
Leslie D. McAllister ◽  
Nancy D. Doolittle ◽  
Paul E. Guastadisegni ◽  
Dale F. Kraemer ◽  
Cynthia A. Lacy ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Central Nervous System Lymphoma ◽  
Cognitive Outcomes ◽  
Long Term Follow Up

Long-Term Follow-Up of Dose-Adapted and Reduced-Field Radiotherapy With or Without Chemotherapy for Central Nervous System Germinoma

2010 ◽  
Vol 77 (5) ◽  
pp. 1449-1456 ◽  
Author(s):  
Ashley W. Jensen ◽  
Nadia N. Issa Laack ◽  
Jan C. Buckner ◽  
Paula J. Schomberg ◽  
Cynthia J. Wetmore ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Long Term Follow Up

Acute Disseminated Encephalomyelitis

2017 ◽  
Author(s):  
Benjamin M. Greenberg ◽  
Allen Desena
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Supportive Care ◽  
Acute Disseminated Encephalomyelitis ◽  
Autoimmune Response ◽  
Inflammatory Disorder ◽  
The Central Nervous System ◽  
The Brain

Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory disorder of the central nervous system (CNS) that can be fatal or lead to long-term disability. Various triggers have been identified in children and adults, which presumably cause an autoimmune response targeting myelin. The resulting inflammation causes demyelination and edema of the brain, spinal cord, and optic nerves. Depending on which portion of the CNS is affected, patients will experience a variety of symptoms including weakness, numbness, ataxia, encephalopathy, and seizures. Treatment is currently focused on reducing the amount of inflammation and supportive care.

scholarly journals Clinicopathological and imaging features of lipoastrocytoma: Case report

2017 ◽  
Vol 31 (1) ◽  
pp. 32-38
Author(s):  
Laxminadh Sivaraju ◽  
Saritha Aryan ◽  
Nandita Ghosal ◽  
Alangar S Hegde
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Pediatric Population ◽  
Pleomorphic Xanthoastrocytoma ◽  
Imaging Features ◽  
Total Excision ◽  
Favorable Prognosis ◽  
The Central Nervous System

Lipidized tumors of the central nervous system are very uncommon, with only a few cases described. We report a case of a 25-year-old woman with a tumor involving the left premotor area. She underwent gross total excision. Histologically, the tumor was composed of glial fibrillary acidic protein-positive glial cells with areas of lipidization. A diagnosis of lipoastrocytoma was rendered. At three-year follow-up she was doing well, supporting the presumed favorable prognosis of these uncommon tumors. Absence of xanthochromic appearance, mitotic activity, necrosis and poor reticulin activity are the differentiating features from the pleomorphic xanthoastrocytoma. We highlighted that these tumors involve the adult and pediatric population and distribute in both supratentorial and infratentorial compartments as well as in the spinal cord.

VHL-RELATED NEUROENDOCRINE NEOPLASMS AND BEYOND: AN ISRAELI SPECIALIZED CENTER REAL-LIFE REPORT

2020 ◽  
Vol 26 (10) ◽  
pp. 1131-1142
Author(s):  
Auryan Szalat ◽  
Kira Oleinikov ◽  
Avital Nahmias ◽  
Vardiella Meiner ◽  
Simona Ben-Haim ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Neuroendocrine Tumor ◽  
Neuroendocrine Neoplasms ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Von Hippel Lindau ◽  
Mutation Carriers ◽  
Long Term Follow Up

