Cystic lesion of the ventriculus terminalis in an adult.

We present a 40-year-old man with conus medullaris syndrome secondary to a cystic lesion of the ventriculus terminalis (CLVT) and review the relevant literature. The patient presented with 4 years of worsening right leg weakness, and examination showed bilateral fasciculations and hyporeflexia. MRI showed a cystic lesion at T11-12. He was managed with a T11-12 laminectomy and fenestration of an intramedullary cyst. A total of 32 patients, including ours, have been described since 1968: 24 were female with a mean age of 46.6 years. All patients presented symptomatically: five were managed conservatively, four using percutaneous aspiration under MRI guidance, and 22 with open surgery. We conclude that symptomatic patients are best managed surgically, although percutaneous aspiration is an emerging technique.

Sporadic encephalitis lethargica.

Three women (aged 21-36 years) developed acute illnesses that were similar to epidemic encephalitis lethargica. Each presented with a neuropsychiatric disturbance that was succeeded by pyrexia, a fluctuating conscious state and involuntary movements including oculogyria. Cerebrospinal fluid examination showed a predominantly lymphocytic pleocytosis (64-120x10(6) cells/L) and oligoclonal bands were detected in two cases. Two patients died, while the third made a gradual recovery. Post-mortem examination in the two fatal cases showed changes of lymphocytic meningitis and focal diencephalic lymphocytic infiltration, although these changes were mild relative to the effects of the clinical illness. The diagnosis of sporadic encephalitis lethargica relies on identifying shared clinical features with the past epidemic disease plus circumstantial evidence of immunological activity from laboratory investigations and some tests of exclusion of other disorders.

Pathology of cyclodiode laser: a series of nine enucleated eyes.

AIM: To study the histological effects of cyclodiode laser treatment in humans, and to compare these findings with the clinical course, treatment response, complications, and indications for enucleation. METHOD: Detailed histological examination of nine enucleation specimens was undertaken in conjunction with a retrospective review of patient case notes. RESULTS: Retreatments had been undertaken in three cases. Although all globes showed damage to pars plicata, intact ciliary processes within the treatment zone were present in all cases. Pars plana injury was also noted in two thirds of cases. Inflammation was mild. Ciliary epithelial proliferation was seen in most cases with increasing time following treatment, in a disorganised pattern, without replication of the ciliary epithelial bilayer. No regeneration of the ciliary processes with fibrovascular cores was found. The three patients with good IOP control at enucleation had all had multiple diode treatments. Neither phthisis nor sympathetic ophthalmia was seen. CONCLUSIONS: Diode laser cyclophotocoagulation produces very characteristic injury to pars plicata, which frequently extends into pars plana, but with only mild persisting inflammation. Ciliary processes are, however, frequently spared within the treatment zone and may account for early or late treatment failure.

Presymptomatic motor neuron loss and reactive astrocytosis in the SOD1 mouse model of amyotrophic lateral sclerosis.

In familial amyotrophic lateral sclerosis (fALS), there is a need to establish more precisely the progression of the disease, particularly whether there is gradual presymptomatic neuronal loss or an abrupt loss coinciding with the symptomatic stage. To elucidate this, we investigated the progression of motor neuron loss through morphological techniques, reactive astrocytosis, and expression of ubiquitin and neurofilament proteins, by immunohistochemistry, in SOD1 G93A mice with a protracted disease course and control mice. Loss of motor neurons in SOD1 G93A mice followed a biphasic progression, with an initial loss at 126 days of age, followed by a gradual loss from onset of symptoms through to end-stage disease. Reactive astrocytosis was first observed at 70 days of age and showed a gradual increase through to end-stage disease. This suggests that there is a need for early detection of fALS cases, and potential therapeutic treatments may be more beneficial if administered at an early stage.

Scedosporium endophthalmitis: two fatal disseminated cases of Scedosporium infection presenting with endophthalmitis.

