1/14. Creutzfeldt-Jakob disease with florid-type plaques after cadaveric dura mater grafting.BACKGROUND: Many reported cases of iatrogenic Creutzfeldt-Jakob disease (CJD) developed after grafting cadaveric dura mater contaminated with CJD prions (dura-associated CJD). They are known to be clinicopathologically similar to sporadic CJD. We report herein 2 autopsy cases of dura-associated CJD with atypical clinicopathological features. patients: Two patients presented with progressive ataxia and mental deterioration 10 or 11 years after neurosurgical treatment with cadaveric dural grafting, which led to their deaths at 8 and 17 months, respectively, after onset. RESULTS: The cases were clinically atypical in exhibiting no or late occurrence of myoclonus and periodic synchronous discharges on electroencephalographic studies. They were pathologically unique in several aspects. The most striking feature was the presence of many prion protein (PrP) plaques in multiple areas in the brain. Some of them were the "florid" type surrounded by a zone of spongiform changes known to be a hallmark for the new variant CJD. The distribution of spongiform degeneration was also unique in that it was intense in the thalamus, basal ganglia, and the dentate nuclei of the cerebellum but milder in the cerebrum. There were no mutations in the PrP gene of the patients. There was no major difference in the size and glycoform pattern between the abnormal isoform of PrP extracted from the brain tissue from the dura-associated cases of CJD and that from a sporadic case of CJD. CONCLUSIONS: These 2 cases are clinicopathologically distinct from typical dura-associated cases of CJD. They may be a subtype with florid-type plaques in dura-associated CJD.- - - - - - - - - - ranking = 1keywords = variant (Clic here for more details about this article) |
2/14. Unusual origin of the flexor digiti minimi brevis muscle.An unusual origin of the flexor digiti minimi brevis muscle of the right hand was discovered during cadaveric dissection. The muscle originated from the anterior aspect of the transverse fibers of the distal antebrachial fascia and inserted onto the base of the fifth proximal phalanx. When traction was applied to the proximal portion of the muscle, flexion was produced at the fifth metacarpophalangeal joint. Other intrinsic muscles of the hand and the muscles of the flexor forearm compartment had normal morphology. Although muscular anomalies in the hypothenar region have been described, the muscular variant described here was distinct in its origin and size from those previously reported. knowledge of anomalous muscles in the hand has important clinical significance in routine surgery and in determining associated pathology.- - - - - - - - - - ranking = 1keywords = variant (Clic here for more details about this article) |
3/14. Xanthogranulomatous pyelonephritis in nonfunctioning transplanted kidney.Xanthogranulomatous pyelonephritis in native and allografted kidneys is a rare variant of severe chronic infection of the renal parenchyma. In a native kidney the diagnosis may sometimes be established by ultrasonography and computed tomography. In the renal allograft, the diagnosis could only be established by histologic evaluation of the transplant biopsy or nephrectomy. The reported case presents a febrile patient with a failing renal graft, in whom xanthogranulomatous pyelonephritis was established by histologic evaluation of transplantectomy specimens. Xanthogranulomatous pyelonephritis should therefore be included in the list of possible etiologies of the fever in patient with nonfunctioning transplanted kidney.- - - - - - - - - - ranking = 1keywords = variant (Clic here for more details about this article) |
4/14. Renal transplantation for apolipoprotein AII amyloidosis.Apolipoprotein AII (ApoAII) amyloidosis, first reported in 2001 in a family with renal amyloidosis, is associated with mutations in the stop codon of the apolipoprotein AII gene resulting in a carboxyl terminal peptide extension of 21 amino acid residues in the protein. Since death from this form of amyloidosis is due to renal failure, kidney dialysis and renal transplantation are presently the only two therapeutic options. We report the case of a Caucasian man who developed proteinuria in his late 20's, had renal biopsy at the age of 33 which gave the diagnosis of renal amyloidosis, and required continuous ambulatory peritoneal dialysis by age 45. He received a cadaver renal transplant at age 47 and has maintained stable renal function for nine years without other evidence for organ system dysfunction from amyloidosis. Laboratory studies confirmed persistence of the ApoAII variant in the patient's plasma in addition to the normal ApoAII protein. This is in agreement with the dna analysis which showed the patient to be heterozygous for the ApoAII stop78Gly mutation. These results indicate that renal transplantation is an effective therapy for apolipoprotein AII amyloidosis since recurrence of amyloid in the graft and progression of other organ involvement may be very slow.- - - - - - - - - - ranking = 1keywords = variant (Clic here for more details about this article) |
5/14. Triplication of the lesser occipital nerve.Triplication of the lesser occipital nerve (LON) was observed bilaterally in an adult male cadaver during routine prosection of the posterior triangle. The three LONs were studied to determine the clinical importance of this variation. The origin of one LON was from a nerve to the trapezius that had a common origin with the trunk of the supraclavicular nerve (C3,4) from the cervical plexus. Such a common origin of a LON may explain the pain referred to the shoulder and arm that is experienced by some patients with cervicogenic headache. Another LON ran across the roof of the posterior triangle, passed through the trapezius and was closely related to the point of exit of the greater occipital nerve (GON) from the trapezius. This LON supplied the nape of the neck, back of the scalp and the auricle. The anomalous course taken by this LON through the trapezius may be an explanation for cervicogenic headache precipitated by neck movement. The close relationship of this variant LON to the exit of the GON from the trapezius seems to be relevant to the management of cervicogenic headache. The authors suggest that the reason for the complete pain relief experienced by some patients with cervicogenic headache by anesthetic blockade of the GON may be because both the GON and LON are blocked simultaneously due to their proximity in these patients.- - - - - - - - - - ranking = 1keywords = variant (Clic here for more details about this article) |
