Ganglioglioma revealed by spontaneous intracerebral hematoma: A cohort study.

BACKGROUND: Gangliogliomas are rare tumors of the central nervous system. Intracerebral hemorrhage (ICH) is a frequent pathological event, rarely related to intracranial tumor. Here we report a cohort of 14 gangliogliomas, 3 of which were revealed by ICH. We also performed a systematic review of the literature on gangliogliomas revealed by ICH. PATIENTS AND METHODS: We retrospectively collected data for all gangliogliomas operated on in our department between January 2015 and December 2019: clinical history, radiological data and outcome, with a special focus on gangliogliomas revealed by spontaneous ICH. All cases had pathological validation. For the systematic review, relevant studies were identified by systematic search of the scientific literature in PubMed. RESULTS: Fifteen patients underwent surgery for ganglioglioma during the study period. Six cases were revealed by seizures, 3 by headache, 1 by hydrocephaly and 1 by developmental disorder. Three patients (21%) had hemorrhagic presentation. All patients were male, aged 30 to 48 years, diagnosed with atypical ICH without vascular abnormality on cerebral angiogram. Conservative management was first decided. Diagnosis of ganglioglioma was made within 3 months for 2 patients and 9 years later for 1, after surgical removal and histological analysis. All hemorrhagic gangliogliomas were BRAF wild type. The systematic review of the literature identified 8 other cases of ganglioglioma revealed by ICH. CONCLUSION: ICH may be a mode of revelation of ganglioglioma, and ganglioglioma is a possible etiology of atypical ICH in young patients. Long-term imaging follow-up is mandatory in patients with unexplained ICH.

Multimodal management of ruptured cerebral micro-arteriovenous malformations: A retrospective series of 19 cases and a review of the literature.

BACKGROUND: Micro-arteriovenous malformations (micro-AVMs) are defined as AVMs just visible on angiography with a nidus size between 0.5 and 1cm. Their principal manifestation is intracranial hemorrhage and their diagnosis and therapeutic management are still unclear. METHODS: The aim of our work was to show the clinical presentation, treatment and outcome of ruptured cerebral micro-AVMs in a retrospective cohort study of 19 patients and a systematic review of the literature. RESULTS: We obtained a total of 20 micro-AVMs in 19 patients. The mean age was 47.3 years. Clinical presentation was acute bleeding. The mean volume of hematoma was 12.9 mm3 (0 - 60.4), with topographic distribution as follows: 64% cortical with supratentorial bleeding, 26% deep, and 10% in the posterior fossa. Among the 20 micro-AVMs of the series, 11 (55%) had endovascular management, 6 (30%) had surgical treatment and 3 (15%) had GK radiosurgery alone. All of our patients have been cured at the end of the follow up without re-permeabilization. In our series, clinical outcome showed good recovery with a mean score of 4.6 on Glasgow Outcome Scale (GOS). In the literature, 88% of patients had a GOS of 4 or 5. DISCUSSION: Intracerebral hematoma (ICH) was the main clinical manifestation. In the case of negative initial angiographic assessment, patients must have supraselective angiographic exploration. In the case of conservative treatment of hematoma, endovascular obliteration and microsurgical exclusion seems to be reasonable therapeutic options, according to our observations.

Impact of discontinuation of antithrombotic therapy after surgery for chronic subdural hematoma.