Objective: Von Hippel-Lindau (VHL) syndrome is a rare and complex disease. In 1996, we described a 3 generation VHL 2A kindred with 11 mutation carriers. We aim to share our experience regarding the long-term follow-up of this family and the management of all our other VHL patients focusing on frequently encountered neuroendocrine neoplasms: pheochromocytoma/paraganglioma and pancreatic neuroendocrine neoplasms (PNEN). Methods: All VHL patients in follow-up at our tertiary center from 1980 to 2019 were identified. Clinical, laboratory, imaging, and therapeutic characteristics were retrospectively analyzed. Results: We identified 32 VHL patients in 16 different families, 7/16 were classified as VHL 2 subtype. In the previously described family, the 4 initially asymptomatic carriers developed a neuroendocrine tumor; 7 new children were born, 3 of them being mutation carriers; 2 patients died, 1 due to metastatic PNEN-related liver failure. Pheochromocytoma was frequent (22/32), bilateral (13/22;59%), often diagnosed in early childhood when active screening was timely performed, associated with paraganglioma in 5/22, rarely malignant (1/22), and recurred after surgery in some cases after more than 20 years. PNEN occurred in 8/32 patients (25%), and was metastatic in 3 patients. Surgery and palliative therapy allowed relatively satisfactory outcomes. Severe disabling morbidities due to central-nervous system and ophthalmologic hemangiomas, and other rare tumors as chondrosarcoma in 2 patients and polycythemia in 1 patient were observed. Conclusion: A multidisciplinary approach and long-term follow-up is mandatory in VHL patients to manage the multiple debilitating morbidities and delay mortality in these complex patients. Abbreviations: CNS = central nervous system; HIF = hypoxia inducible factor; PGL = paraganglioma; PHD = prolyl hydroxylase; Pheo = pheochromocytoma; PNEN = pancreatic neuroendocrine neoplasms; PNET = pancreatic neuroendocrine tumor; RCC = clear cell renal cell carcinoma; VHL = Von Hippel-Lindau

Hemangioblastomas of the Central Nervous System in von Hippel-Lindau Syndrome and Sporadic Disease

Neurosurgery ◽  
2001 ◽  
Vol 48 (1) ◽  
pp. 55-63 ◽  
Author(s):  
James E. Conway ◽  
Dean Chou ◽  
Richard E. Clatterbuck ◽  
Henry Brem ◽  
Donlin M. Long ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Von Hippel Lindau ◽  
Multiple Lesions ◽  
Younger Age ◽  
The Central Nervous System ◽  
Sporadic Disease ◽  
Vhl Disease

Abstract OBJECTIVE The presentation, screening, management, and clinical outcomes of patients who presented to our institution from 1973 to 1999 with central nervous system (CNS) hemangioblastomas in von Hippel-Lindau (VHL) syndrome and sporadic disease were analyzed. METHODS The surgical pathology database of our institution was searched to identify all patients with histologically verified CNS hemangioblastomas occurring from 1973 to 1999. The medical, radiological, surgical, pathological, and autopsy records from these patients were reviewed retrospectively and statistically analyzed. RESULTS Forty patients (21 males and 19 females) presented with CNS hemangioblastomas. Twenty-five patients (62%) harbored sporadic hemangioblastomas. Fifteen patients (38%) had VHL syndrome. These 40 patients presented with 61 hemangioblastomas (8 patients had multiple lesions). Ten patients (25%) harbored spinal cord hemangioblastomas (5 patients had multiple lesions). Patients with VHL disease tended to present with neurological symptoms and signs at a younger age than patients with sporadic disease (P = 0.09), to present with multiple lesions (53%), and to develop new lesions (rate, 1 lesion/2.1 yr). Hemangioblastomas of the spinal cord were more prevalent in patients with VHL syndrome (P = 0.024). Neuroradiological screening of patients with VHL syndrome allowed identification of more than 75% of new lesions before they became symptomatic. Sixty-six surgical procedures were performed (12 patients required multiple operations). Six patients with VHL syndrome required surgery for new lesions. Surgical complications occurred in six patients (15%). Symptom resolution or arrest of progression at 1 year was documented in 88% of patients. Recurrence of symptoms from partially resected lesions occurred in eight patients (20%). No deaths associated with surgery occurred. One patient with sporadic disease and one patient with VHL syndrome (5%) died as a result of late medical complications from CNS hemangioblastomas. CONCLUSION Surgical outcomes for patients with CNS hemangioblastomas are favorable. However, management of hemangioblastomas is a more difficult and prolonged endeavor for patients with VHL syndrome. In patients with VHL syndrome, neuroradiological screening allows identification of lesions before they become symptomatic. Because patients with VHL syndrome are at risk for development of new lesions, they require lifelong follow-up.

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