The incidence of disseminated infection with Scedosporium species is increasing in patients with haematological malignancy. Two fatal cases are reported of patients with acute myeloid leukaemia and neutropenia who presented with Scedosporium endophthalmitis. Diagnosis of fungal infection was delayed as blood and vitreous cultures were positive only after 3 days in patient 1 and blood culture was positive at 7 days in patient 2. Despite antifungal therapy with amphotericin B and additional fluconazole in patient 2, both patients died of overwhelming fungal septicaemia. Post-mortem examination of the right globe in patient 1 showed haemorrhagic necrotizing chorioretinitis with numerous fungal hyphae in choroidal vessels, choroid, retina and vitreous. Scedosporium species are often resistant to conventional antifungal therapy including amphotericin B. Diagnosis is difficult and mortality in disseminated infection is high.

Impression cytology following mitomycin C therapy for ocular surface squamous neoplasia.

BACKGROUND/AIMS: Topical mitomycin C (MMC) therapy has been used for treatment of ocular surface squamous neoplasia (OSSN) since 1994. Relatively few studies have reported the cellular changes in ocular surface following MMC. METHODS: Impression cytology was studied in four patients with ocular surface squamous neoplasia, either primary or recurrence after previous excisional biopsy. The authors studied samples obtained using Millipore filters at intervals between 4 and 17 weeks after commencement of MMC, and compared them with pretreatment cytology. RESULTS: MMC induced changes of cytomegaly, cytoplasmic vacuolation, nucleomegaly with nuclear wrinkling, and binucleation or multinucleation were seen in some cells in all samples. However, nuclear/cytoplasmic (N/C) ratio in these enlarged cells was normal. These changes mimicked those seen following radiation therapy in uterine cervix. Changes of increased nuclear and cell size with increased N/C ratio were seen in some dysplastic cells. The predominant form of cell death was apoptosis with fewer cells showing necrosis. CONCLUSIONS: MMC appears to produce cell death in OSSN by apoptosis and necrosis. Cellular changes related to MMC mimic those caused by radiation-cytomegaly, nucleomegaly, and vacuolation. MMC related changes may persist in ocular surface epithelium for at least 8 months following MMC therapy.

Reliability of impression cytology for the diagnosis of ocular surface squamous neoplasia employing the Biopore membrane.

AIM: To evaluate the accuracy of impression cytology employing a Biopore membrane device in the diagnosis of ocular surface squamous neoplasia (OSSN). METHODS: The histology of patients undergoing excision biopsy for the suspected diagnosis of OSSN over a 20 month period was compared with the reported cytology of impression cytology specimens obtained preoperatively using the Biopore membrane device. RESULTS: 25 excision biopsies were performed for suspected OSSN. There was accurate correlation in 20 out of the 25 cases (80%). In three cases (12%), there was poor correlation in that only a few dysplastic cells and hyperkeratosis were noted on cytology, whereas histology showed keratinising dysplasia in two cases and a moderately differentiated keratinising squamous cell carcinoma in the third. There were two cases (8%) of non-correlation--one keratinising dysplasia on histology, but anucleate keratin, squamous metaplasia, and rare atypical cells on cytology, and another case with very mild focal dysplasia on histology but squamous metaplasia on cytology. Review of the cytology showed that dysplastic cells were either rare or absent from the cytology specimens in these two cases; it was felt that these represented sampling problems rather than true false negatives. There were no false positives on cytology. Final diagnosis on histology was squamous cell carcinoma in one; carcinoma in situ in two; keratinising dysplasia in 15, non-keratinising dysplasia in seven. CONCLUSION: Impression cytology employing the Biopore membrane accurately predicts the histological diagnosis of OSSN. The presence of hyperkeratosis, inflammatory cells with only a few dysplastic cells, may indicate high grade keratinising dysplasia or squamous cell carcinoma. The method is both rapid and easy to perform in routine clinical practice. These findings suggest that there may be a role for its use in the initial assessment and follow up of patients with suspected OSSN.

Ocular adnexal lymphoproliferative disease: a series of 73 cases.

