Predicting postoperative hypocortisolism in patients with non-aldosterone-producing adrenocortical adenoma: a retrospective single-centre study.

BACKGROUND: Limited information exists on postoperative hypocortisolism and hypothalamus-pituitary-adrenal axis recovery in patients with adrenal incidentaloma following unilateral adrenalectomy. We evaluated frequency of postoperative hypocortisolism and predictors for recovery in non-aldosterone-producing adrenocortical adenoma patients after unilateral adrenalectomy. METHODS: A retrospective analysis of 32 adrenal incidentaloma patients originally included in the ITACA trial (NCT04127552) with confirmed non-aldosterone-producing adrenocortical adenoma undergoing unilateral adrenalectomy from September 2019 to April 2023 was conducted. Preoperative assessments included adrenal MRI, anthropometrics, evaluation of comorbidities, adrenal function assessed via ACTH, urinary free cortisol, and 1 mg dexamethasone suppression test. ACTH and serum cortisol or Short Synacthen test were performed within 6 days, 6 weeks, 6 months, and a year after surgery. RESULTS: Six days postoperative, 18.8% of patients had normal adrenal function. Among those with postoperative hypocortisolism, 53.8% recovered by 6 weeks. Patients with earlier adrenal recovery (6 weeks) had lower preoperative 1 mg dexamethasone suppression test (median 1 mg dexamethasone suppression test 76.2 [61.8-111.0] nmol/L vs 260.0 [113.0-288.5] nmol/L, p < 0.001). Univariate analysis showed preoperative 1 mg dexamethasone suppression test negatively related with baseline ACTH levels (r = - 0.376; p = 0.041) and negatively associated with the 6-week baseline (r = - 0.395, p = 0.034) and 30-min cortisol levels during Short Synacthen test (r = - 0.534, p = 0.023). Logistic regression analysis demonstrated preoperative 1 mg dexamethasone suppression test as the only biochemical predictor for 6-week adrenal recovery: ROC curve identified a 1 mg dexamethasone suppression test threshold of 131 nmol/L predicting 6-week recovery with 89.5% sensitivity and 72.7% specificity (AUC 0.87; 95% CI 66.9-98.7, p < 0.001). Other preoperative assessments (tumor size, ACTH levels and anthropometrics) were not associated with postoperative hypothalamus-pituitary-adrenal axis function, but the presence of diabetes was associated with a lower probability of recovery (OR = 24.55, p = 0.036). ACTH levels increased postoperatively in all patients but did not predict hypothalamus-pituitary-adrenal axis recovery. CONCLUSIONS: The preoperative 1 mg dexamethasone suppression test cortisol value and presence of diabetes are the only relevant predictor of hypothalamus-pituitary-adrenal axis recovery in patients with non-aldosterone- producing adrenocortical adenoma undergoing surgery, regardless other clinical and biochemical variables. Notably, pre- and postoperative ACTH levels did not predict hypothalamus-pituitary-adrenal axis recovery. These findings point towards the potential for saving resources by optimizing their allocation during follow-up assessments for patients with non-aldosterone-producing adrenocortical adenoma undergoing unilateral adrenalectomy.

Spinocerebellar ataxia type 2 (SCA2) with white matter involvement.

Two sisters presented with olivopontocerebellar atrophy, neuronal loss in the substantia nigra, intranuclear ubiquitin-, ataxin-2-positive inclusions in neurons, and severe demyelination and axon loss of the cerebral white matter with no accompanying inflammatory pathology. The genetic study demonstrated a 22/36 CAG triplet expansion in the SCA2 gene in one of the sisters; SCA1, SCA3, SCA6, SCA7, SCA8, SCA12, SCA17 and DRPL were ruled out in this patient. The present report shows that severe cerebral white matter pathology may occur in the context of SCA2.

Peripheral neuropathy in essential mixed cryoglobulinemia.

Sixteen patients with essential mixed cryoglobulinemia were studied and followed up clinically and electrophysiologically for 4.2 years. Peripheral neuropathy was diagnosed in seven cases. Five of these patients had distal symmetrical sensorimotor polyneuropathy. Nerve conduction velocities were normal and therefore indicative of pure axonal neuropathy. Sural nerve biopsy showed moderate loss of myelinated axons in two patients and severe loss in one. This patient also had necrotizing arteritis. The remaining two had both clinical and electrophysiologic signs of overlapping mononeuritis multiplex with severe denervation in the territory of the involved nerves, but normal conduction velocities. Sural nerve biopsy in one of these two patients showed marked loss of myelinated fibers and signs of vasculitis. Two types of neuropathy were noted: (1) a mild distal neuropathy with relatively minor neurologic deficit, probably due to vasa nervorum microcirculation occlusion caused by intravascular deposits of cryoglobulins and (2) a severe distal symmetrical sensorimotor neuropathy or overlapping mononeuritis multiplex, associated with necrotizing vasculitis.

