Calcium pyrophosphate dihydrate deposition disease of the filum terminale.

INTRODUCTION: Calcium pyrophosphate dihydrate crystal deposition disease (CPPDD) is a rare benign inflammatory joint disorder characterized by the presence of calcium pyrophosphate dihydrate crystal in the interarticular and periarticular tissue. It has been rarely described with spinal localization. METHODS: A 50-year-old woman, affected by CPPDD, presented a progressive weakness of both lower limbs associated with neurogenic claudication. Neuroradiological examinations revealed the presence of two intradural calcified lesions at level L3-L4, with no post-contrast enhancement. RESULTS: Surgery was performed and the histopathological exams documented the presence of rod-shaped crystals embedded in a fibrocartilaginous stroma. The postoperative course was uneventful and the patient experienced complete symptoms relief with a 5-year follow-up. CONCLUSION: Intradural CPPD localization at the filum terminale is an extremely rare occurrence. Total removal should be preferably attempted with a long-term focal control of the disease as we observed in our case.

Discovery of a transient absorption edge in the X-ray spectrum of GRB 990705.

We report the discovery of a transient equivalent hydrogen column density with an absorption edge at approximately 3.8 kiloelectron volts in the spectrum of the prompt x-ray emission of gamma-ray burst (GRB) 990705. This feature can be satisfactorily modeled with a photoelectric absorption by a medium located at a redshift of approximately 0.86 and with an iron abundance of approximately 75 times the solar one. The transient behavior is attributed to the strong ionization produced in the circumburst medium by the GRB photons. The high iron abundance points to the existence of a burst environment enriched by a supernova along the line of sight. The supernova explosion is estimated to have occurred about 10 years before the burst. Our results agree with models in which GRBs originate from the collapse of very massive stars and are preceded by a supernova event.

Effects of iron overload on bile secretion and hepatic porphyrin metabolism in ethinyl estradiol-treated rats.

We investigated the effects of ethinyl estradiol (5 mg/kg body wt daily for 5 days, orally) and/or iron sorbitol (50 mg/kg body wt daily for 5 days, i.m.) on bile flow, bile salt independent fraction (BSIF), hepatic delta-aminolevulinate synthase (ALA-S) and uroporphyrinogen decarboxylase (URO-D) in female rats. Ethinyl estradiol administration was associated with a significant decrease of bile flow and BSIF and an increase in URO-D activity in comparison to control values. Iron alone did not modify biliary parameters, but significantly increased the activity of ALA-S. Combined treatment with ethinyl estradiol plus iron partially corrected the reduction of BSIF and restored the activity of ALA-S and URO-D to control levels. Thus iron appears to exert a partially protective effect against ethinyl estradiol-induced cholestasis. No porphyrinogenic effect was observed.

Bile secretion and liver microsomal mixed function oxidase system in mice with griseofulvin-induced hepatic protoporphyria.

Administration of 2.5% griseofulvin in the diet to male CD1 mice produced protoporphyria and cholestasis. Protoporphyria became evident as early as after 10 days of treatment, whereas cholestasis, expressed in terms of total bile flow reduction, developed only after 45 days of griseofulvin. Bile flow impairment was due both to the length of treatment and to the severity of liver protoporphyria. Griseofulvin administration was also associated with a significant modification of the relative amounts of hepatic microsomal cytochromes P-450 and b5, a loss in concentration/mg of protein of cytochrome P-450 and a concomitant increase of b5. Despite these changes, the activity of aniline hydroxylase expressed per mg of microsomal protein, assessed in vitro, was not modified.

Different susceptibility of mouse tissues to porphyrogenic effect of 2,3,7,8-tetrachlorodibenzo-p-dioxin.

