Primary hydatidosis of the central nervous system: A retrospective study of 39 Tunisian cases

Objective

To analyze epidemiological characteristics, clinical symptoms, radiological aspects, treatment and outcome of central nervous system hydatidosis and compare our results with those reported in literature.

Patients and methods

In our retrospective study, we reviewed 39 cases of primary central nervous system hydatid cysts operated on in our hospital between 1998 and 2007.

Results

There were 20 male and 19 female patients (sex-ratio M/F = 1.05) between 2 and 68 years of age (mean = 26.5 years). Thirteen of the patients were children (33.3%) with a mean age of 6.8 years and 26 were adults (66.7%) with a mean age of 36.3 years. The location of hydatid cysts was intracranial in 27 cases (69.2%) and spinal in 12 cases (30.8%). Headache and motor deficits were the predominant symptoms in patients with intracranial hydatidosis whereas back pain and spinal cord compression syndrome were the most frequent clinical presentations in patients with spinal hydatidosis. All patients underwent surgical resection of the cyst. Pathologic findings were consistent with hydatid cyst in all cases. During the follow-up period which ranged between 12 months and 5 years, 12 patients had recurrence (30.7%). Only one patient with intracranial hydatid cyst died postoperatively due to anaphylactic shock.

Conclusion

Despite all the advances in imaging techniques and therapeutic methods, central nervous system hydatidosis remains difficult to cure and patient outcomes are not satisfactory especially in case of spinal involvement due to the high incidence of recurrence.     

Long-term follow-up of vanishing tumors in the brain: How should a lesion mimicking primary CNS lymphoma be managed?

Objectives

The spontaneous disappearance of a tumor is referred to as a vanishing tumor. Most vanishing tumors in the brain are eventually diagnosed as malignant tumors or multiple sclerosis. However, their long-term clinical course remains unclear. This study aims to elucidate the management of vanishing tumors in the brain.

Patients and methods

We defined a vanishing tumor as a case in which the tumor spontaneously disappeared or decreased to less than 70% of the initial tumor volume before definitive diagnosis and treatment (other than steroid treatment). Ten cases of vanishing tumors are reviewed.

Results

Nine patients underwent biopsy at least once. Five patients, all of whom had malignant tumors (primary central nervous system lymphoma: 4, germinoma: 1) that recurred in 4–45 months (median: 7 months), underwent a second biopsy after the reappearance of the tumors. Five patients (tumefactive demyelinating lesion: 1, undiagnosed: 4) who had no relapse are alive, and their median follow-up time is 44 months. No cases have yet been reported of malignant brain tumors that recurred more than 5 years after spontaneous regression.

Conclusions

Patients with vanishing tumors should be followed up carefully by magnetic resonance imaging for at least 5 years, even after the disappearance of an enhancing lesion.     

Cefaleas en la persona mayor: características de una serie de 262 pacientes

Introduction

Although headache prevalence decreases in patients older than 65, headaches are a common complaint and their different clinical and therapeutic features must be understood. This article analyses the clinical characteristics of elderly patients treated in an outpatient headache unit.

Methods

We collected demographic and clinical data from patients treated in a tertiary hospital headache unit between January 2008 and May 2013. Headaches were codified according to the International Classification of Headache Disorders, 2nd edition (ICHD-2).

Results

Of a total of 1868 patients treated, 262 patients (14%, 189 women and 73 men) were older than 65 years. Ninety-nine (68 women, 31 men, 5.3% of the total) were over 75. Headaches began after the age of 65 in only 136 patients (51.9%). The 362 headaches were codified as follows: 23.8% as Group 1 (Migraine) and 28.7% as Group 2 (Tension-type headache). We diagnosed 58 (16%) secondary headaches; 26 (7.2%) were classified as Group 13 (Cranial neuralgias) and 23 (6.4%) in Group 14 (Other headaches). Symptomatic medication overuse was detected in 38 patients (14.5%). We also identified headaches considered typical in the elderly, including chronic migraine (41 cases), hypnic headache (6), occipital neuralgia (4), SUNCT (2), cervicogenic headache (1), primary cough headache (1), and giant cell arteritis (2).

