Relapsing meningitis and confusion: limbic encephalitis revealing Hodgkin's disease

Introduction

An observation of limbic encephalitis associated with Hodgkin’s disease is compared with rare cases of the literature. The clinical presentation was relapsing episodes of febrile cognitive disorders with confusion and meningitis, curable after treatment of Hodgkin’s disease. Recent concepts on limbic encephalitis are discussed.

Case report

A seventy-five-year-old patient was hospitalized because of relapsing feverish confusion episodes with meningitis. During the year before his admission he had experienced four spontaneously regressive episodes of feverish confusion. Exploration of these episodes disclosed a paraneoplastic limbic encephalitis due to an underlying Hodgkin’s disease. The treatment of Hodgkin’s disease led to perfect recovery of cognitive function, so that the patient could drive his car.

Conclusion

Lymphoproliferative disease, such as Hodgkin’s disease, is a possible diagnosis in patients with limbic encephalitis. A dysimmune process underlying Hodgkin’s disease might be operating in this association.     

Intracranial atherosclerotic disease

Atherosclerotic disease of the intracranial arteries is responsible for at least 10% of ischaemic strokes worldwide. Symptomatic disease has been extensively studied in the past few years, using diagnostic methods including multi‐slice computed tomography and high resolution magnetic resonance imaging. A literature search was performed using PubMed and OvidSP between 1984 and May 2013. Variations of the terms ‘intracranial atherosclerosis’ plus ‘ischemic stroke’, ‘plaque’, ‘morphology’, ‘imaging’ were used and a combination of them. The reference lists of identified articles were also consulted for additional references. Amongst symptomatic patients the prevalence of intracranial atherosclerotic disease is around 10%, depending on race ethnicity, and the diagnosis requires the presence of ≥50% stenosis in the territory of the symptomatic vessel in a patient with stroke or transient ischaemic attack. The prognosis of intracranial atherosclerotic disease related stroke is poor. Although risk factor control can lead to a better outcome of intracranial atherosclerotic disease related strokes, the significance of asymptomatic disease is still a matter of debate.     

Intracranial localization of arachnoid granulations in rats

BACKGROUND： This paper describes histomorphologic studies on arachnoid granulations in rats, which have not been investigated in China to our knowledge. OBJECTIVE： To observe the distribution of intracranial arachnoid granulations in rats. DESIGN, TIME AND SETTING： The observational experiment was performed in the Academy of Life Sciences of Shandong Taishan Medical College from May to August 2004. MATERIALS： Thirty healthy adult Wistar rats （3-4 months old） of SPF grade, equal numbers of each sex, were selected for this study. Methylene blue parenteral solution was provided by Jiangsu Jichuan Pharmaceutical Company （China）, and an optical microscope （Type： CH20; Olympus Co. Ltd., Japan） was used for observation of the histomorphology of the arachnoid granulations. METHOD： Injection of methylene blue parenteral solution into the cerebellomedullary cistern of rats. MAIN OUTCOME MEASURE： The blue stained parts of the lateral sinus were sectioned, stained by hematoxylin and eosin, and then observed under the microscope. RESULTS： The cavitas subarachnoidealis had extensive blue staining after methylene blue injection, while the arachnoid and dura were without dye accumulation. The blue dye indicated the location of the arachnoid granulations. The location of these granulations was fixed, mainly in the lateral sinus at both sides of confluence within 4 mm of the internal jugular vein. CONCLUSION： The arachnoid granulations of the rat were located mainly in the lateral sinuses of the dura mater.     

Intracranial cortical localization of the human K-complex

ObjectiveThe K-complex was first identified in human sleep EEG more than 70 years ago, but the localization of its intracranial generators is an unresolved issue. In this study, K-complexes recorded using simultaneous scalp and intracranial EEG were analyzed to discover the intracranial distribution of the human K-complex.MethodsStereoelectroencephalographic recordings were performed in six patients with medically-refractory epilepsy. Full 10–20 scalp montages were used and intracranial macroelectrodes sampled medial, lateral and basal frontal and temporal cortices, medial and lateral parietal and occipital cortices, as well as the hippocampus and thalamus. Spontaneous K-complexes were visually identified in stage II sleep and averaged off-line.ResultsThe intracranial K-complex field was maximal over the anterior and superior aspects of the medial and lateral frontal lobe cortices, consistent with the frontal midline scalp EEG maximum. The frontal maximum surface-negative field was volume conducted as an inverted, positive field posteriorly and inferiorly, the polarity reversing laterally above the inferior temporal region and medially above the cingulate cortex.ConclusionsAs suggested by the scalp EEG topography, the intracranial distribution of the human K-complex is maximal over the anterior and superior frontal cortices. K-complex generation appears limited to cortical regions above the inferior temporal sulcus laterally, the cingulate sulcus medially and the parietooccipital junction posteriorly.SignificanceThe human K-complex is produced by synchronous cortical activity that appears maximal intracranially over the superior medial and lateral aspects of the frontal lobes. The cingulate cortex and functionally related mesial temporal structures appear uninvolved in human K-complex generation.     

