Subacute Evolution Research Articles

Clinical and Neuropathological Variability in Clinically Isolated Central Nervous System Whipple's Disease

Central nervous system Whipple's disease (CNS-WD) with clinically isolated neurological involvement is a rare condition fatal without an early diagnosis. We aimed to present clinical and neuropathological features of three cases of pre- or post-mortem polymerase chain reaction confirmed CNS-WD with distinct clinical presentation, outcome and pathological findings. One patient had an acute onset with spinal and brainstem involvement and died without CNS-WD diagnosis after 14 weeks. Neuropathology showed extensive inflammatory and necrotizing lesions with abundant foamy periodic-acid-Schiff (PAS)+ macrophages. The second patient had a subacute evolution with late CNS-WD diagnosis and death occurring 18 months after onset despite antibiotic treatment. Brain examination showed inflammatory lesions in the brainstem, thalamus and cerebellum, and abundant foamy PAS+ macrophages. The third case was diagnosed within 4 weeks of onset and treated with an excellent response. He died after a disease-free period of 24 months of unrelated causes. Neuropathology showed cystic residual lesions devoid of microorganisms without inflammatory reaction. CNS-WD may have an acute or subacute course with variable response to treatment. Accordingly, subjacent lesions may be those of a severe acute necrotizing encephalitic process or subacute inflammatory lesions involving diencephalic, brainstem, cerebellar and spinal regions. Chronic, cavitary brain lesions may be sequelae of a successful treatment. Early diagnosis should allow appropriate treatment and improve prognosis.

Mort subite au cours d’une dégénérescence cérébelleuse subaiguë paranéoplasique avec dysautonomie associée aux anticorps anti-Hu révélant un cancer bronchique à petites cellules

IntroductionParaneoplastic syndromes are a rare cancer complication with a frequent subacute evolution. ObservationA 62-year-old man was admitted presenting with a cerebellar syndrome and orthostatic hypotension with dysautonomia. Anti-Hu antibody research was positive. A subcarinal adenopathy biopsy found out a small cell lung carcinoma. Despite a treatment with immunoglobulin and chemotherapy, the patient died suddenly, after a raise of dysautonomia symptoms. ConclusionSudden death observations represent exceptional complications of paraneoplastic syndrome. They might be secondary to arrhythmias, ictal asystol or laryngospasm. Systematic research of paroxystic heart arrhythmias with holter-ECG in paraneoplastic syndrome may prevent sudden deaths.

Facial Sporotrichosis in children from endemic area in Peru

Sporotrichosis is a subcutaneous mycosis subacute or chronic evolution, caused by the dimorphic Sporothrix complex, which includes five species: Sporothrix albicans, Sporothrix brasiliensis, Sporothrix globosa, Sporothrix mexicana and Sporothrix schenckii (sensu stricto). The infection occurs after trauma with contaminated material, which inoculated the fungus on the skin. The clinical types of sporotrichosis are lymphocutaneous sporotrichosis, fixed cutaneous (nodulopapular, ulcerative, verrucouse and furunculoide) and extracutaneous [1,2]. It is a mycosis of a universal distribution, but more commonly seen in tropical or subtropical countries. The majority of cases are found in Mexico, Central America and Peru [1]. However, the region of Abancay in Peru is considered hyperendemic and has an annual incidence of 48-60 cases per 100 000 population [3]. Also, the incidence is three times higher in children aged 0-14 in ≥ 15 years of age, approximately 1 case/1000 children 0-14 years and a frequency of 60% [4,5]. In pediatric patients from endemic areas the most anatomical site affected is the facial region (40-42%) [5,6], with lesions on the nose, face, chin, malar, genian and palpebral that is clinically rarely mentioned in the literature [7].