Conclusions

Elderly patients were frequently treated in our outpatient headache unit. Tension-type headache was the most common diagnosis in this population. Geriatric headache syndromes such as hypnic headache or occipital neuralgia were also represented in our series.     

A rare case of nonenhancing primary central nervous system lymphoma mimic multiple sclerosis

Primary central nervous system lymphoma (PCNSL) is reported to have increased in the last decades. Early diagnosis is crucial for proper management of this tumor. We report a case of a 48-year-old man who was initially diagnosed with multiple sclerosis. Magnetic resonance imaging of the brain revealed multiple lesions with hypersignals in the bilateral basal ganglia and brain stem in T2-weighted image and non-enhancement, while positron emission tomography showed a low uptake of ¹⁸F-fluorodeoxyglucose in the affected brain, indicative of demyelination. However, this individual was correctly diagnosed with PCNSL after biopsy and further histological analysis. Primary central nervous system lymphoma must be considered even when nonenhancing, diffuse lesions are seen on MRI. A visible tumor on imaging is essential to ensure an early brain biopsy and histological diagnosis.Primary central nervous system lymphoma (PCNSL) accounts for 3.3% of all brain tumors.1 Primary central nervous system lymphoma in immunocompetent patients is non-Hodgkin lymphomas of germinal B-cell origin in the vast majority of cases, which arise from the brain, spinal cord, cerebrospinal fluid (CSF), or eyes in the absence of systemic disease.1,2 Because of its ambiguous clinical manifestation, neuroradiographic, and CSF cytology, PCNSL can be easily misdiagnosed. Our objective in presenting this particular case is to highlight that PCNSL should be considered even when non-enhancing, diffuse lesions are seen on MRI.     

Central nervous system fungal infections: Observations from a large tertiary hospital in northern India

Objective

To report our observations regarding fungal infections of the brain over two years from a large tertiary hospital in northern India. To identify fungal infections in immunocompetent and immunocompromised patients and to compare the two groups with respect to their age of occurrence, demographic data, clinical profile, radiological findings, response to treatment and outcome.

Methods

All consecutive cases of central nervous system (CNS) fungal infections admitted to the hospital over two years were included in this study. The patients were categorized as immunocompetent and immunocompromised, the predisposing factors, symptoms and clinical presentation were studied in detail and the outcomes of the two groups were compared.

Results

Of the 50 cases, 25(50%) were that of mucormycosis, 17(34%) were cryptococcosis and 8(16%) cases were that of aspergillosis. 14(28%) cases were immunocompetent and 36(72%) were immunocompromised. The outcome studied was as follows: 28% died of which 4% were HIV positive, 12% were diabetics with severe rhinorbital involvement, 2% had idiopathic thrombocytopenic purpura (ITP), 6% had advanced cancer and 4% had no predisposing illness.

Conclusions

We observed fungal infections of the brain in both immunocompetent and immunocompromised patients. In immunocompetent patients, early diagnosis and appropriate treatment frequently leads to recovery from the illness. The mortality and morbidity of fungal infections are less in the immunocompetent group of patients.     

Elevated titers of anti-thyroperoxidase antibodies in patients with multiple system atrophy: A pilot study

Objectives

Multiple system atrophy (MSA) is a neurodegenerative disease characterized by progressive neuronal loss and alpha-synuclein deposition in oligodendroglial cells in the central nervous system. The cause of MSA remains essentially unknown. A cerebellar syndrome was associated with autoimmune thyroid disease in some cases, apparently not related to MSA and was partially responsive to immunomodulatory therapy.

Patients and methods

28 euthyroid patients who fulfilled the diagnostic criteria for probable MSA, 11 with MSA-cerebellar type (MSA-C), 17 with MSA-parkinsonian type (MSA-P), 28 patients with Parkinson's disease (PD) and 26 normal euthyroid controls were tested the for serum levels of anti-thyroperoxidase antibodies (anti-TPO) and anti-thyroglobulin (Anti-TG) antibodies (Ab).

Results

The laboratory results were statistically similar in all three groups, but 3 MSA-C patients had highly elevated anti-TPO Ab titers.