INTRODUCTION: The management of antithrombotic therapy (AT) after surgery for chronic subdural hematoma (cSDH) requires taking account of the balance of risk between hemorrhage recurrence (HR) and the prophylactic thromboembolic effect (TE). The goal of the present study was to evaluate the prevalence of vascular events (VE: TE and/or HR) in the first 3 postoperative months after cSDH evacuation in patients previously treated by AT. The impact of AT resumption was also evaluated. PATIENTS AND METHODS: This observational prospective multicenter collaborative study (14 French neurosurgery centers) included patients with cSDH treated by AT and operated on between May 2017 and March 2018. Data collection used an e-CRF, and was principally based on an admission questionnaire and outcome/progression at 3 months. RESULTS: In this cohort of 211 patients, VE occurred in 58 patients (27.5%): HR in 47 (22.3%), TE in 17 (8%), with mixed event in 6 cases (2%). Median overall time to onset of complications 26 days±31.5, and specifically 43.5 days±29.25 for HR. Non-resumption of AT significantly increased the relative risk of VE [OR: 4.14; 95% CI: 2.08 - 8.56; P <0.001] and especially of TE [OR: 7.5; 95% CI: 1.2 - 42; P<0.001]. The relative risk of HR was significantly increased when AT was resumed at less than 30 days (P=0.015). CONCLUSION: The occurrence of VE in patients operated on for cSDH and previously treated by AT was statistically significant (27.5%). HR was the most common event (22.3%), whereas TE accounted for only the 8%, although with shorter time to onset. In order to prevent TE risk, AT should be restarted after 30 days, as HR risk is greatly decreased beyond this time.

Cervical pedicular agenesis: Case report and a review of the literature.

Cervical pedicular agenesis, an unusual disorder, is a rare clinical and radiological finding, which can lead to misdiagnosis, moreover in a traumatic situation. The authors report the case of a young woman with a C3 right congenital absence of the cervical pedicle. A systematic review of literature found more than 70 reported cases. In patients with congenital agenesis of the cervical pedicle, the two most common levels of this congenital absence are C6 and C5. The three radiological findings were: the false appearance of an enlarged ipsilateral neural foramen due to the absent pedicle; a dysplastic, dorsally displaced ipsilateral articular pillar and lamina; and a dysplastic ipsilateral transverse process. These pedicle ageneses are a stable congenital anomaly.

Growth of giant intracranial aneurysms: An aneurysmal wall disorder?

The enlargement of giant intracranial aneurysms (IA) can be observed in 30 % of cases resulting in a neurological deficit and epilepsy due to its mass effect. This growth process could be due to a morphological disorder of the IA wall. The authors report on 2 cases of giant IA growth responsible for intracranial hypertension. The treatment of these giant IA required a microsurgical excision combined with a series of cerebral revascularization procedures. The role of vasa vasorum on the inflammatory granuloma outside the vessel, which induced the enlargement, is discussed. These cases illustrate the abluminal vasculopathy as the main involvement of this unfavourable natural history.

Effects of melatonin in the treatment of asthenia in aneurysmal subarachnoid hemorrhage.

BACKGROUND AND OBJECTIVES: Survivors of aneurysmal subarachnoid hemorrhage (aSAH) commonly experience sleep disorders resulting in asthenia. The objective of this prospective study was to determine, in a cohort of patients with treated ruptured intracranial aneurysm (IA), the proportion of asthenia at 2months, in a cohort of patients treated with melatonin and in a control cohort. PATIENTS AND METHODS: Twenty consecutive patients admitted for the treatment of ruptured IA and able to answer a standardized questionnaire were included in the study. After evaluation for fatigue at discharge, we divided our population into 2 cohorts of 10 patients: the first cohort was treated with melatonin for a period of 2months; the second cohort had no specific treatment for fatigue. The primary endpoint was the proportion of asthenia at 2months in both groups. Confounding factors, such as depression, autonomy and apathy were evaluated at the same time. RESULTS: At discharge, there was no significant difference observed between both groups in terms of mean age and initial clinical status (WFNS, Rankin Scale and Fatigue Severity Scale). At 2months, the mean FSS score in the control group was of 4.7±1.0 versus 3.8±0.9 in the melatonin group (P=0.03). The mean MADRS score in the control group was of 1.1±1.45 versus 2.7±2.5 in the melatonin group (P=0.10). The mean LARS score in the control group was of -32.5±1.7 versus -31.7±1.9 in the melatonin group (P=0.24). DISCUSSION: In a prospective evaluation of post-aSAH fatigue, we suggest that melatonin could decrease fatigue. There is no significant impact on depression and apathy. Further studies would be necessary to improve our comprehension of fatigue physiopathology in a context of aSAH.