BACKGROUND: This study involved 73 patients with lymphoproliferative lesions of the ocular adnexa. The lesions were categorized using the Revised European American Lymphoma classification of lymphoid tissues and analysed to determine the frequency and prognostic impact of tumour type, location, stage and patient's age and sex. METHODS: The clinical, histopathological, immunohistochemical and phenotypic analysis by flow cytometry and follow-up data were studied. RESULTS: The ocular adnexal lymphoproliferative lesions included 70 lymphomas and six reactive lymphoid hyperplasia. Seventy-nine per cent had stage IE disease, 4% stage II, 1.5% stage III and 15.5% stage IV. Five patients (7%) had a past history of systemic lymphoma. Major histological types were extranodal marginal zone lymphoma (MZL) in 44 (63%), follicular (FL) in 12 (17%), diffuse large B-cell (DLBCL) in eight (11%), mantle cell (MCL) in two (3%), B-cell chronic lymphocytic leukaemia (CLL)/small lymphocytic lymphoma in two (3%), peripheral T-cell lymphoma (PTCL) one (1.5%) and natural killer cell lymphoma (NKCL) in one (1.5%). Longest survival was seen in those with low-grade lymphomas (MZL and FL) and worst in PTCL and NKCL. Lymphoma-related mortality was 2% for MZL, 33% for FL, 38% for DLBCL, and 100% for MCL, PTCL and NKCL. Systemic lymphoma was present prior to, at presentation or at subsequent follow up in 26/68 (39%) of all lymphoma patients, 17% for MZL, 38% for DLBCL, 83% for FL, and 100% for MCL, CLL, PTCL and NKCL. CONCLUSION: The majority of ocular adnexal lymphomas were low-grade B-cell lymphomas (MZL). Multivariate analysis showed that the only significant independent predictors of all causes of mortality were the histological type of lymphoma and the stage of disease at presentation.

Multifocal chorioretinal atrophy associated with herpes zoster ophthalmicus.

A 73-year-old woman developed multiple depigmented lesions in the fundus 4-6 months after an episode of acute Herpes zoster ophthalmicus. Post-mortem examination of the globe 15 years after this acute episode confirmed multiple old chorioretinal scars probably due to vasculitis of the short posterior ciliary arteries and branches. Patchy old infarcts were also noted in the iris.

Ocular-central nervous system lymphoma mimicking posterior scleritis with exudative retinal detachment.

OBJECTIVE: We describe an unusual ocular presentation of ocular-central nervous system lymphoma in a young patient. DESIGN: Interventional case report and literature review. METHODS: A previously well 24-year-old white woman presented with left eye pain and reduced vision. Episcleral injection, globe tenderness, an afferent pupil defect, and exudative retinal detachment were present. Computed tomographic scan of the head and orbits demonstrated scleral thickening, retinal detachment, and no other abnormality. A provisional diagnosis of posterior scleritis with exudative retinal detachment was made. Investigation for underlying connective tissue diseases was negative. There was an initial prompt response to corticosteroid therapy. The patient's symptoms and signs then recurred, and a left third cranial nerve palsy developed. Systemic investigations including lumbar puncture ultimately led to the diagnosis of primary T-cell central nervous system (CNS) lymphoma. Serologic tests for human immunodeficiency virus were negative. MAIN OUTCOME MEASURES AND RESULTS: The patient underwent orbital and cranial irradiation and intrathecal and systemic chemotherapy. Despite an initial response to treatment, she returned with a recurrence of the lymphoma in the anterior segment of the left eye. Her systemic disease progressed rapidly, and she died shortly thereafter. CONCLUSIONS: This patient's young age and initial presentation mimicking posterior scleritis with unilateral exudative retinal detachment, without evidence of vitreous involvement, are highly unusual for ocular involvement in primary CNS lymphoma. A review of the literature highlights the atypical nature of this presentation.

Diode laser cyclophotocoagulation: histopathology in two cases of clinical failure.