Molecular adsorbent recirculating system therapy in a rare case of fulminant hepatitis

Background: Acute hepatitis C virus infection leading to fulminant hepatitis is very rare whereas Hepatitis C virus (HCV) infection is one of the main causes of chronic liver disease worldwide. The repertoire of substances that accumulate in the blood in fulminant hepatic failure cause neurological abnormalities, aggravate injury to the liver and other organs, suppress the ability of residual hepatocytes to perform organ-specific functions (sick cell syndrome), and inhibit the hepatic regenerative response especially in fulminant hepatitis Virus C which has subacute clinical evolution and takes time to manifest. Liver support technology is evolving as different techniques become available that assist the remaining functional cell mass by providing specific liver functions. Case Presentation: We report a case of Fulminant C virus Hepatitis, successfully treated with albumin dialysis Molecular Adsorbent Recirculating System (MARS). At time of admittance the patient presented: Model End-stage Liver Disease (MELD)-36; Child Turcotte Pugh (CTP)-C(13); Sequential Organ Failure Assestment (SOFA)-12, Glasgow Coma Score (GCS)-11. The patient underwent six sessions of MARS in Intensive Care Unit (ICU) in association with standard medical therapy (SMT). The patient survived and was discharged from the hospital in good condition after 40 days without liver transplantation (LT).

Toxic intracerebral demyelination in a case of suicidal Cypermethrin poisoning

A 15 years old boy was admitted with history of suicidal pyrethroid compound 'cypermethrin' consumption. Subacute evolution of central nervous system manifestations and radiologically documented subcortical demyelination were observed. Gradual and partial recovery could be achieved with conservative treatment over few weeks. Although generally considered safe pyrethrin compounds can occasionally result in devastating neurological consequence as observed in this case.

Cerebral vein and dural sinus thrombosis: an evaluation of 30 cases

Background and objectives: cerebral vein and dural sinus thrombosis (CVT) present a variety of non-specific clinical signs, but, due to the development of neuroimaging techniques, especially magnetic resonance imaging, their identification has increased in recent years, due to the development of neuroimaging techniques, especially of magnetic resonance imaging. The aim of our study was to identify causes and risk factors, to describe the demographic, clinical, laboratory and neuroimaging data, and to evaluate the treatment and outcome of patients with C.V.T., comparing our experience with international literature. Patients and methods: we included 30 patients (pts) with CVT, between January 1998 and September 2010. The diagnosis was based in all cases on neuroimaging features. The pts were examined at admission and after three months, using the mRS scores. Results: mean age was 37.2 years (SD 8.6), sex ratio: male/female was 1/2. 80% of women were fertile. The main clinical manifestations were headache: 22 pts (73.3%), and papilledema: 16 pts (53.3%). The most frequent neurological syndrome at onset was intracranial hypertension with subacute evolution. CT showed direct signs of dural sinuses thrombosis: (dense triangle sign in 3 pts, delta sign in 7 cases); in 12 pts, we identified a venous cerebral infarct (indirect sign of dural sinuses thrombosis). MRI identified thrombosis of SSS in 21 pts, transverse sinus in 12 cases, cavernous sinus in 3 pts, cerebral edema in 18 pts. In 12 cases, we identified a venous cerebral infarct. 7 out of 30 MRI had a normal CT. DSA revealed isolated cortical veins occlusion, without sinus occlusion in 2 cases (CT, MRI, and MR-A were normal). Risk factors were identified in 22 pts (73.3%); congenital thrombophilia being the most common (9 pts). All pts received anticoagulant therapy. After 90 days from admission, complete resolution of symptoms was seen in 14 cases, minimal neurological deficits in 7 pts, and the death rate was 16.7% (5 pts). Conclusions: CVT appear to be underdiagnosed in our region, due to low percentage of admissions for benign intracranial hypertension. CVT was common in women of fertile age, but oral contraceptive was not an important risk factor. The outcome was favorable with adequate therapy.

Autoimmune Limbic Encephalitis as an Emerging Pediatric Condition: Case Report and Review of the Literature

Limbic encephalitis, first described in the 1960s as a paraneoplastic condition, has emerged as an autoimmune condition, occurring often without evidence of an underlying tumor. Many novel autoantibodies have been identified, and this diagnosis is increasingly being made in the pediatric population. This article reports the case of a 16-year-old boy who presented following gastrointestinal illness with subacute evolution of neuropsychiatric symptoms. Brain magnetic resonance imaging revealed progressive hippocampal signal abnormality and swelling. N-methyl-D-aspartate (NMDA) receptor antibody was detected in serum. The patient responded well to pharmacological immunotherapy but has residual cognitive deficits. The available literature on this condition is reviewed. Limbic encephalitis should be considered in the differential diagnosis in children presenting with encephalopathy, particularly with neuropsychiatric manifestations. Long-term surveillance and close follow-up are required to accurately clarify tumor risk and natural history of this condition in children and balance these factors with risks of radiation exposure through imaging.