Lipid patterns in the saliva of smoking young adults.

Salivary lipids are important for the maintenance of oral cavity health. Elevated salivary lipid levels are associated with an increase of caries incidence, plaque development, calculus formation and periodontal disease. However, the regulation of lipid salivary levels is scarcely known. Cigarette smoke is considered a risk factor for oral cavity diseases. We study how cigarette smoke may affect the secretion of salivary lipids. To this purpose, we determine the salivary levels of cholesterol and of glycerolipids in saliva sampled from smokers and non-smokers at various times of day. We observe an increase of glycerophospholipid and a decrease of cholesterol levels in the smokers' saliva collected at 10 p.m. On the other hand, unsaturated fatty acids in chief phospholipids of saliva are lower in smokers at 7 a.m. Therefore, for the first time, we demonstrate that cigarette smoke induces variations of saliva lipid pattern in young people even moderately smoking.

Saturday night brachial plexus palsy.

An unusual case of brachial plexopathy following an alcohol binge is presented. The patient developed numbness and weakness of his right hand and neurophysiological tests demonstrated that the lesion level was at the brachial plexus. MRI of the brachial plexus, cerebrospinal fluid examination and DNA analysis for hereditary neuropathy with liability to pressure palsies were normal. Repeated neurological examination and neurophysiological studies 60 days later were normal. A diagnosis of brachial plexus neuropathy consequent to non-traumatic stretching of the middle and the lower trunks was made.

Post-malaria neurological syndrome: clinical and laboratory findings in one patient.

Post-malaria neurological syndrome (PMNS) is a rare complication of malaria. It follows recovery from an episode of Plasmodium falciparum malaria and is characterised by symptoms and signs of encephalopathy. Patients usually improve without any specific treatment. The pathogenesis is unknown, but it is probably immunologically mediated. The objective of this case study is to describe the first Italian patient with PMNS. A 60-year-old Italian man developed acute P. falciparum malaria after a stay in French Guinea. Twenty days after recovering from malaria, he became confused, developed generalised weakness, limb tremors, shivering and dizziness. These symptoms continued for three days, then resolved spontaneously. Neuroimaging was normal. Cerebrospinal fluid analysis revealed breakdown of the blood/brain barrier, without oligoclonal bands and normal IgG index. Our patient presented a mild diffuse encephalopathy suggestive of a generic activation of the immune system without any specific reaction against antigens within the CNS.

Cervical artery dissection: a 5-year prospective study in the Belluno district.

BACKGROUND: Artery dissection is an unusual cause of ischemic stroke, particularly frequent among young patients. The aim of this study was to collect epidemiological data on artery dissection in a hospital-based community, set up a diagnostic protocol and discover outcome predictors. METHODS: Among patients suffering from cerebral infarction resident in our country, those with clinical and radiological features suggestive of artery dissection were selected. Risk factors, investigative techniques and treatment were evaluated. Patients were subjected to clinical examinations and were regularly tested neuradiologically. RESULTS: Out of 895 ischemic stroke patients, 10 patients with cervical artery dissection (1.1%) were found. Seven patients were treated with anticoagulants and 3 received antiplatelet agents. One posttraumatic artery dissection patient died within a few days of the stroke. None of the patients suffered from a recurrence, while serious disability occurred in 4 of them. CONCLUSIONS: Artery dissection should be suspected in any cerebral infarction patient, especially in young patients without risk factors for cerebrovascular diseases. The treatment of choice consists of anticoagulants. An early clinical diagnosis, strongly supported by radiological tests, is mandatory to start the proper treatment and achieve the best possible outcome.

Antithyroid antibodies in the CSF: their role in the pathogenesis of Hashimoto's encephalopathy.

Antithyroid antibodies and circulating immune complexes (CIC) were found in the CSF of six patients with Hashimoto's encephalopathy (HE) but not in the CSF of 21 controls. The synthesis of autoantibodies and CIC was intrathecal and their titers were independent of the patients' clinical status or therapy. Their presence in the CSF of patients with acute or subacute encephalopathy may be useful in diagnosing HE.