Plasmapheresis and antineoplastic treatment in CNS paraneoplastic syndromes with antineuronal autoantibodies.

We retrospectively evaluated the effect of plasmapheresis (PE) in seven patients with paraneoplastic encephalomyelitis (PEM), small-cell lung carcinoma, and anti-Hu antibodies, and four patients with paraneoplastic cerebellar degeneration (PCD), ovarian or breast cancer, and anti-Yo antibodies. In addition to PE, patients received prednisone (nine), cyclophosphamide (eight), or treatment of the tumor (five). All but one patient were severely disabled by the time PE began. The clinical outcome was compared with that of five patients (PEM, four; PCD, one) who only had treatment of the tumor. Only one of these five patients had a severe neurologic deficit at the onset of the antineoplastic treatment. No patient improved. Two patients treated with PE and antineoplastic therapy and three who only received treatment of the tumor remained stable for at least 6 months. Four of the five patients with a stable course started the treatment when the neurologic deficit was not severe. We conclude that the efficacy of PE with other immunosuppressive therapies in the stabilization of the neurologic deficit is uncertain.

Indolent anti-Hu-associated paraneoplastic sensory neuropathy.

Paraneoplastic sensory neuropathy (PSN) usually runs a subacute progressive course, leaving the patient with severe sensory dysfunction in weeks to months. We describe five patients with PSN, high titers of anti-Hu antibodies (type 1 antineuronal nuclear autoantibodies), and an indolent clinical course. The patients had a median age of 55 years (range, 41 to 72). Four had small-cell (3) or undifferentiated large-cell (1) lung cancer. Patients presented with mild, asymmetric sensory symptoms; in two, the neuropathy was predominant in the arms. Two patients also had a visceral neuropathy causing gastrointestinal dysfunction. The PSN was stable or progressed very slowly without treatment for a median of 18 months (range, 5 to 32) and remained so after treatment with immunoglobulins (1 patient), chemotherapy (3), or both therapies (1). All patients were ambulatory, leading an independent life up until the time of the last visit or until death from the tumor (2 patients). The median follow-up was 36 months (range, 22 to 52). A paraneoplastic origin should be considered in patients with mild, very slowly progressive sensory neuropathies.

[Nuclear resonance in intracranial venous thrombosis]. / Resonancia nuclear en la trombosis venosa intracraneal.

We have reviewed the nuclear resonance NR images in three patients with intracranial venous thrombosis. Tomodensitometric findings are not reliable in intracranial venous thrombosis and cerebral angiography is required for the diagnosis. The usefulness of NR for the investigation of venous disease has been recently demonstrated. Ten patients in whom a diagnosis of intracranial venous thrombosis was made with NR have been reported. Initially, the vacuum signal in the T1 enhanced sequences disappears and the thrombus becomes hypointense in the T2 enhanced sequences. In the following phase, proton relaxation is impaired by methemoglobin production and thrombosis appears as hyperintense in both the T1 and T2 enhanced sequences. During the resolution phase, the vacuum signal in the vascular lumen reappears and the hyperintensity of the thrombus signal becomes attenuated. In our study we have shown the different stages of intracranial venous thrombosis with NR. In addition, we have demonstrated the close relation between these images and the clinical evolution. NR is a useful noninvasive investigation in patients with suspected intracranial venous thrombosis.

[Antiaggregant treatment for cerebral ischemia: ticlopidine versus aspirin]. / Tratamiento antiagregante en la isquemia cerebral: ticlopidina frente aspirina.

INTRODUCTION: There at present exists marked controversy as to the possible greater efficacy of ticlopidine as opposed to aspirin in brain ischaemia secondary prophylaxis. In our study we compare the efficacy and safety of antiaggregant treatment with ticlopidine as opposed to aspirin in a group of 310 patients admitted to the 'Hospital Mutua de Terrassa' between the years 1990 and 1994. RESULTS: In the group of patients treated with ticlopidine we found a larger number of new cerebrovascular incidents (p = 0.02) and peripheral vascular incidents (p = 0.01). New cerebrovascular incidents were more frequent in males (p = 0.03), in those patients with substantiated infarct (p = 0.02) and in patients with ischaemia in the carotidal region (p = 0.04). On the other hand, the group of patients treated with ticlopidine presented more secondary effects than the group treated with aspirin at the digestive (p < 0.001) and haematologic (p < 0.001) levels. The most frequent digestive secondary effects were diarrhoea (p < 0.001) and hepatopathy (p = 0.02); abnormalities in the leucocyte count were more frequent in patients treated with ticlopidine (p < 0.001), neutropenia being found in 0.8% of cases. CONCLUSION: In the patients we studied ticlopidine was less efficacious than aspirin in the secondary prophylaxis of new vascular incidents (both cerebrovascular and peripheral vascular) and also presented a greater incidence of secondary (digestive and haematological) effects.

