Bilateral posteroventral pallidotomy in advanced Parkinson's disease in three patients

In this report, we describe the effect of staged bilateral posteroventral pallidotomy in three patients with advanced Parkinson's disease who were all of the young-onset type. Two patients had developed response fluctuations after the use of levodopa, with severe hypokinesia, painful dystonia, and rigidity in the "off" phase and violent dyskinesias in the "on" phase. One patient, in a continuous hypokinetic rigid state, was totally unresponsive to dopaminergic medication. All were at Hoehn and Yahr stage 5 in the "off" phase before surgery. After surgery, the hypokinetic state was reversed and dyskinesias were abolished in all patients. Hoehn and Yahr stages were 3 in the "off" phase postoperatively. Overall functional improvement was marked and lasting after follow-up for 7, 12, and 13 months, respectively. Complications were visual field deficit and transient central facial paresis, both in the same patient. Bilateral posteroventral pallidotomy can ameliorate response fluctuations, hypokinesia, rigidity, and painful dystonia in advanced Parkinson's disease.     

Medial posteroventral pallidotomy for the treatment of Parkinson's disease]

Stereotactic medial posteroventral pallidotomy for treatment of Parkinson's disease attracts increasing attention. We report on the preliminary results of 12 patients at 1 year after microelectrode-guided unilateral pallidotomy. The primary indications were severe bradykinesia and levodopa-induced dyskinesias. After radiofrequency lesioning all patients had immediate improvement of contralateral parkinsonian signs. Postoperative magnetic resonance imaging confirmed the localization of the lesions. At the 1-year follow-up, all patients had sustained benefit. The global improvement was rated as moderate in six cases, and as marked in six other cases. The mean values of various subscores of the Unified Parkinson's Disease Rating Scale (UPDRS) showed highly significant changes in the "off" state (pre/postoperatively): UPDRS Motor score (60.3/31). UPDRS Activities of Daily Living (ADL) score (33.2/18.3), gait/postural stability score (13.8/7.0), and subscores for contralateral rigidity (4.9/2.1), tremor (7.1/1.4) and bradykinesia (11.6/5.3). There was also significant improvement of ipsilateral bradykinesia and rigidity. Furthermore, we found significant changes of the mean values of the UPDRS ADL and motor "on" scores, an increase of the percentage of "on" time with reduced on-off fluctuations, and a decrease of the percentage of levodopa-induced dyskinesias, with marked improvement or complete abolition of contralateral dyskinesias in particular. The preoperative levodopa regimen was maintained, in general, or only slightly modified, if necessary. Two patients had transient complications: one patient suffered postoperative pneumonia and altered mental status; another patient displayed mild Broca's aphasia secondary to a small stroke involving the dorsal thalamus and the adjacent white matter. There were no persistent side effects at the 1-year follow-up. Contemporary unilateral pallidotomy is an effective and promising therapeutical option for surgical treatment of advanced Parkinson's disease.     

Neuropsychological consequences of posteroventral pallidotomy for the treatment of Parkinson''s disease

The proapoptotic protein BAX contains a single predicted transmembrane domain at its COOH terminus. In unstimulated cells, BAX is located in the cytosol and in peripheral association with intracellular membranes including mitochondria, but inserts into mitochondrial membranes after a death signal. This failure to insert into mitochondrial membrane in the absence of a death signal correlates with repression of the transmembrane signal-anchor function of BAX by the NH2-terminal domain. Targeting can be instated by deleting the domain or by replacing the BAX transmembrane segment with that of BCL-2. In stimulated cells, the contribution of the NH2 terminus of BAX correlates with further exposure of this domain after membrane insertion of the protein. The peptidyl caspase inhibitor zVAD-fmk partly blocks the stimulated mitochondrial membrane insertion of BAX in vivo, which is consistent with the ability of apoptotic cell extracts to support mitochondrial targeting of BAX in vitro, dependent on activation of caspase(s). Taken together, our results suggest that regulated targeting of BAX to mitochondria in response to a death signal is mediated by discrete domains within the BAX polypeptide. The contribution of one or more caspases may reflect an initiation and/or amplification of this regulated targeting.     