[Quadriplegia and prolonged respiratory insufficiency due to tick-borne encephalomyelitis. Case report]. / Tetraplegia ed insufficienza respiratoria prolungata conseguenti ad encefalomielite virale da puntura di zecca. Descrizione di un caso.

Tick-borne encephalitis (TBE) is an uncommon and potentially severe illness. TBE virus is transmitted to the humans by an infected tick and it spreads to the central nervous system determining various clinical pictures. A case in which TBE virus caused an encephalomyelitis with quadriplegia and respiratory insufficiency that persist one year after the diagnosis, is reported. Such a clinical manifestation of TBE has never been described in Italy till now.

Autoimmunity to beta IV spectrin in paraneoplastic lower motor neuron syndrome.

Paraneoplastic neurological disorders may result from autoimmunity directed against antigens shared by the affected neurons and the associated cancer cells. We have recently reported the case of a woman with breast cancer and paraneoplastic lower motor neuron syndrome whose serum contained autoantibodies directed against axon initial segments and nodes of Ranvier of myelinated axons, including the axons of motoneurons. Here, we show that major targets of the autoantibodies of this patient are betaIVSigma1 spectrin and betaIV spectrin 140, two isoforms of the novel betaIV spectrin gene, as well as a neuronal surface epitope yet to be identified. Partial improvement of the neurological symptoms following cancer removal was associated with a drastic reduction in the titer of the autoantibodies against betaIV spectrin and nodal antigens in general, consistent with the autoimmune pathogenesis of the paraneoplastic lower motor neuron syndrome. The identification of betaIV spectrin isoforms and surface nodal antigens as novel autoimmune targets in lower motor neuron syndrome provide new insights into the pathogenesis of this severe neurological disease.

Type III export: new uses for an old pathway.

Gram-negative bacteria use type III secretion (TTS) systems to translocate proteins into the extracellular environment or directly into eukaryotic cells. These complex secretory systems are assembled from over 20 different structural proteins, including 10 that have counterparts in the flagellar export pathway. Secretion substrates are directed to the TTS machinery via mRNA and/or amino acid secretion signals. TTS chaperones bind to select secretion substrates and assist in the export process. Recent progress in the understanding of TTS is reviewed.

Can seizures be the only manifestation of transient ischemic attacks? A report of four cases.

Several studies have investigated the frequency of epileptic seizures following ischemic strokes and transient ischemic attacks (TIAs). Little attention has been paid to the possibility that seizures may be precipitated by TIAs. We examined if seizures can be the only symptom of a TIA and how often this might occur. We performed a retrospective analysis of clinical charts and electroencephalograms of 160 consecutive patients evaluated for a first-ever seizure from January 1997 to December 1999 at Belluno General Hospital. From January to May 2000, 19 more first-ever seizure patients were evaluated directly. Four patients (2%) had seizures in the presence of important risk factors for ischemic stroke (atrial fibrillation in two patients, atrial fibrillation and ventricular mural thrombus in one patient, hemodynamically significant left carotid stenosis in one patient). Seizures were not accompanied by other neurological deficits or brain lesions on CT or MRI. As risk factors for brain ischemia are frequent in the general population not developing seizures, our results do not prove that the occurrence of seizures was more than casual in these patients. Yet they indicate that in a small percentage of patients, seizures can occur in a context highly suggestive of TIA, with no other focal deficits.

A novel antineuronal antibody in a motor neuron syndrome associated with breast cancer.

A 72-year-old woman developed a lower motor neuron syndrome (MNS) 4 months before the appearance of breast cancer. Monoparesis progressed to quadriparesis despite high-dose IV immunoglobulins, plasma exchange, and azathioprine, and high-dose IV methylprednisolone. The patient improved only after the removal of the tumor. MRI demonstrated hyperintensities in the cervical spinal cord. The patient had antibodies that reacted with axonal initial segments and nodes of Ranvier. The findings suggest that in this patient lower MNS may be a paraneoplastic condition associated with breast cancer.