The porphyrogenic effect of chronic administration of 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) (25 micrograms/kg/week) to male C57BL/6 mice was evaluated through quantitative and qualitative analysis of the porphyrins accumulated and of porphyrinogen carboxylase activity in liver, kidney, spleen, brain and erythrocytes. The liver was the principal site of action, both for porphyrin accumulation and for enzyme inhibition, with kidney next, whereas brain and erythrocytes were unaffected. In the spleen, despite unchanged formation of total products of uroporphyrinogen III decarboxylation, both an increase and a decrease of coproporphyrinogen formation were observed, the decrease being concomitant with a higher accumulation of tissue porphyrins. When a response to TCDD was found, the formation of the products of decarboxylaction of uroporphyrinogen III were affected to different extents. The pattern of enzyme inhibition paralleled data reported in the literature regarding tissue distribution of TCDD and indicated that TCDD porphyria is a suitable experimental model for the human 'sporadic' type of porphyria cutanea tarda (PCT).

In vitro inhibitory effect on porphyrinogen carboxylyase of liver extracts from TCDD treated mice.

Marked inhibition of porphyrinogen carboxylyase was produced in vitro by cytosol fractions, deproteinized and free of porphyrins, obtained from livers of mice made porphyric by 9 weeks i.p. treatment with 25 micrograms/kg/week of 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD). Inhibition was proportional to the amount of the fraction added, was increased by preincubation in the absence of the substrate and, once established, could not be reversed by dialysis. TCDD itself, added to the control enzyme in the incubation mixture, did not affect enzyme activity up to a concentration of 77 nM, which is 10 times higher than the liver TCDD concentration found after in vivo TCDD treatment.

Diaphragma sellae meningiomas. Report of two cases and a review of the literature.

Diaphragma sellae meningiomas are very rare and often manifest themselves with aspecific symptoms. Before the advent of MR, the diagnosis was very difficult or even impossible. For this reason they were often included in the broader category of suprasellar meningiomas. We describe two cases of diaphragma sellae meningiomas anterior to the pituitary stalk, manifesting with visual disturbances. We present the clinic and diagnostic data and we compare our experience with that reported in the literature. The tumor were removed by pterional (case 1) and subfrontal approach (case 2) with a recover of vision. Our experience support a clear demarcation of the diaphragma sellae meningioma among the wither group of suprasellar meningiomas. It is mandatory to define its position relative to the pituitary stalk and to the diaphragma sellae to chose the best surgical approach.

Automated percutaneous discectomy in herniated lumbar discs treatment: experience after the first 200 cases.

Two hundred patients presenting lumbo-sacral radicular pain were treated with automated percutaneous discectomy and were divided into two groups, on the ground of their symptomatology: group A included those patients that, otherwise, would undergo conservative therapy, because of their moderate pain; group B gathered patients whose severe pain needed undelayed surgery. The success rate that we reported in group A was 85%, while, in group B, it was 64%. Recurrences needing open surgery occurred in 15% of group B. Although characterized by delayed recovery, this technique seems to have good results even in so called surgical patients, in comparison with open surgery.

Epidermoid cysts of the fourth ventricle.

Epidermoid tumors located in the fourth ventricle are exceedingly rare. Seven cases of this pathological condition were observed during a 10-year period. Patients were mostly middle-aged men, with a clinical history of relatively short duration (5 months). Clinical symptoms consisted of vertigo and ataxia, followed by incoordination, dysmetria, and tremor at a later stage. Computed tomography scanning represented the main diagnostic technique for these lesions, and typically showed a highly hypodense, round-shaped area within the fourth ventricle, occasionally accompanied by hydrocephaly. Subtotal surgical removal of the cysts produced excellent results in 86% of the cases. The implications of these findings are discussed.

Treatment of the failed back surgery syndrome due to lumbo-sacral epidural fibrosis.