Delayed internal capsule infarctions following radiofrequency pallidotomy. Report of three cases

The authors report on a series of patients with idiopathic Parkinson's disease (IPD) who underwent stereotactic radiofrequency (RF) pallidotomies, three of whom suffered delayed postoperative strokes. These three belonged to a group consisting of 42 patients with medically intractable IPD in whom 50 pallidotomies were performed. All three patients had significant previous vascular disease and were in a high-risk group for cerebral infarction. A postoperative magnetic resonance (MR) image was obtained immediately after the pallidotomy was performed to document the placement of the RF lesion and to rule out any hematoma. The delayed strokes occurred on postoperative Days 10, 51, and 117 in patients with previous vascular disease (Group 1, 11 patients). No strokes occurred in the group with the vascular disease risk factor (Group 2, 11 patients) or in the group with no risk factors for vascular disease (Group 3, 20 patients). This observation is statistically significant (p < 0.05). The T2-weighted MR images showed the lesions as high-intensity signals extending to the posterior limb of the internal capsule ipsilateral to the pallidotomy site. The poststroke T1-weighted images obtained in two patients showed persistent contrast enhancement of the RF lesion and no enhancement around the stroke lesion. Clinically and radiographically, these discrete new lesions represent delayed infarctions, suggesting that RF lesioning can induce delayed injury in adjacent tissue. Patients with previously identified vasculopathy may be at risk for delayed capsular infarction following RF pallidotomy.     

Amyloid goiter. Report of two cases

Congenital pedicle abnormalities are rare. Unilateral aplastic and hypoplastic lumbar pedicles have been reported, but these were usually discovered incidentally and did not need surgical treatment. We present a case of absence of both pedicles and the neural arch of L2, with associated kyphoscoliosis with neurological involvement, that needed a two-stage corrective surgery. An L1-L4 fusion was achieved with relief of the symptoms.     

Post-traumatic flank hernias. Report of two cases

Dissecting aneurysms of the hepatic artery are extremely rare. We report herein the case of a 51-year-old man with massive hepatic metastases from rectal carcinoma, who was found incidentally to have a left hepatic artery aneurysm by a preoperative angiography. After replacing the left hepatic artery with a 2-cm segment of long saphenous vein, an extended right lobectomy with partial resection of the left lateral segment for multiple metastases was successfully performed. To prevent thrombus formation at the anastomosis, the Pringle maneuver was not used during dissection of the hepatic parenchyma. Histological examination of the resected specimen showed a dissecting aneurysm of the left hepatic artery, but there were no findings to suggest the etiology of this disease. The patient is currently alive 13 months after his operation without any evidence of further recurrence of the carcinoma. To the best our knowledge, 12 case reports of this anomaly have been documented, but only one of these describes a successful operation. The clinical features and etiology of the hepatic artery aneurysm that develops independent of other vascular diseases are discussed following this case report.     

Spontaneous drainage of syringomyelia. Report of two cases

Two cases are reported of Arnold-Chiari type I malformation associated with syringomyelia, in which magnetic resonance (MR) imaging revealed spontaneous decompression of the syrinx. In one case axial MR imaging sections showed a communication between the syrinx and the spinal subarachnoid space, which supports the hypothesis that fissuring of the cord parenchyma is instrumental in the spontaneous resolution of syringomyelia. The MR imaging changes were not accompanied by variations in the patients' clinical course.     

Orbital involvement by plasmacytoma. Report of two cases

Two patients with orbital involvement of plasma cell myeloma are presented. The first patient presented an isolated plasmacytoma in the orbit; the second patient had generalized plasma cell myeloma. In both cases X-rays and computed tomographic scanning gave valuable information and biopsy confirmed the diagnosis.     

Giant-cell arteritis of the uterus. Report of two cases

Two cases of giant-cell arteritis of uterus were found incidentally in association with uterine prolapse. One similar case has been reported in the literature. Microscopic examination disclosed fragmentation of the elastic laminae of the myometrial arteries with a giant-cell and mononuclear infiltrate. The relationship between giant-cell arteritis of the uterus, temporal arteritis, and polymyalgia rheumatica is discussed in view of similar cases reported in the literature.     