Conclusion

We identified the presence of elevated anti-TPO levels in a small subgroup of MSA-C patients but neither in MSA-P or PD patients nor in healthy controls. These findings may suggest an autoimmune etiology in some cases of MSA-C.     

Central nervous system immune reconstitution inflammatory syndrome in AIDS: experience of a Mexican neurological centre

Background

Highly active antiretroviral therapy (HAART) restores the inflammatory immune response in AIDS patients and it may unmask previous subclinical infections or paradoxically exacerbate symptoms of opportunistic infections. Up to 25% of patients receiving HAART develop immune reconstitution inflammatory syndrome (IRIS). We describe six patients with IRIS central nervous system (CNSIRIS) manifestations emphasizing the relevance of CSF cultures and neuroimaging in early diagnosis and management.

Methods

Patients with CNSIRIS were identified among hospitalized HIV-infected patients that started HAART from January 2002 through December 2007 at a referral neurological center in Mexico.

Results

One-hundred and forty-two HIV-infected patients with neurological signs were hospitalized, 64 of which had received HAART, and six (9.3%) developed CNSIRIS. Five patients were male. Two cases of tuberculosis, two of cryptococcosis, one of brain toxoplasmosis, and one possible PML case were found. IRIS onset occurred within 12 weeks of HAART in five patients. Anti-infective therapy was continued. In one case, HAART was temporarily suspended. In long-term follow-up the clinical condition improved in all patients.

Conclusions

CNSIRIS associated to opportunistic infections appeared in 9% of patients receiving HAART. Interestingly, no cases of malignancy or neoplasm IRIS-related were found. Frequent clinical assessment and neuroimaging studies supported diagnosis and treatment. Risk factors were similar to those found in other series.     

Suicide attempts by deliberate self-poisoning in children and adolescents

Aim

The objective of the study was to examine the toxicological characteristics of suicide attempts by deliberate self-poisoning in children and adolescents.

Method

From the Toxicological Information Centre's database, the inquiries due to the suicide attempts in children (9–13 years old) and adolescents (14–18 years old) were evaluated.

Results and conclusions

From 10,492 calls concerning suicide attempts, 2393 concerned children and adolescents. Most suicide attempts were attempted in spring (31.3%). Among the toxic agents, drugs were used in 97.8% cases. The most frequent ingestions appeared using drugs affecting the nervous system and anti-inflammatory non-steroids. The dose was evaluated as toxic in 73.4%, severely toxic in 3.0% and unknown in 11.2% cases. Only one in 10 children used a non-toxic dose. Girls, more frequently than boys (13.2% vs. 8.9%), used non-toxic doses. The symptoms of moderate and severe intoxications were present in 10.5% of the cases. Poison centre consultation was accessed within the first hour after the ingestion in one-fifth of the patients. In both age groups, the severity of the intoxication was greater among elder males who reached the medical facilities later than 4 h after the poisoning. The combinations of three or more drugs affecting central nervous system were present in the most severe cases.     

Posterior reversible encephalopathy syndrome as a complication of acute lupus activity

Objectives

We aimed to describe the clinical and imaging characteristics; associated risk factors and neurological outcome of posterior reversible encephalopathy syndrome (PRES) in patients with systemic lupus erythematosus (SLE).

Methods

From October 2001 to January 2007, we identified patients with SLE and the criteria for PRES in our institution, which is a tertiary-care referral center for patients with SLE; the patients were evaluated at baseline and followed to determine the clinical outcome.

Results

We identified 22 episodes of PRES in 21 patients; 20 (95.2%) were women, mean age of onset was 24.9 ± 8.6 years, all patients had high systemic activity (SLEDAI scores from 12 to 39). Acute hypertension was observed in 18 episodes (81.8%), and renal failure in 16 (72.7%); only 3 patients were on cyclophosphamide at the time of the onset of PRES. Persistent neurological deficit was observed in 2 cases; one patient died during the acute episode.

Conclusions

PRES is a central nervous system syndrome that is observed in SLE patients. It was associated mainly to high systemic activity, acute hypertension, and renal failure. Although reversibility is common, residual neurological damage may be observed.     