Research on the Use of Enzyme Proteolyitic Based Products in Salpingites, Peri and Parametritis Treatment in Cows

This paper aims to assess the opportunity of including in therapy protocols for localized puerperal affections with acute and subacute evolution in bovine species, of products containing proteolytic enzymes (alpha-chymotrypsin, trypsin and papain) in order to lyse the deposits of fibrin resulted from the performance of localized inflammatory processes. Research has been conducted on 180 cattle from three exploitations of intensive farming in the county Cluj-Napoca, diagnosed with localized puerperal disease (salpingitis, peri-and parametritis) with acute and subacute evolution. Protocol of administration of preparations containing proteolytic enzymes involves making three parenteral (IM) administrations, at 48 hours interval. Using proteolytic enzymes in the classical protocols of such therapy resulted in cure of disease, confirmed after transrectal ultrasound exam, followed by a gestation rate of 65% of cattle in the lot of research compared with a recovery of only 20% to the witness group.

Homer 3 autoimmunity in subacute idiopathic cerebellar ataxia

Few adult patients present with an irreversible subacute cerebellar ataxia of unknown cause.1,2 The subacute evolution and the presence of CSF pleocytosis suggest a possible immune-mediated pathogenesis. In this study, we characterize the antigen of a new anti-Purkinje cell antibody identified in a patient with idiopathic subacute cerebellar ataxia.1,2 A 65-year-old woman presented in October 2000 with a week history of vertigo and vomiting. She denied symptoms of previous infectious disease. Neurologic examination revealed dysarthria, horizontal nystagmus, and severe limb and gait ataxia. Routine blood analysis, thyroid hormones, antineuronal antibodies (Hu, Yo, Ri, Ma1 and 2, CV2, Tr, GAD, and amphiphysin), brain MRI, colonoscopy, esophagogastroscopy, and CT of the chest and abdomen were normal or negative. CSF showed 27 lymphocytes/mm3 with a CSF IgG index of 1.4 (n < 0.7). She did not improve after treatment with steroids. At the last evaluation in June 2006, the cerebellar syndrome was unchanged with no cancer clinically evident. Immunohistochemistry was performed as described.3 Cerebellum samples from normal rats or mice and Homer 3–deficient mice (gift of Dr. Paul F. Worley) were subjected to standard immunoblot procedures.3 …

Subacute progression of human T-lymphotropic virus type I-associated myelopathy/tropical spastic paraparesis

Although human T-lymphotropic virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is usually described as a chronic disabling disease, a rapid course over months or even weeks has been reported in some patients. The authors describe the clinical features of HAM/TSP in a Brazilian cohort and evaluate the prevalence of patients with a subacute progression of the disease. This was defined as the requirement of a wheelchair during the first 2 years after the onset of symptoms. Patients with this subacute course and patients with the chronic clinical course were compared in terms of their HTLV-I proviral loads (PLs) using real-time polymerase chain reaction (PCR). Seven out of 88 patients (7.9%) had a subacute progression. All patients were women and 5/7 acquired HTLV-I through sexual contact. There was no significant difference in the real-time PLs between the group with subacute evolution (mean 8.5 copies/100 cells, range 6.03 to 12.09) and those patients with a typical course of disease (mean 11.34 copies/100 cells, range 0.4 to 67.72) (P = .68), suggesting that factors other than the number of infected cells are implicated in the development of such an aggressive course of disease. Early recognition of this subgroup is important because immunosuppressive treatment might be beneficial if instituted promptly.

Síndrome de Ogilvie o pseudobstrucción aguda del colon: una causa rara de abdomen agudo, de elevada mortalidad y asociada a patologías frecuentes en un Servicio de Medicina Interna

The acute pseudobstruction of the colon or Ogilvie's syndrome is a infrequent pathology of acute or subacute evolution, that is associated to different clinical or surgical pathologies, and whose pathophysiology continues to be unknown. The treatment begins with general measures and if these are not effective, with the surgical decompression of colon to avoid the most serious complication, and frequently that is perforation of the cecum. We present a case of an acute and idiopathic dilatation of colon in an adult man who entered by an episode of acute respiratory insufficiency, and that was solved with preservative medical treatment.