Diode laser photocoagulation is an alternative technique to Nd:YAG laser or cryotherapy in cycloablation. It may be more titratable with fewer local side effects. The effect is not, however, always maintained in the long term. The authors wished to establish histologic correlates with in vivo clinical outcomes of failure. Histologic findings in two cases of clinical failure of a single treatment with contact diode laser cyclophotocoagulation are presented. A recurrence of uncontrolled intraocular pressure (IOP) finally led to enucleation of the eyes 5 and 6 months after laser treatment. Histologic examination demonstrated preservation in both specimens of some ciliary processes outside the treatment zone, which was evidently over the pars plana in a case with a relatively low axial length. Persisting damage of treated ciliary processes was seen. Some efforts at ciliary epithelial regeneration were made in these, but in a disorganized and presumably nonfunctional fashion, so that a histologic explanation for the gradual postoperative rise in IOP was not evident. Diode laser cyclophotocoagulation may lower IOP by mechanisms other than destruction of ciliary epithelium. Reversal of a laser-induced increase in uveoscleral outflow may explain the gradual rise in IOP seen postoperatively in these two cases, given the ineffectual secretory epithelial regeneration in one case and the treatment over the pars plana in the other. Methods to enhance probe placement, such as ultrasound biomicroscopy, transillumination, or axial length measurement, may be useful to ensure that treatment is directed to the pars plicata. [Ophthalmic Surg Lasers 1998;29:852-856.] Cyclodestructive procedures have been likened to "duck hunting in the dark (without radar) since the 'shooter' sees neither the target nor the effect of the blast!" Although the use of Nd:YAG and semiconductor diode laser cyclophotocoagulation instead of cyclocryotherapy has progressively refined the caliber of the weapon and its titratability, aim in the placement of the destruction remains inexact. The assumption has generally been, however, that treatment must be directed to ablation of the ciliary epithelium to achieve a hypotensive effect. We present the histopathology from two cases of contact diode laser cyclophotocoagulation (DLCPC), both of which resulted in initially successful intraocular pressure (IOP) control, but which finally required enucleation for problems supervening on a recurrently raised IOP.

Pyogenic granuloma of the cornea.

BACKGROUND: Ocular pyogenic granulomata are uncommon and are often associated with a chalazion or previous ocular and adnexal surgery The avascular nature of the cornea may explain the rarity of pyogenic granulomata at this site. We report on a case of corneal pyogenic granuloma following previous corneal surgery. METHODS: Ophthalmologists who had treated the patient previously were contacted and earlier clinical notes and histopathological examinations were reviewed. The clinical course following excision of the pyogenic granuloma is described, as are the histopathological findings. RESULTS: Histological examination confirmed the diagnosis of pyogenic granuloma. Excision was followed by a clinical recurrence at a different site with spontaneous resolution. CONCLUSIONS: Pyogenic granulomata of the cornea are rare. However, the present case illustrates the importance of considering benign inflammatory causes in the differential diagnosis of a corneal mass lesion so as to avoid unnecessarily aggressive intervention. To our knowledge, spontaneous resolution of a corneal pyogenic granuloma has not been previously described.

Bilateral trigeminal amyloidoma: an unusual case of trigeminal neuropathy with a review of the literature. Case report.

Isolated amyloidomas may, albeit rarely, involve the central nervous system. There are three previous reports of amyloidomas that involved the gasserian ganglion and caused unilateral trigeminal neuropathies. The authors report the case of a 49-year-old woman with apparently isolated amyloidomas that caused slowly progressive bilateral trigeminal neuropathies. Magnetic resonance imaging of the brain revealed mild swelling of the left trigeminal nerve within the cavernous sinus and uniform enhancement with gadolinium throughout the length of the nerve. At craniotomy, the trigeminal nerve and ganglion were observed to be infiltrated by a tumor-like mass. Biopsy showed extensive infiltration of the nerve and ganglion by amyloid. Immunocytochemical studies of the amyloid were negative for immunoglobulins, kappa and lambda light chains, beta-amyloid A4 protein, transthyretin, beta 2-microglobulin, cystatin C, and gelsolin, but weak focal immunoreactivity with antiamyloid AA antibody was seen in the amyloid in vessels and in some intraneural deposits. Extensive investigations failed to reveal evidence of either systemic amyloidoses or an underlying inflammatory disorder or malignancy.

Two cases of idiopathic acute gastric necrosis.