Depression in multiple sclerosis

Introduction

Multiple sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system. Prevalence of depression in MS is significant. Existence of mood disorders alters the patients’ life quality.

Objective

To determine the prevalence of depression in MS and establish the relationship between the severity of the disease, the onset of depression and their correlation with neuroimaging.

Methods

Fifty patients with MS aged 20 to 50 years followed at the Neurology Department of Charles-Nicolle Hospital from 2008 to 2012 participated in this study. The evaluation included a neuropsychological assessment, physical examination with EDSS and MRI.

Results

Association between MS and depression is common and known. In our study, prevalence of depression was 65%. Coexistence of other psychiatric disorders was found in around 10% of patients. Mood disorder was inaugural in some cases and delayed in others. This suggests that depression in MS may be linked to the disease or a result of a functional disability process. Occurrence of depression was not directly related to disease severity in all cases studied.

Conclusion

Depression is a possible manifestation of MS. This mood disorder is due to the demyelinating brain damage or to a genetic susceptibility. However, a fortuitous association cannot be excluded.     

Headaches related to psychoactive substance use

Aim

To investigate a possible association between headache and psychoactive substance use.

Methods

1055 psychoactive substance abusers were consecutively admitted. All patients filled out a detailed headache questionnaire and 1015 patients were included.

Results

Twenty seven percent of patients reported having headache. Eighteen percent of patients reported having headache attributed to a substance or its withdrawal and 1.4% had unclassified headache. The most commonly used substances were cannabis (80.5%), alcohol (74.6%), methylamphetamine (18.7%), benzodiazepine (10.4%), volatile solvent (5.8%), cocaine (4.4%), heroin (2.1%), opioids (0.5%), and other substances (1.7%). Fifteen patients reported that onset of headache occurred prior to onset of substance use, while 94.5% had headaches occurred after substance abuse. A higher incidence of headache was found in the benzodiazepine, methylamphetamine, cocaine, heroin, volatile solvent abusers. Seventy-eight percent of headache patients have never sought help from a physician despite the severity and frequency of headache.

Conclusions

In our study, the prevalence of headache among all psychoactive substance abusers was 26.9%. Although this is one-group study without any comparison with non-addict population and associational data must be interpreted with caution, the results of this study indicate a possible relationship may exist between headache and substance use since 94.5% of substance users described headaches after the onset of substance use. The younger start and the longer duration of cannabis use caused the higher incidence of headache, but this correlation was not observed in other substance use. Migraine was far more prevalent in the abusers than in previously reported community populations.     

The different faces of Creutzfeldt-Jacob Disease CJD in psychiatry

Objective

Creutzfeldt-Jacob Disease (CJD) is a rapidly progressive spongiform disease of the central nervous system. Psychiatric symptoms, though considered rare, can be the presenting symptoms of CJD and impose diagnosis difficulties. We reviewed prospectively our database to identify the frequency of psychiatric symptoms as identifying symptoms among our community.

Methods

We included all patients in Sheba Medical Center who were diagnosed with CJD between the years 2006 and 2012. Data were collected retrospectively.

Results

Twenty-three patients with CJD were admitted to our hospital during this 6-year period. Among them, 10 (44%) were diagnosed first as “psychiatric patients” due to psychiatric presenting symptoms.

Conclusion

In our series, the frequency of misleading psychiatric symptom was 44%. Clinicians should therefore include CJD in their differential diagnoses of new onset dementia, particularly when associated psychosis and depression symptoms persist and worsen, despite standard psychiatric treatments.     

Brainstem lesions may be important in the development of epilepsy in multiple sclerosis patients: An evoked potential study

Objective

Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system, with epileptic seizures sometimes observed in the same patients. In this study, we used evoked responses to study the pathogenesis of epilepsy in MS.

Methods

Patients with a diagnosis of definite MS and who had EPs performed (visual (VEP), brainstem auditory (BAEP) and short latency somatosensory (upper (USSEP) and lower (LSSEP))) were retrospectively included in this study. They were divided into three groups; Group I: Patients with no epilepsy and who were not taking anti-epileptic drugs (AED); Group II: Patients with epilepsy and taking AEDs; and Group III: Patients with no epilepsy who were taking AEDs for symptoms related to neuropathic pain.