6/14. A case of abnormal findings in the course of the vertebral artery associated with an ossified hyoid apparatus. A contraindication for manipulation of the cervical spine?OBJECTIVE: To describe a case of a simultaneous occurrence of an ossified stylohyoid ligament in a 56-year-old male cadaver and anomalies of the vertebral artery, and to consider the clinical implications for manipulative therapists. INTERVENTION AND OUTCOME: dissection showed a simultaneous occurrence of complete developmental ossification of the left hyoid apparatus, variants of the vertebral artery, and a left superior vena cava in a 56-year-old male cadaver. DISCUSSION: Developmental variants, posttraumatic and degenerative changes of the hyoid apparatus may result in variable degrees of ossification or calcification. CONCLUSION: This unusual disorder should be considered in the differential diagnosis of facial and neck pain especially within the scope of manipulation of the upper cervical spine. Cervical spine manipulation may exacerbate existing pathological conditions of the stylohyoid apparatus, thereby irritating neurovascular structures, and induce a fracture. Developmental ossification of this apparatus might be associated with anomalies in the atlantic section of the vertebral artery which make the patient more susceptible to vertebrobasilar insufficiency. We conclude that extreme care should be taken in the presence of such an ossification to avoid trauma to the stylohyoid apparatus and maybe even because of increased vertebrobasilar risk.- - - - - - - - - - ranking = 2keywords = variant (Clic here for more details about this article) |
7/14. Ectopic supernumerary tooth on the inferior nasal concha.Variations regarding the location of an ectopic tooth in the human nasal cavity, although rare, are documented in the literature, but presence of an ectopic tooth on the inferior nasal concha (INC) has not been reported. We observed an anomalous tooth projecting from the posterior margin of the right INC in two adult female skulls. A small quadrangular tooth projected beyond the posterior margin of the hard palate in one of the skulls and a medium sized conical tooth was observed in the other skull. The affected INC in both skulls were located more inferiorly compared to the opposite side and were in close approximation with the hard palate. No similar findings were noted on the contralateral side nor were there any associated congenital or iatrogenic deformity. The phylogenetic, ontogenetic, and clinical importance of this variant is described. knowledge of such an anomaly is of paramount importance to otorhinolaryngologists, reconstructive and dental surgeons, and radiologists for identification of such rarities encountered during invasive or non-invasive procedures.- - - - - - - - - - ranking = 1keywords = variant (Clic here for more details about this article) |
8/14. rupture of the anterior tibial tendon: three clinical cases, anatomical study, and literature review.BACKGROUND: We report three cases of anterior tibial tendon ruptures and the results of an anatomical study in regard to the tendon's insertion site and a literature review. methods: Three patients were referred to our hospital with anterior tibial tendon ruptures. In the anatomical study, 53 feet were dissected, looking in particular for variants of the bony insertion of the tendon. RESULTS: Two patients had surgical treatment (one primary repair and one semimembranosus tendon graft) and one conservative treatment. After a mean followup of 14 weeks all patients had satisfactory outcomes. In the anatomical study, we noted three different insertion sites: in 36 feet the tendon inserted into the medial side of the cuneiform and the base of the first metatarsal bone and in 13 feet only into the medial side of the cuneiform bone. In the remaining four feet the tendon inserted into the cuneiform and the first metatarsal bone, but an additional tendon was noted taking its origin from the anterior tibial tendon near its insertion into the medial cuneiform and attaching to the proximal part of the first metatarsal. CONCLUSIONS: According to literature, surgical repair is the treatment of choice for acute ruptures and for patients with high activity levels. For chronic ruptures and patients with low demands, conservative management may lead to an equally good outcome. knowledge of the anatomy in this region may be helpful for diagnosis and for the interpretation of intraoperative findings and choosing the most appropriate surgical procedure.- - - - - - - - - - ranking = 1keywords = variant (Clic here for more details about this article) |
9/14. Metastatic malignant melanoma transplanted via a renal homograft: a case report.A case of transplantation of a malignant melanoma via a cadaver renal homograft is presented. The recipient developed a widespread malignancy ninteen months after renal transplantation. At autopsy the tumor was diagnosed as a malignant melanoma. autopsy of the donor had previously revealed a spindle cell malignancy in the spleen. It is postulated that the splenic tumor in the donor represents a spindle cell variant of malignant melanoma, and hence this malignancy was inadvertently transferred to the recipient via the renal homograft.- - - - - - - - - - ranking = 1keywords = variant (Clic here for more details about this article) |
10/14. An unusual variant of membranous nephropathy with abundant crescent formation and recurrence in the transplanted kidney.A patient with what initially appeared to be a typical membranous nephropathy had a progressive course to renal failure, nephrectomy, and transplantation. The nephrectomy specimen revealed abundant glomerular crescents and capsular synechiae. Post-transplantation the patient again developed a membranous nephropathy with florid crescents. radioimmunoassay and indirect immunofluorescence tests failed to reveal anti-glomerular basement membrane antibody in the serum or kidney. It appears that there is a form of membranous nephropathy with crescent formation, unrelated to anti-GBM antibody, which has the capacity to recur after transplantation.- - - - - - - - - - ranking = 4keywords = variant (Clic here for more details about this article) |
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