DOPA-Responsive Tremor Associated With Gammopathy: A Case Report and Literature Review.

Tremors have been well-described in association with monoclonal gammopathy. We report a case of a patient with asymmetric hands tremor who responded well to levodopa-carbidopa treatment. Further workup showed an underlying gammopathy. To our knowledge, this is one of the rarest case reports of successful treatment of gammopathy-related tremors with levodopa-carbidopa. The patient was a 75-year-old male who presented to the neurology clinic for a one-year history of worsening tremors in bilateral upper extremities. A review of systems was only remarkable for mild numbness and tingling in his feet. His neurological examination was remarkable for asymmetric right more than left (R > L) resting and kinetic tremor in both upper extremities associated with mild rigidity and bradykinesia in right upper extremity and decreased bilateral ankle jerks. With the primary diagnosis of Parkinson's disease, he was started on levodopa-carbidopa and a neuropathy workup was requested. His follow-up visit after three months was remarkable for significant improvement of his tremors with carbidopa-levodopa. However, his blood work was consistent with a significant increase in lambda light chain levels and the presence of an M spike in serum protein electrophoresis. Based on the presentation and clinical workup, he was finally found to have both multiple myeloma and ​Waldenstrom's macroglobulinemia. Underlying malignancy was treated with chemotherapy and immunotherapy. Levodopa-carbidopa was discontinued after three months of chemotherapy and his tremor did not recur in one year of follow-up. Gammopathy is one of the well-known causes of tremors in the adult population. It can cause both resting and kinetic tremors in the upper extremities. It is supposed that peripheral neuropathy associated with gammopathy is the main underlying cause of tremors in these groups of patients. However, central causes are also suggested. In this case, we are led to conclude that our patient's tremor was centrally mediated since it responded well to dopamine replacement therapy. However, further study is needed to elucidate the role of dopamine depletion in the pathogenesis of tremors associated with gammopathies.

Craniopagus, Laleh and Ladan twins, sagital sinus.

Adult craniopagus twins aged 29 years who succumbed to the surgical separation are being presented posthumously. Neuroimaging studies had revealed a total type 3 craniopagus with a common superior sagittal sinus beginning at its distal half and draining into a common Torcular Herophili. In July 2003, neurosurgeons at Singapore embarked on the mission to tackle the separation of the most complex and challenging adult craniopagus of the present millennium, unfortunately ending up with devastating results. The authors intend to expand upon their life style, shared intracranial structures, the potential risks of a one-stage procedure as carried out in this case and finally offer some suggestions in tackling such rare and complex cases in neurosurgery.

Cutoff values of plasma d-dimer level in patients with diagnosis of the venous thromboembolism after elective spinal surgery.

STUDY DESIGN: A prospective clinical study. PURPOSE: Our objective in the present study was twofold. First, we sought to evaluate the relationship between postoperative venous thromboembolism (VTE) development and concentration of D-dimer to determine the cutoff value in patients who underwent elective spinal surgery. Second, we identified the predictive risk factors for postoperative VTE. OVERVIEW OF LITERATURE: VTE affects the general health of patients and may even cause death. Since the complications of VTE are difficult to predict, the safest and most cost effective diagnostic method should be used in order to confirm a suspected VTE event after spinal surgery. METHODS: This study was performed on 97 patients who underwent elective spinal surgery. The D-dimer assay was carried out on the day before surgery, and on days 1, 3, and 10 following surgery. VTE occurrence and D-dimer levels were compared between the VTE and the control groups. RESULTS: Four patients (4.1%) were diagnosed with deep vein thrombosis and one patient (1%) was diagnosed with a pulmonary embolism. The optimum D-dimer cutoff value on day 3 following surgery in the VTE group was determined to be more than 2.1 µg/mL with a sensitivity of 100% and a specificity of 80.7%. Moreover, the duration of postoperative recumbency was a significant risk factor for the development of VTE in this study. CONCLUSIONS: We have demonstrated that postoperative D-dimer measurements in patients who underwent elective spinal surgery can provide a complementary diagnostic screening for VTE during the first week after surgery.

Incidence of venous thromboembolic complications in instrumental spinal surgeries with preoperative chemoprophylaxis.

OBJECTIVE: Venous thromboembolism (VTE) after spinal surgery affects a patients' postoperative recovery and also carries a mortality risk. Some studies recommended chemical prophylaxis for high-risk patients and for those after complex spinal surgeries. However, chemoprophylaxis for VTE in spinal surgery is underemployed and there is no agreement on the use of VTE prophylaxis in spinal surgery. The aim of this study was to document the incidence of VTE after an elective instrumental spinal surgery, among those receiving preoperative chemoprophylaxis as compared with patients who did not receive it. METHODS: This study was carried out on eighty-nine patients allocated randomly to receive either low molecular weight heparin (LMWH) or no prophylaxis before elective instrumental spinal surgery. All patients received postoperative compression stockings. A compression Doppler ultrasonography was performed for all patients to detect postoperative deep vein thrombosis. In addition, further imaging studies were performed for patients suspected of VTE. RESULTS: Three (3.3%) patients were diagnosed with VTE. One of them had received preoperative chemoprophylaxis. There were no significant difference in incidence of VTE between the two groups (p>0.95; 95% confidence interval, 0.06-8.7). Laterality of gender and postsurgical recumbence duration were all independent predictors of VTE (p=0.01 and p<0.001, respectively). CONCLUSION: The difference in the incidence of thromboembolic complications between the two groups was not significant. Moreover, we found that preoperative prophylactic LMWH injection has no major bleeding complications altering postoperative course; still, the issue concerning the initiation time of chemoprophylaxis in spinal surgery remains unclear.

Cerebral phaeohyphomycosis masquerading as a parafalcian mass: case report.

BACKGROUND: Cerebral phaeohyphomycosis caused by Fonsecaea pedrosoi is a rarity. However, about four cases have been reported in the literature. The disease remains mostly fatal despite employment of new treatment modalities. CASE: An 18-year-old boy presented seizures of recent onset. Two years back, he developed cutaneous phaeohyphomycosis after a splinter scratch on his chest wall. Imaging revealed a contrast enhancing parafalcian solid mass. Right frontal parasagittal craniotomy was performed and the lesion resected as much as possible, followed by IV amphotericin B and oral itraconazole treatment. The patient has been doing well during a 15-month follow-up period. DISCUSSION: Cerebral phaeohyphomycosis is an extremely rare lesion, which could masquerade as a parafalcian mass. Radical surgical removal together with antimicrobials remains the cornerstone treatment of cerebral phaeohyphomycosis.