The failed back surgery syndrome (FBSS) is a severe, long-lasting, disabling and relatively frequent (5-10%) complication of lumbosacral spine surgery. Wrong level of surgery, inadequate surgical techniques, vertebral instability, recurrent disc herniation, and lumbo-sacral fibrosis are the most frequent causes of FBSS. The results after repeated surgery on recurrent disc herniations are comparable to those after the first intervention, whereas repeated surgery for fibrosis gives only 30-35% success rate, and 15-20% of the patients report worsening of the symptoms. Computerized tomography (CT) with contrast medium and, in particular, Gd-DPTA enhanced MRI have recently allowed a differentiation between these two pathologies permitting us to adopt different therapies. In 1982-92 we applied spinal cord stimulation (SCS) as a first therapy of FBSS with proven lumbo-sacral fibrosis. Fifty-five patients underwent percutaneous trial SCS with a mono/multipolar electrode placed at the level of Th9-12. In the 36 patients who had a positive response to the trial stimulation, the electrode was connected to an implantable neurostimulator. On January '94 a third party, not involved in the treatment of the patients, controlled 34 of the 36 patients with a mean follow-up of 55 months. We classified the patients reporting at least 50% pain relief and satisfaction with result as successful, and 56% of the patients fell in that category. 10 out of 34 patients were able to resume their work. The success rate was significantly higher in females (73%) than in males, and in radicular rather than axial pain. Our data have led us to consider SCS as a first choice treatment in FBSS due to lumbo-sacral fibrosis.

Clinical results of epidural spinal cord electrical stimulation in patients affected with limb-threatening chronic arterial obstructive disease.

Between 1982 and 1990, 76 patients (33 women, 43 men, mean age 71.4 +/- 10 years) affected with limb-threatening peripheral vascular disease (claudication < 20 m: 3 patients; rest pain: 10 patients; necrosis1 smaller than 3 cm2: 28 patients; necrosis2 larger than 3 cm2: 35 patients) not amenable to medical and/or surgical therapy, were treated by epidural spinal cord electrical stimulation (ESES). Effectiveness of ESES was evaluated by consideration of pain control, walking distance, and healing of ischemic lesions. At a mean follow-up of twenty-six months (range: one to seventy-six) 44 limbs (58%) were amputated (rest pain 2; necrosis1 13; necrosis2 29) and 39% of necrotic lesions smaller than 3 cm2 healed. The overall limb salvage rate was 42%. Pain control was obtained in 80% of patients at the one-year and 75% at the two-year follow-up, with infrequent use of pain relievers. Despite the poor clinical results observed, the limb salvage rate testifies to the effectiveness of ESES in limb-threatening ischemia. Moreover, the authors noticed a good ESES effect on pain relief, maximal in the early and intermediate postimplant periods. In conclusion ESES must be considered the last resort in peripheral vascular disease in patients in whom medical and/or surgical therapies are ineffective or impossible. Necrotic lesions larger than 3 cm2 contraindicate, in their opinion, ESES implant.

[Epidural spinal stimulation in the treatment of refractory angina pectoris. Its clinical efficacy, complications and long-term mortality. An Italian multicenter retrospective study]. / La stimolazione spinale epidurale nel trattamento dell'angina pectoris refrattaria. Efficacia clinica, complicanze e mortalità a lungo termine. Studio retrospettivo multicentrico Italiano.

BACKGROUND: Spinal cord stimulation has been used for many years in the treatment of refractory angina pectoris. Its anti-anginal and anti-ischemic effect has been well documented in several studies, but the long-term efficacy, safety and survival rate are not well known. The aim of this study was to carry out a retrospective analysis of a series of patients from the Italian Multicenter Registry, the data of which were collected in five centers, by means of a questionnaire. METHODS: One hundred and thirty patients (83 males, 47 females, mean age 74.8 +/- 9.8 years) were submitted to spinal cord stimulator implantation for refractory angina pectoris in the period 1988-1995 and controlled during a mean follow-up of 31.4 +/- 25.9 months. A previous myocardial infarction had already occurred in 69.3% of patients, whereas in 67.6% multivessel coronary artery disease was documented. A left ventricular dysfunction (ejection fraction < 0.40) was present in 34% of patients; bypass surgery and coronary angioplasty were performed in 49.6% and in 27% of patients respectively. In 96.3% of cases revascularization procedures were not advisable. RESULTS: A complete follow-up of 116 patients (89.2%) was available. The spinal cord stimulator induced a significant reduction in NYHA functional class from 2.5 +/- 1.2 to 1.5 +/- 0.9 (p < 0.01). During the follow-up 41 patients (35.3%) died, and in 14.2% a new acute myocardial infarction developed. The total percentage of minor spinal cord stimulation-related complications was 6.8%. No major complications occurred. The annual total mortality rate was 6.5%, whereas the cardiac mortality rate was 5%. Compared to the survivors, patients who died showed a higher incidence of left ventricular dysfunction, previous myocardial infarction and bypass surgery at implantation. CONCLUSIONS: In our experience, spinal cord stimulation is an effective therapy in patients affected by refractory angina pectoris and who cannot undergo revascularization procedure. The complication rate is low, with the total and cardiac mortality showing a trend as that reported for patients with similar coronary disease.