Two cases of acute hemorrhagic gastric necrosis are presented. The first patient was a 62 yr old man with a past history of chronic gastritis, Parkinson's disease and dementia. The second case was a 25 yr old woman with recent abuse of oral analgesic agents. Both presented with an acute abdomen and peritonitis, and underwent urgent gastrectomy for gastric necrosis with perforation. The first patient died, whereas the second recovered. Histology of the gastrectomy specimens showed severe hemorrhagic transmural gastric necrosis with minimal inflammatory changes. Only occasional Gram positive rods were seen in case 1, and microbiological cultures were negative. The etiology of the gastric necrosis in these 2 cases is unclear.

A fatal case of emphysematous gastritis and esophagitis.

Emphysematous gastritis is a rare form of infectious gastritis characterized by intramural gas production by gas forming organisms. We report a fatal case of this disorder with involvement of both stomach and esophagus in a 76 yr old man who had a past history of alcohol abuse, and whose recent therapy included nonsteroidal anti-inflammatory agents.

Autopsy evidence of pulmonary thromboembolism.

OBJECTIVE: To determine the prevalence of pulmonary thromboembolism (PE) and underlying risk factors at autopsy, compared with clinical diagnosis. DESIGN: Retrospective review of autopsy records, death certificates and medical histories. PATIENTS AND SETTING: All 132 patients who underwent autopsy at St Vincent's Hospital, Melbourne, in 1992. RESULTS: Sixteen cases (12% of autopsies) of PE were found at autopsy. In only two had PE been recorded on the death certificate; in one other, diagnosis had been made before death. Associated pulmonary infarction and/or haemorrhage was found in only six patients with PE. All 16 had at least one underlying risk factor: advanced age, cancer, heart disease, or recent pelvic or abdominal surgery. In four patients with missed PE, clinical records showed episodes consistent with PE. There were four false-positive diagnoses. CONCLUSIONS: Significant undiagnosed pulmonary embolism is not uncommon at autopsy. Many episodes are clinically silent, but the diagnosis should be suspected in at-risk patients with unexplained episodes of dyspnoea and tachycardia.

Aseptic meningoencephalitis in primary Sjögren's syndrome. Response to plasmapheresis and absence of CNS vasculitis at autopsy.

It has been suggested that cerebral vasculitis is the pathogenetic mechanism of the central nervous system manifestations of primary Sjögren's syndrome. We present a fatal case of aseptic meningoencephalitis in an 18-year-old woman with primary Sjögren's syndrome in whom there was no evidence of cerebral vasculitis at autopsy. On two occasions her condition improved markedly following plasmapheresis.

An epithelial cyst of the suprasellar region.

A case of a 26 year old woman with increasing headaches due to a suprasellar cyst is described. Histology was consistent with an epithelial cyst with features resembling both Rathke's cleft cysts and enterogenous cysts. The differential diagnosis and treatment of suprasellar cysts is discussed.

Medical certification of causes of death in an Australian metropolitan hospital. Comparison with autopsy findings and a critical review.

OBJECTIVES: To compare the causes of deaths recorded on death certificates with findings at autopsy and to identify other deficiencies in the completion of death certificates. DESIGN AND SETTING: The causes of death (Part I and II) recorded on the death certificates of all patients undergoing autopsy at St Vincent's Hospital, Melbourne, in 1992 were compared with the autopsy findings. Additional clinical information about previous medical and surgical history and antemortem investigations was obtained from the clinical summary in the autopsy reports, and from hospital charts in some cases. PATIENTS: In 1992, 132 hospital autopsies were performed (autopsy rate of 24.2%). Of these patients, 68% were aged 65 years or over, and 30% were aged 75 years or over. RESULTS: Major discrepancies between the cause of death listed on the certificates and autopsy findings were found in 16 cases (12%). Other deficiencies of death certification included listing the mode of death (e.g., cardiac failure) without an underlying cause in 14 cases (11%); failure to cite recent major surgery in 17 of 20 cases (85%); failure to specify site or organism in 33 of 40 cases (82.5%) of infection or sepsis. CONCLUSIONS: This study confirmed findings of previous studies with respect to missed major diagnoses, but identified other deficiencies in certification of causes of death, which could compromise accuracy of statistics obtained from death certificates. Mechanisms by which these deficiencies can be prevented or corrected are discussed.