Results

Three hundred and fifty-five patients were included in this study; Group I: 229 patients (64.5%), Group II: 20 patients (5.6%) and Group III: 106 patients (29.9%). The proportion of patients with abnormal BAEP and USSEP was higher in Group II.

Conclusions

A positive association exists between the presence of epilepsy in MS patients and BAEP and USSEP abnormalities. Analysis of Group III ruled out AED use as a factor.

Significance

Brainstem lesions may be the cause of epileptogenicity in MS.     

Significance of the T2*-weighted gradient echo brain imaging in patients with infective endocarditis

Background

Although aneurysm formation accompanying parenchymal hemorrhage is one of devastating complications in the central nerves system (CNS), imaging studies of the brain are not routinely warranted in patients with infective endocarditis (IE). To assess the clinical importance for detecting silent lesions in the central nervous system, we investigated hypointense signal spots detected on the brain T2*-weighted MR imaging in patients with IE.

Methods and results

Eleven patients with IE were retrospectively reviewed. Seven patients (63.6%) showed hypointense signal spots on T2*-weighted MR images. The number of hypointense signal spots increased within only a few weeks in five patients.

Conclusion

The brain T2*-weighted MR imaging in patients with IE may have a potential role to detect CNS lesions with clinical significance of potentially high risk of intracranial hemorrhage. T2*-weighted hypointense signal spots may be specific to brain involvement, and be quite useful in monitoring CNS lesions associated with IE, even if they are asymptomatic.     

Manifestaciones clínicas y neurorradiológicas en los adultos con neurofibromatosis tipo 1

Introduction

Type 1 neurofibromatosis is the most common neurocutaneous syndrome. Most published case series study the paediatric population.

Material and methods

Cross-sectional study of cases of type 1 neurofibromatosis from neurology departments that were recorded in a database. We analysed the different clinical variables providing the diagnosis as well as demographic and neuroradiological variables.

Results

We found a total of 31 patients with type 1 neurofibromatosis. The mean age was 28.9 years and 58.4% were women. Subjects with unidentified bright objects (UBOs) were younger than those without them (22.45±8.22 years vs. 32.5±10.64; P=.011). In contrast, subjects with neurofibromas were older than those without them (30.56±10.68 years vs. 18.25±4.34; P=.032). No sex differences were found in the presentation of clinical or radiological variables. Seven patients (22.6%) had tumours; 3 were optic pathway gliomas (1 bilateral), 3 were plexiform neurofibromas, and 1 was a pilocytic astrocytoma in the brainstem.

Conclusions

Patients with type 1 neurofibromatosis presented both peripheral neurofibromas and tumorous lesions of the central nervous system. Subjects with neurofibromas were older than those who did not present them, while subjects with UBOs were younger than those without such lesions.     

Evaluation on a clinical course of subdural hematoma in patients undergoing epidural blood patch for spontaneous cerebrospinal fluid leak

Objective

Subdural hematoma (SDH) is a frequent complication of spontaneous intracranial hypotension (SIH), in which epidural blood patch (EBP) may be applied as a treatment to stop cerebrospinal fluid (CSF) leak. However, a clinical course of SDH in SIH patients has not been sufficiently evaluated. We retrospectively evaluated the temporal relationships between EBP and SDH in the patients with SIH.

Methods

Twenty-nine consecutive patients, diagnosed as SIH, were studied. Clinical records and images were retrospectively evaluated. When orthostatic headache continued for 2 weeks regardless of conservative treatment, EBP was performed under fluoroscopy.

Results

We detected 13(45%) cases of SDH (mean age 44 years, 8 males and 5 females). In 6 patients, SDHs disappeared after effective EBP, i.e., after the disappearance of orthostatic headache. In 3 patients, SDHs were enlarged or recurred after effective EBP, and in 4 patients, SDHs were first detected after effective EBP.

Conclusion

The knowledge of the presence of these types of SDH (enlarged or recurred or detected after EBP) may deserve clinical attention.     