Inflammatory pseudotumor of the liver. Report of two cases

Hypersensitivity reactions from trimethoprim/sulfamethoxazole are likely caused by a reactive nitroso intermediate formed from sulfamethoxazole hydroxylamine. This pilot study tested whether cimetidine inhibits the urinary excretion of sulfamethoxazole hydroxylamine. Ten outpatients infected with human immunodeficiency virus (HIV) and currently receiving trimethoprim/sulfamethoxazole prophylaxis were randomly selected from 59 eligible patients. Five received cimetidine 800 mg twice daily for 1 week and five served as controls. Two spot urine samples one week apart were obtained after a trimethoprim/sulfamethoxazole dose for all patients. Patients taking cimetidine had a significant decrease in excretion of sulfamethoxazole hydroxylamine relative to total excreted drug in the two urine samples compared with control patients. Cimetidine likely caused this decrease in sulfamethoxazole hydroxylamine excretion through inhibition of CYP3A4. Because of potential differences between HIV-infected patients and healthy subjects in oxidative metabolism, future studies of inhibitors of sulfamethoxazole hydroxylamine formation should be conducted in the HIV population.     

Osteopetrosis and total hip arthroplasty. Report of two cases

In a 47-year-old male cadaver the splenic artery traveled partially through the substance of the pancreas. Additionally, the pancreas was apparently large and had an unusual shape; the neck of the organ could not be differentiated; the uncinate processus was absent; two pancreatic ducts were present. The probable reason for this combined anomaly was abnormal fusion of the ventral and dorsal pancreatic buds due to different levels of origin.     

Metastatic carcinoma to the mandible. Report of two cases

Two cases of rare metastases from malignant neoplasms to the posterior mandible in young adults are reported. Multiple imaging modalities were necessary to identify the many metastases and, in one case, the suspected primary site. Many of the radiographic images are presented for that case.     

Late postirradiation occlusive vasculopathy in childhood medulloblastoma. Report of two cases

The kinetics of the interaction of Rab7 with REP-1 have been investigated using the fluorescence of GDP and GTP analogs at the active site of Rab7. The results show that REP-1 has higher affinity for the GDP bound form of Rab7 (Kd=1 nM) than for the GTP bound form (Kd=20 nM). Both affinities should still be sufficient for the formation of stable complexes in the cell. The association reaction proceeds in two steps for the GDP bound form. The initial step is fast (k+1 = ca. 10[7] M[-1] s[-1]) and concentration dependent while the second represents a slow equilibration (k+2 + k-2 = 3.5 s[-1]) which has little effect on the overall equilibrium. The difference in affinity of the two nucleotide bound forms arises from a difference in dissociation rates (0.012 s[-1] for Rab7 x GDP and 0.2 s[-1] for Rab7 x GTP).     

Familial erythrophagocytic lymphohistocytosis. Report of two cases and clinicopathologic review

This paper deals with the kinetic inhibition of six cephalosporinases (cephalosporin amido-beta-lactamhydrolase, EC 3.5.2.8) by carbenicillin. In previous cases, the inhibition has appeared usually to be competitive and slowly reversible. This makes it possible to measure the two terms of the ratio Ki=k5/k4;k4 and k5 being respectively the velocity constants of formation and destruction of the enzyme-inhibitor complex. A program was prepared which made it possible to obtain these constants from only one experiment. With ampicillin and cloxacillin, we verified that the reaction is faster, and that only Ki can be measured. These facts suggest that special precautions should be taken in order to obtain signigicant values for the constants governing inhibition.     

Persistent hypoglossal artery associated with aneurysms. Report of two cases

The approach to management of idiopathic thrombocytopenic purpura (ITP) during pregnancy remains controversial--particularly regarding the method of delivery. Regardless of the maternal platelet count, we believe that these patients should be delivered by elective cesarean section at term because of the continued presence of maternal antiplatelet IgG antibodies, which cross the placenta. Despite the method of treatment used to increase maternal platelets, immunologic factors are significnat for neonatal involvement and should be considered in the management of these patients. The single largest factor responsible for reported neonatal losses has been intracranial hemorrhage secondary to thrombocytopenia and birth injury. Two patients with ITP are presented with data to substantiate our position on the method of delivery. The infants were transiently thrombocytopenic following delivery (despite adequate maternal levels of platelets).     

Hypothyroidism presenting as a polymyositis-like syndrome. Report of two cases

Two patients with proximal muscle weakness and marked elevations of serum muscle enzymes were initially believed to have polymyositis; however electromyography and muscle biopsies were normal. Both patients were subsequently found to have hypothyroidism. Each regained her muscle strength, and serum enzymes normalized with thyroid hormone replacement. Because muscle weakness and an elevated creatine phosphokinase occur in most patients with hypothyroidism, thyroid dysfunction must be considered in the differential diagnosis of polymyositis.