Decompressive craniectomy may cause diagnostic challenges to assess brain death by computed tomography angiography.

According to Italian legislation to diagnose brain death (BD) after the initial documentation of the clinical signs, repetition of clinical testing and confirmation of the loss of bioelectrical activity of the brain (EEG) is required. However, when EEG is unreliable it is necessary to demonstrate cerebral circulatory arrest (CCA). Accepted imaging techniques to demonstrate CCA include: cerebral angiography, cerebral scintigraphy, transcranial Doppler (TCD) and computed tomography angiography (CTA). This latter technique, due to its large availability, low invasivity and easy and fast acquisition is widely used over the country. Nevertheless its diagnostic reliability is affected by some limitations in patients with decompressive craniectomy. Here we report two cases of brain injury with clinical signs of BD and at the same time, opacification of intracranial arteries on CTA and a pattern consistent with flow arrest on the corresponding insonable arteries on TCD. The discrepancy between CTA and TCD results points out a methodology limitation that could be overcome by updating Italian legislation according to other European Countries legislation.

Endovascular treatment of primitive persistent trigeminal artery aneurysm associated with middle cerebral artery aneurysm.

We report the case of a primitive trigeminal artery aneurysm associated with an ipsilateral middle cerebral artery aneurysm. A 64-year-old Caucasian woman suffered from a severe acute headache. A head CT scan displayed subarachnoid hemorrhage and subsequent cerebral angiography showed right, wide-necked persistent trigeminal artery and ipsilateral middle cerebral artery aneurysms. The patient underwent embolization of both aneurysms with Guglielmi detachable coils. The association of a PPTA aneurysm and an ipsilateral MCA aneurysm has not been reported in the English literature.

Spinal cord stimulation in peripheral vascular pain.

Results in 12 patients suffering from pain due to peripheral vascular pathology and treated with spinal neurostimulation are reported. The best results were obtained in stage III of vascular disease; however, our results indicate that regression of the pain symptom and healing of trophic lesions do not always correspond to any variation in blood flow.

[Cerebral abscess as a result of a ventriculo-peritoneal shunt]. / Ascesso cerebrale da shunt ventricolo-peritoneale.

The authors relate a rare case of cerebral abscess, complication of a ventriculo-peritoneal derivation. They discuss its etiopathogenesis and underline the importance of cerebral TAC for both diagnostical and post-operatory checking purposes.

Epidermoid cysts of the fourth ventricle: very long follow up in 9 cases and review of the literature.

This study reports late surgical results obtained in 9 patients operated on for an epidermoid cyst of the fourth ventricle. They represent 19/9% of all cases of epidermoids treated at our Department over a period of 25 years (1975-2000). 7 cases were diagnosed with CT and 2 cases with MR. Duration of clinical history ranged from 2 months to 6 years (mean: 2,2 years). Preoperatively, 2 cases presented spontaneous remission of neurological symptoms and signs lasting about 1 year. 7 (77,8%) patients underwent subtotal removal of the cyst whereas in 2 patients the lesion was totally excised. 3 patients (33,3%) underwent a second operation for symptomatic recurrence diagnosed between 10 and 17 years from the first operation. At present, 7 (77,8%) patients are alive (mean follow up of 14,5 years, ranging from 5 to 23 years) without neuroradiological and clinical evidence of tumour recurrence. In this group, 6 have no neurological problems and 1 suffers from a light vestibulo-cerebellar syndrome. Fourth ventricle epidermoids have a good long-term prognosis even in the case of only subtotal removal. Follow-up clinical and MR controls allow an earlier diagnosis of recurrence. Surgery of recurrent cysts can be more demanding; nonetheless, long term prognosis can be quite good for patients in well preserved neurological condition.