Etiology and therapy of the internal jugular vein thrombosis

BACKGROUND: Thromboses of the upper limp and neck are rare in comparison with those of the lower extremities. Internal jugular vein thrombosis (IJVT) is a serious event with a potentially fatal outcome. Complications include pulmonary embolism, sepsis with septic emboli to different organs and tissues as well as intracranial propagation of the thrombus with cerebral edema. As any thrombosis, IJVT is precipitated by Virchow's triad: endothelial damage, alteration of blood flow and hypercoagulability. The history and examination in patients with an IJVT may be vague and misleading. Patients may present with a painful swelling of the neck but they may also be absolutely asymptomatic. Imaging procedures frequently used to diagnose an IJVT include sonography with color-coded duplex sonography, computed tomography, magnetic resonance imaging as well as magnetic resonance venography. Up to date, there is no standardized treatment regimen for patients with an IJVT. PATIENTS AND METHODS: This retrospective study includes all ten patients with an IJVT who were seen at our department between January 2000 and January 2004. There were six female and four male patients. The average age was 49.7 years, ranging from 28 to 79 years. RESULTS: In five cases, the thrombosis was associated with a malignant tumor. In four patients, it was caused by a deep neck space infection and in one case the IJVT was due to cervical, intravenous drug abuse. Two patients were found to be pregnant (one tumor patient and one patient with a deep neck space infection). In all cases, a ten day treatment regimen with intravenous antibiotics and anticoagulant therapy was initiated. Oral or subcutaneous anticoagulation was continued for six weeks to six months. No complications were seen in any patient. In three patients a revascularization of the affected vessel could be demonstrated with color-coded duplex sonography six months after the initial presentation. CONCLUSIONS: Thrombosis of the IJV is probably underdiagnosed. Since the clinical presentation may be vague or misleading, a high degree of suspicion is required to make the diagnosis. The potential complications such as pulmonary embolism or intracranial propagation of the thrombus may be fatal. Whenever the thrombosis is not caused by an inflammatory process, a malignant tumor should be excluded. We recommend a therapy with intravenous antibiotics as well as a systemic anticoagulation. Ligation or resection of the internal jugular vein is reserved for patients who develop complications despite adequate medical therapy.     

Subtemporal approach according to house versus Wigand in intrameatal CPA tumors type A

BACKGROUND: The subtemporal approach is indicated in intrameatal CPA tumors type A in order to preserve hearing. The exploration of the inner auditory canal for tumor exposure varies. It reaches from a locally limited uncovering of the bony inner auditory canal to a complete removal of the surrounding bony bed with the circular skeletization (360 degrees ) according to Wigand of the 7 (th) and 8 (th) nerve. Concerning the preservation of the function of the cranial nerve as well as an avoidable hyperelevation of the temporal cerebral lobe with a possible consecutive organic brain syndrome, both approaches have often been discussed controversially. METHOD: In a quality assurance analysis, we examined patients suffering from a unilateral, intrameatally limited CPA tumor type A in tumor stages 1 to 5. The functions of the 7 (th) and 8 (th) cranial nerves were assessed according to the consensus conference "Systems for reporting results in acoustic neuroma", Tokyo, November 2001, under consideration of the recommendations of the "American Academy of Otolaryngology, Head and Neck Surgery -- Committee on hearing and equilibrium guidelines for the evaluation of hearing preservation in acoustic neuroma", 1995, as well as indications for a possible organic brain syndrome. The results then were compared to current literature. RESULTS: 37 patients with an intrameatal confined CPA tumor after subtemporal tumor exstirpation were evaluated. In these patients, the inner auditory canal was only exposed in the area of the bony tectum (90 degrees - 120 degrees ) adjusted to the volume of the tumor, as described by House: 1 patient with tumor stage 1, 2 patients stage 2, 12 patients stage 3, 16 patients stage 4 and 6 patients with a tumor stage 5. The N. VII was anatomically preserved in 100 %. Immediately after surgery the function of N. VII was assessed in 32 % of the cases as stage I, 43 % stage II, 3 % stage IIIa, 14 % stage IIIb, 3 % stage IV, 0 % stage V, 5 % stage VI. All patients in stage VI had a restricted function of N. VII in stage II or IIIa already before surgery. N. VIII could be preserved anatomically in 78 % of the cases. 1 to 6 months postoperatively the hearing was at stage A in 10 % of the cases, at 25 % in stage B, 33 % in stage C, 16 % in stage D, 5 % in stage E and 11 % in stage F. One patient demonstrated signs of a possible organic brain syndrome postoperatively even after 12 months of latency (headache, unsteady walking, attention disorders). CONCLUSION: When comparing own data with the clinical results from literature, no differences can be found concerning the function of the 7 (th) and 8 (th) cranial nerves. There are no data concerning the incidence of a potential organic brain syndrome. The advantage of the House method is a noticeably reduced drilling time as well as possibly a reduced elevation of the temporal cerebral lobes. The Wigand method, however, allows a better overview and is needed for extrameatally growing tumors anyway.     

Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations

Context  Germline mutations of the genes encoding succinate dehydrogenase subunits B (SDHB) and D (SDHD) predispose to paraganglioma syndromes type 4 (PGL-4) and type 1 (PGL-1), respectively. In both syndromes, pheochromocytomas as well as head and neck paragangliomas occur; however, details for individual risks and other clinical characteristics are unknown. Objective  To determine the differences in clinical features in carriers of SDHB mutations and SDHD mutations. Design, Setting, and Patients  Population-based genetic screening for SDHB and SDHD germline mutations in 417 unrelated patients with adrenal or extra-adrenal abdominal or thoracic pheochromocytomas (n = 334) or head and neck paragangliomas (n = 83), but without syndromic features, from 2 registries based in Germany and central Poland, conducted from April 1, 2000, until May 15, 2004. Main Outcome Measures  Demographic and clinical findings with respect to gene mutation in SDHB vs SDHD compared with nonmutation carriers. Results  A total of 49 (12%) of 417 registrants carried SDHB or SDHD mutations. In addition, 28 SDHB and 23 SDHD mutation carriers were newly detected among relatives of these carriers. Comparison of 53 SDHB and 47 SDHD total mutation carriers showed similar ages at diagnosis but differences in penetrance and of tumor manifestations. Head and neck paragangliomas (10/32 vs 27/34, respectively, P<.001) and multifocal (9/32 vs 25/34, respectively, P<.001) tumors were more frequent in carriers of SDHD mutations. In contrast, SDHB mutation carriers have an increased frequency of malignant disease (11/32 vs 0/34, P<.001). Renal cell cancer was observed in 2 SDHB mutation carriers and papillary thyroid cancer in 1 SDHB mutation carrier and 1 SDHD mutation carrier. Conclusions  In contrast with SDHD mutation carriers (PGL-1) who have more frequent multifocal paragangliomas, SDHB mutation carriers (PGL-4) are more likely to develop malignant disease and possibly extraparaganglial neoplasias, including renal cell and thyroid carcinomas. Appropriate and timely clinical screening is recommended in all patients with PGL-1 and PGL-4.      

Clinical reasoning and decision making in practice. A depressive foreign woman with symptoms of malaise

A 27-year-old woman was admitted to the hospital with a depression, anaemia and fatigue. She had come from Angola to the Netherlands as a refugee 2 years before this evaluation. As an explanation for her symptoms tropical infectious diseases of parasitic origin were considered, but no clues were found in this direction. The test for trypanosomiasis was considered to be suggestive for an infection in the past (persistent titre 1:200). She was discharged but readmitted 6 months later because of a deterioration of her clinical condition. Magnetic resonance imaging showed bilateral signal abnormalities within the white matter of the brain. On examination no neurological signs or abnormalities were found. Again, no definite diagnosis could be made and the patient was discharged. Because of a further deterioration of her clinical condition she was readmitted a short time later for the third time. On the MRI the white matter lesions had increased. The serum protein electrophoresis was markedly abnormal with an elevated IgM Level. Finally, at a repeated lumbar puncture mobile trypanosomes were found. The diagnosis of 'West African sleeping sickness' was made and the patient was treated with eflornithine. She recovered completely during the next 18 months.     

Differential effects of various treatment combinations on cardiovascular dysfunction in patients with Parkinson's disease