[Voluntary changes of visual evoked potentials in cases with hysteria and/or simulation]. / Alteración voluntaria de los potenciales evocados visuales (VEP) en casos de histeria y/o simulación.

We report a 25-year-old woman with gradual loss of visual acuteness, during puerperium and blindness in fifteen days. Two months latter she remain steady. Visual evoked potentials using monocular checkerboard pattern-reversal, were abnormal. Clinical suspicion of voluntary alteration of VEP was considered. A second VEP exploration with binocular stimulation and maneuvers of distraction were carried out. Normal VEP were recorded. Deliberate alteration of the visual evoked potential should be considered in patients suspicious of hysteria or malingering.

[Multiple sclerosis epidemiological situation update: pertinence and set-up of a population based registry of new cases in Catalonia]. / Situación epidemiológica actual de la esclerosis múltiple: pertinencia y puesta en marcha de un registro poblacional de nuevos casos en Cataluña.

INTRODUCTION: The first epidemiological studies on multiple sclerosis (MS) around the world pictured a north to south latitudinal gradient that led to the first genetic and environmental pathogenic hypothesis. MS incidence seems to be increasing during the past 20 years based on recent data from prospective studies performed in Europe, America and Asia. This phenomenon could be explained by a better case ascertainment as well as a change in causal factors. The few prospective studies in our area together with the increase in the disease in other regions, justifies an epidemiological MS project in order to describe the incidence and temporal trends of MS. DEVELOPMENT: A prospective multicenter MS registry has been established according to the actual requirements of an epidemiological surveillance system. Case definition is based on the fulfillment of the McDonald diagnostic criteria. The registry setting is the geographical area of Cataluna (northeastern Spain), using a wide network of hospitals specialized in MS management. CONCLUSION: Recent epidemiological studies have described an increase in MS incidence. In order to contrast this finding in our area, we consider appropriate to set up a population based registry.

[Survey on the current situation of the young neurologists in Spain: analysis of their current working stability and degree of social protection]. / Encuesta sobre la situación actual de los neurólogos jóvenes en España: análisis de la estabilidad laboral y de la protección social.

INTRODUCTION: The aims of this study were to evaluate the working stability and degree of social protection of the Spanish young neurologists, and to know their opinion about their own situation. METHODS: The 343 neurologists that became specialists in Spain between 2000 and 2004 were asked to participate in two consecutive surveys. The first, conducted online, included questions about the availability to change the place of work and the opinion about the situation of young neurologists, and obtained 66 answers. The second was a telephonic and online survey, answered by 217 neurologists, whose questions referred to: places of neurological education and work, type of working contract, and degree of social protection (estimated by the percentage of worked time during which they paid Social Security contributions). RESULTS: Sixty-three per cent (136/217) of the Spanish young neurologists had an unstable job. The most frequent unstable working contracts were: eventual (n=65; 31%), on-call contracts (n=54; 25%) and grants (n=53; 24%). Forty-eight per cent of the neurologists who ended their specialization in 2000 still remained working-unstable. The mean percentage of worked time with full social protection was 71.01+/-36.74%. Less than a half (n=101; 46%) had social protection during the entire worked time, 60 (28.6%) were socially protected during <50% of the worked time, and 23 (11%) never had social protection. A direct relationship was observed 68 between working instability and lower social protection (p=0.0002). The working situation of the Spanish young neurologists was seen as problematic by 97% of the 66 participants in the first survey. CONCLUSIONS: The current situation of the Spanish young neurologists, attending their working stability and degree of social protection, seems precarious and problematic. Urgent actions should be taken by the Administration to improve it.

NARP-MILS syndrome caused by 8993 T>G mitochondrial DNA mutation: a clinical, genetic and neuropathological study.

The 8993 T>G mutation in mitochondrial DNA has been associated with variable syndromes of differing severity ranging from maternally inherited Leigh's syndrome (MILS) to neuropathy, ataxia, retinitis pigmentosa (NARP), depending on the mutation loads in affected patients. We report a kindred with several members in the same generation suffering NARP or Leigh's syndrome due to a 8993 T>G mutation. Post-mortem studies of the brain in one affected member clinically presenting with a neurological disorder intermediate between adult Leigh's syndrome and NARP showed symmetrical lesions of the basal ganglia and brainstem closely resembling those usually described in typical Leigh's syndrome. Analysis of mtDNA in different tissues showed a high proportion of mutant genome in brainstem, cerebral cortex, putamen, cerebellum and thalamus. These observations illustrate the continuum of clinical and neuropathological manifestations associated with the 8993 T>G mutation of the mtDNA.