Retrospective study of 23 pathologically proven cases of central nervous system tuberculomas

Introduction

Extrapulmonary manifestations of tuberculosis involving the central nervous system (CNS) due to haematogenous spread are not a rare entity. It presents as meningitis or tuberculoma. Tuberculoma is a granulomatous inflammatory process mimicking a neoplasm radiologically, so usually a biopsy is performed.

Material and results

Our study consisted of 23 pathologically proven cases of tuberculomas between 1988 and 2003. Patients were discussed clinically, radiologically and histologically. Headache, fever, weight loss and weakness are the most common clinical manifestations. Our patient's ages vary from 3 to 67 years with a mean of 31.8 years. Ninety-five percent of patients had bad social, economic and nutritional conditions. None of them were infected by human immunodeficiency virus (HIV). All patients had similar contrast-enhancing lesions radiologically. The majority of tuberculomas were located supratentorially. Only one patient presented two foci of (cerebral and cerebellar) tuberculomas. Nineteen tuberculomas were intracerebral; two were located in the cerebellum and one was intramedullary. Among those lesions, one cavernous sinus tuberculoma and one sellar tuberculoma were identified. Only two patients underwent stereotactic biopsy and 21 patients underwent surgical excision. Histopathologic examination revealed granulomatous inflammation with central caseous necrosis in all patients.

Discussion

Diagnosis of tuberculoma can be difficult, and in most of our cases, the clinical diagnosis was ‘neoplasm’. For this reason, clinicians must always be aware of it and consider it in the differential diagnosis of central nervous system mass lesions.     

Efferent and afferent evoked potentials in patients with adrenomyeloneuropathy

Objective

This paper investigates efferent and afferent conductions of the central nervous system by various evoked potentials in patients with adrenomyeloneuropathy (AMN).

Patients and methods

Ten pure AMN patients without cerebral involvement were studied. Motor evoked potentials (MEPs), somatosensory evoked potentials (SEPs), auditory brainstem response (ABR), and pattern reversal full-field visual evoked potentials (VEPs) were recorded. For MEP recording, single-pulse or double-pulse magnetic brainstem stimulation (BST) was also performed.

Results

Abnormal MEP was observed in all ten patients, abnormal SEP in all ten, abnormal ABR in nine, and abnormal VEP in only one. Brainstem latency was measured in three of the seven patients with central motor conduction time (CMCT) prolongation. The cortical–brainstem conduction time was severely prolonged along the normal or mildly delayed brainstem–cervical conduction time in those three patients.

Conclusions

The pattern of normal VEP and abnormal MEP, SEP, ABR is a clinically useful electrophysiological feature for the diagnosis. BST techniques are helpful to detect, functionally, intracranial corticospinal tract involvement, probably demyelination, in pure AMN patients.     

Medical complication in adults with spina bifida

Setting

Cohort study.

Introduction

Spina bifida (SB) is a congenital malformation affecting the central nervous system (CNS) and is one of the most prevalent CNS disorders in children. Hydrocephalus (HC) is present in 80% of newborns with SB. The aim of the present study was to analyze the medical complications and to relate the complications to age at examination, the level of injury, AIS grade and presence of HC in adults with SB.

Materials and methods

SB patients were recruited from the Spinalis out-patient clinic at the Karolinska University Hospital at their annual follow-up. The patients underwent a thorough general and neurological examination and background data including medical complications were retrieved from the medical files.

Results

127 of 157 (82%) SB patients (114 with MMC and 13 SB occulta) with a mean age of 34 years were included. Half of the patients had a complete SCI and a lumbar level was most common. Nearly 60% of the patients had HC. 88 patients (69%) suffered from at least one medical complication. Urinary tract infection (UTI), scoliosis and pain were the most common complications found in 46%, 30% and 28% of the patients, respectively. Less common complications were epilepsy, pressure ulcers (PU) and spasticity.

Discussion

SB gives a disability including motor, sensory dysfunctions and the patients suffer from a high frequency of medical complications like UTI, scoliosis, pain, and epilepsy. Data gives basis for adequate routines for medical examination at the follow-up.