Testing of the AVL OPTI 1 portable blood gas analyzer during inflight conditions.

The "AVL OPTI 1" (AVL Medical Instruments, Saint-Ouen l'Aumone, France), a completely automated portable blood gas analyzer, was chosen because of its accuracy under usual sea-level conditions and because of its new technology which broadens the possibilities for in-flight blood determinations: a) a single use cassette made of plexyglass containing the measurement chamber and the aligned sensors required for pO2, pcO2 and pH determination; b) calibration coefficients memorized in a bar code label fixed on the packing material; c) a quality control of each individual cassette prior to the measurement and at least once a day by two standard reference cassettes simulating high and low levels of pH, pcO2 and pO2; and d) a fully automatic introduction of the blood sample. The complete analytical cycle requires about 3 min with a sample volume of 80 microL of whole blood. After the measurement, the cassette containing blood sample is destroyed. Moreover, this device uses optical electrodes or "optodes" (fluorescence sensors). We tested the accuracy and imprecision of pO2 and pcO2 sensors on fresh blood which was equilibrated with four different gas mixtures at four different altitudes (250, 8000, 10,000 and 13,000 ft), simulated in a decompression chamber. Gas measurement optodes had linear responses and were accurate for all measured pO2 and pco2 values (n = 66), except for at high values of PO2 (>150 mmHg) and pco2 (>65 mmHg). The pressure value given by the AVL OPTI 1 was controlled before the experiment began and during the different depression levels. Barometric pressure results showed: a) concordance of pressure values with those of ground instrumentation; b) stable response; and c) absence of hysteresis. We conclude that the performance of the AVL OPTI 1 is satisfactory during inflight conditions.

Marchiafava-Bignami disease: computed tomographic scan, 99mTc HMPAO-SPECT, and FLAIR MRI findings in a patient with subcortical aphasia, alexia, bilateral agraphia, and left-handed deficit of constructional ability.

OBJECTIVES: To report and discuss the neuropsychological deficits and neuroimaging findings in a patient with probable Marchiafava-Bignami disease. DESIGN AND METHOD: A right-handed woman with chronic alcoholism demonstrated mutism, impaired comprehension of spoken language, alexia, and right-handed agraphia. The syndrome of interhemispheric disconnection was manifested by left-handed deficit of constructional ability and agraphia. The patient underwent brain computed tomographic scans, technetium 99 hexylmethylpropylene amineoxime-single photon emission computed tomography, and magnetic resonance imaging (MRI) that also included fluid attenuated inversion recovery images. SETTING: Clinical neurology department. RESULTS: The patient's symptoms were related to scattered lesions of the corpus callosum and to extensive symmetrical lesions of the centrum semiovale. Only the latter were detected by computed tomographic scans. Results of single photon emission computed tomography did not show areas of focal hypoperfusion. Results of fast spinecho MRI showed all lesions were hyperintense in T1-weighted images and hypointense in T2-weighted images. Fluid attenuated inversion recovery images revealed that periventricular lesions had a hypointense core surrounded by a hyperintense rim; callosal lesions were still hyperintense. CONCLUSIONS: We believe that our patient's symptoms are due to the discontinuous affection of the corpus callosum and to the bilateral cutting of the outflow from the cortex. The MRI findings may be interpreted as indicating central necrosis and peripheral demyelination of periventricular lesions and demyelination of the corpus callosum. The combined use of fast spin echo and fluid attenuated inversion recovery MRI reproduced with more accuracy than fast spin echo MRI alone some features of Marchiafava-Bignami disease known from observations at autopsy.

Recurrent external ophthalmoparesis during hormonal therapy after thyroid ablation. Case report.

We here report the case of a patient who had undergone total thyroid ablation for Graves' disease. After the beginning of oral therapy with 1-thyroxine, she developed a left external ophthalmoparesis that remitted with the discontinuation of the drug and recurred whenever the replacement therapy (1-thyroxine or tri-iodothyronine) was reintroduced.