OBJECTIVES: Patients with Parkinson's disease (PD) frequently suffer from cardiovascular dysfunction, which may be enhanced to various extents by different antiparkinsonian drugs. MATERIALS AND METHODS: We analysed electrocardiogram (ECG) abnormalities, cardiovascular reflexes (CVR) and orthostatic hypotension (OH) in 148 patients with idiopathic PD assigned to five different combination therapies of levodopa (LD) plus either bromocriptine (BRO), ropinirole (ROP), selegiline (SEL), anticholinergic (ACH) or amantadine (AMA) or to LD monotherapy before and after a 1-week washout of the add-on drug. Patients were matched for age and disease severity (Hoehn and Yahr stage 2-3). Rater-blinded cardiovascular testing was performed at baseline, and following a 1-week washout period of the add-on drugs. RESULTS : We found that the incidence of cardiovascular dysfunction was generally higher in patients receiving a combination therapy compared with patients on LD monotherapy. ECG abnormalities were found in 40-52% of patients in combination therapy, but in only 20% of the patients receiving LD monotherapy. After discontinuation of BRO and SEL, there were significant improvements in ECG, OH and CVR. After washout of ACH and AMA, a significant improvement was found only in the CVR score. AMA and ROP were the add-on drugs with the least adverse cardiovascular effects. CONCLUSION: We conclude that pre-existing cardiovascular autonomic dysfunction should be investigated and taken into account when deciding which combination therapy to choose in the treatment of parkinsonian patients.     

Outcomes after resection of giant emphysematous bullae

Background

Giant emphysematous bullae represent a rare form of emphysematous lung destruction. Surgical resection has traditionally been indicated when there is hyperexpansion of the chest, compromised pulmonary function, and evidence of underlying, relatively normal compressed lung. We review our experience and intermediate-term follow-up after the resection of giant bullae.

Methods

Forty-three patients underwent resection of giant emphysematous bullae at Barnes-Jewish Hospital between March 1994 and June 2002. All had limiting dyspnea and radiologic evidence of hyperinflated giant bullae compressing adjacent lung parenchyma. Forty-one patients underwent preoperative pulmonary rehabilitation. Twenty-two patients underwent a bilateral procedure and 21 underwent a unilateral procedure. Mean follow-up was 4.5 years.

Results

One early death occurred on postoperative day 20 from heparin-induced thrombocytopenia and pulmonary embolism. Complications included prolonged air leak of more than 7 days in 23 (53%), atrial fibrillation in 5 (12%), postoperative mechanical ventilation in 4 (9%), and pneumonia in 2 (5%). Kaplan-Meier survival at 1, 3, and 5 years was 98%, 92%, and 89%, respectively. Four late deaths occurred at 1.4, 2.8, 3.5, and 5.9 years. Functional measures preoperatively and at 6 months and 3 years postoperatively were a forced expiratory volume in 1 second L (% predicted) of 1.2 ± 0.6 (34%), 1.9 ± 0.9 (55%), and 1.5 ± 0.8 (49%); residual volume L (% predicted) of 5.1 ± 1.2 (262%), 3.6 ± 1.2 (154%), and 4.1 ± 2.2 (209%); 6-minutes walk (ft) of 1230 ± 361, 1393 ± 300, and 1271 ± 423; supplemental O₂ used continuously (% patients) of 42%, 9%, and 21%; and O₂ used during exercise of 73%, 37%, and 42%, respectively.

Conclusions

In a contemporary series, giant bullectomy is shown to produce significant immediate functional improvement. This benefit declines with time but persists at least 3 years.     

Assessment of suspected dementia

At the Second Canadian Consensus Conference on Dementia (CCCD) (February, 1998), a group of neurologists, geriatricians, and psychiatrists met to consider guidelines for evaluation of dementia in Canada. This review paper formed a background paper for their discussion of dementia diagnosis. These experts from across the country concluded that diagnosis of suspected dementia cases continued to rest on skilled clinical assessment. Mental status exam, preferably in some quantifiable form, has become an essential part of the assessment. Selected laboratory tests are advisable in all cases (CBC, TSH, electrolytes, calcium, and glucose), but the CCCD continued to advise that CT scanning was mandatory only in selected cases where clinical findings pointed to another possibility besides Alzheimer's disease. The growing list of other diagnostic measures with potential usefulness in diagnosis of Alzheimer's disease or dementia in general was reviewed, but the evidence was judged as insufficient to support routine use of these tests by physicians. As new treatments for Alzheimer's disease become available, neurologists face new diagnostic challenges--differentiating Mild Cognitive Impairment, Frontotemporal dementias and Mixed dementias, and Lewy Body Dementia. Guidelines to aid in differential diagnosis are presented.     

Workshop I: Parkinson's disease and sleep--results of the group discussion

The group agreed on the facts that unwanted sleep onset has been observed after non-ergot as well as ergot dopamine agonists, that patients on these drugs need to be warned, that patients who have experienced sleepiness already must not drive a car unless the dosage is lowered and sleepiness has vanished, that a genetic predisposition for narcoleptic cataplexy has to be ruled out, that predictors of so-called sleep attacks need to be explored individually with the help of sleepiness scales and collectively in a careful study, respectively.