Intracranial localization of arachnoid granulations in rats

BACKGROUND: This paper describes histomorphologic studies on arachnoid granulations in rats, which have not been investigated in China to our knowledge.OBJECTIVE: To observe the distribution of intracranial arachnoid granulations in rats. DESIGN, TIME AND SETTING: The observational experiment was performed in the Academy of Life Sciences of Shandong Taishan Medical College from May to August 2004.MATERIALS: Thirty healthy adult Wistar rats (3-4 months old) of SPF grade, equal numbers of each sex, were selected for this study. Methylene blue parenteral solution was provided by Jiangsu Jichuan Pharmaceutical Company (China), and an optical microscope (Type: CH20; Olympus Co. Ltd., Japan) was used for observation of the histomorphology of the arachnoid granulations.METHOD: Injection of methylene blue parenteral solution into the cerebellomedullary cistern of rats.MAIN OUTCOME MEASURE: The blue stained parts of the lateral sinus were sectioned, stained by hematoxylin and eosin, and then observed under the microscope.RESULTS: The cavitas subarachnoidealis had extensive blue staining after methylene blue injection, while the arachnoid and dura were without dye accumulation. The blue dye indicated the location of the arachnoid granulations. The location of these granulations was fixed, mainly in the lateral sinus at both sides of confluence within 4 mm of the internal jugular vein.CONCLUSION: The arachnoid granulations of the rat were located mainly in the lateral sinuses of the dura mater.     

Rigid spine syndrome revealing late-onset Pompe disease

The authors describe a 50-year-old man who was evaluated for a rigid spine syndrome with onset at age 15, and subsequent walking difficulties. Cardiac and pulmonary functions were normal. Deltoid biopsy revealed the presence of small vacuoles and increased glycogen with Periodic Acid Schiff staining in a limited number of fibers. Acid α-glucosidase staining was decreased in leucocytes, and genetic analysis identified the presence of two mutations in that gene. This observation suggests that Pompe disease should be considered in the differential diagnosis of rigid spine syndrome, even in patients without respiratory involvement or with a muscle biopsy showing only mild histopathological changes.     

Necrotizing myelopathy complicating Hodgkin's disease.

A case of necrotizing myelopathy is presented as a complication of Hodgkin's disease. The distinctive pathologic feature of this case was severe acute vasculitis involving only the vessels of the spinal cord that was considered to have been the cause of the segmental necrosis.     

Intracranial rebleeding in moyamoya disease.

Cerebral ischaemia and intracranial haemorrhage are the major catastrophic events in the natural course of moyamoya disease. To prevent cerebral ischaemia extracranial/intracranial (EC/IC) bypass surgery has been done and the results of the surgery have been well documented. However, the mechanism(s) underlying intracranial bleeding and the therapeutic modalities used to prevent bleeding have not been well elucidated. We have retrospectively analysed 45 cases with intracranial haemorrhage among 192 patients with moyamoya disease treated and followed at our institute. Analysis of the initial patients with haemorrhage did not provide any new information other than the already reported theories about the origin and mechanism of bleeding. Analysis of 15 patients in whom haemorrhage occurred at least twice suggested that there were two different mechanisms of rebleeding. One group consisted of 7 cases, where haemorrhages were confined to the original bleeding site. In 3 of these 7 cases a small aneurysm was identified at the periphery of a moyamoya vessel. In 1 case a ruptured aneurysm was found on a major vessel. In 3 other cases no source of bleeding was identified. In all but 1 of these cases rebleeding occurred within 2 months after the initial ictus. The second group consisted of 8 cases where haemorrhage occurred repeatedly but at variable sites. In none of these cases were aneurysms or other vascular abnormalities identified to suggest the source of bleeding and in all of these cases rebleeding occurred more than 2 months after the initial ictus. In moyamoya disease intracranial bleeding may occur as a result of rupture of a tiny aneurysm situated at the periphery of the moyamoya vessels and which may have been destroyed after the initial bleeding. If the aneurysms persist after rupture they may re-rupture after a fairly short interval. In other cases bleeding occurs at different sites from the initial haemorrhage; these are considered to be a result of rupture of weak moyamoya vessels which are forced to act as collateral pathways and are under unusually increased haemodynamic stress. EC/IC bypass surgery is considered to be effective for the prevention of rebleeding in both groups of patients but in selected cases. Direct surgery is recommended when a peripheral aneurysm is found on a moyamoya vessel which has bled once.     

Intracranial atherosclerotic disease: An update

The consensus conference on intracranial atherosclerosis provides a comprehensive review of the existing literature relevant to the epidemiology, diagnosis, prevention, and treatment of intracranial atherosclerosis, and identifies principles of management and research priorities. Patients who have suffered a stroke or transient ischemic attack attributed to stenosis (50–99%) of a major intracranial artery face a 12 to 14% risk for subsequent stroke during the 2‐year period after the initial ischemic event, despite treatment with antithrombotic medications. The annual risk for subsequent stroke may exceed 20% in high‐risk groups. In patients with intracranial atherosclerotic disease, short‐term and long‐term anticoagulation is not superior to antiplatelet treatment. Overall, the subgroup analyses from randomized trials provide evidence about benefit of aggressive atherogenic risk factor management. Intracranial angioplasty with or without stent placement has evolved as a therapeutic option for patients with symptomatic intracranial atherosclerotic disease, particularly those with high‐grade stenosis with recurrent ischemic symptoms, medication failure, or both. A multicenter randomized trial is currently under way to compare stent placement with intense medical management for patients with high‐grade symptomatic intracranial atherosclerotic disease. Ann Neurol 2009;66:730–738     

Primary Hodgkin's disease of the falx cerebri

Summary A case of the falx tumor of a 60-year-old man is reported. The tumor consisted of a pleomorphic mixture of lymphoid cells, histiocytes, atypical reticulum cells, and bizarre giant cells, including the diagnostic Reed-Sternberg cells. No transitional forms among these cells were observed. This is the first report of primary Hodgkin's disease of the dura mater, as verified by complete necropsy.