Bartonella quintana endocarditis in east Africa

Bartonella quintana endocarditis is characterized by sub-acute evolution and severe valvular damage, and is associated with homelessness, alcoholism, and lice infestation. We present a case of B. quintana endocarditis in an Ethiopian immigrant without known risk factors for disease acquisition. This is the first case of B. quintana endocarditis reported from east Africa.

Outcome and treatment of Bartonella endocarditis.

Endocarditis caused by Bartonella species is a potentially lethal infection characterized by a subacute evolution and severe valvular lesions. To evaluate the outcome of patients with Bartonella endocarditis and to define the best antibiotic regimen using the following measures: recovery, relapse, or death. We performed a retrospective study on 101 patients who were diagnosed in our laboratory as having Bartonella endocarditis between January 1, 1995, and April 30, 2001. Bartonella infection was diagnosed using immunofluorescence with a 1:800 cutoff, polymerase chain reaction amplification of DNA, and/or culture findings of Bartonella species from whole blood, serum, and/or valvular biopsy specimens. A standardized questionnaire was completed by investigators for each patient. Twelve of the 101 patients died and 2 relapsed. Patients receiving an aminoglycoside were more likely to fully recover (P =.02), and those treated with aminoglycosides for at least 14 days were more likely to survive than those with shorter therapy duration (P =.02). Effective antibiotic therapy for Bartonella endocarditis should include an aminoglycoside prescribed for a minimum of 2 weeks.

Thalamic tumors: clinical presentation.

Thalamic tumors (TTs) account for approximately 1% of all intracranial neoplasms. TTs are seen predominantly in children and young adults. Most childhood neoplasms in this location are of glial lineage, a large proportion being low-grade tumors. The evolution of symptoms before diagnosis is characteristically shorter in children than in adults. Clinical features of TTs reflect the pressure of the mass on the cerebrospinal fluid pathways, the pyramidal tracts, the thalamic nuclei and the optic radiations. In this paper, we review the clinical data of 20 children with TTs seen at our hospital and compare the findings with those in the current literature. An acute presentation is not rare and was encountered in 20% of our patients, although most individuals had a subacute or slow evolution. Symptoms and signs of raised intracranial pressure (65%), motor deficits (40%), and seizures (35%), alone or in combination, were the most frequent manifestations of TTs. Behavioral and mental changes are not uncommon in TTs and were found in 25% of our patients. Involuntary movements (10%) and the classic thalamic syndrome (5%) were quite exceptional. Infants and young children with TTs may present with macrocephaly, psychomotor delay, and failing vision or disorders of ocular movements.

Changes in diffusion-weighted MRI after status epilepticus

A 3-year-old male developed hemiplegia and aphasia after convulsive status epilepticus. Diffusion-weighted magnetic resonance images demonstrated cytotoxic edema in the white matter 6 days after the seizure episode and subsequently in the gray matter after an additional 7 days. Diffusion-weighted magnetic resonance images demonstrated a subacute evolution of the pathologic process after the status epilepticus.

Liver rupture after cardiopulmonary resuscitation in a patient receiving thrombolytic therapy

A patient with an acute myocardial infarction had to be resuscitated due to recurring ventricular fibrillation. After stabilisation, she received thrombolytic treatment with anistreplase. Fourteen hours later, clinical signs of hemoperitoneum developed and the diagnosis of liver rupture was made. After abdominal surgery and coronary revascularisation, the subsequent clinical course was uncomplicated. The literature on liver trauma after CPR as well as thrombolysis in association with CPR is reviewed. This observation suggests that there are no convincing arguments to withhold thrombolytic therapy in acute myocardial infarction after CPR in the absence of gross trauma. We stress that a high degree of clinical alertness is necessary to diagnose this life-threatening condition because of its frequent subacute evolution in patients that are per se hemodynamically unstable.

Cavitating Pulmonary Nodules Complicating Hodgkin's Disease

Dr. Hatfield: This 64-year-old man with a six-year history of Hodgkin's disease complained of pain in the right side of the chest of three days' duration. For six years he had received high doses of steroids, which had induced myopathy in his legs. He also had severe coronary artery disease with at least one myocardial infarct, angina, atrial fibrillation, and recurrent congestive failure. He first received melphalan (Alkeran) two years previously when he was admitted to the hospital with pain in the right side of the chest and lowgrade fever. Results of physical examination were unremarkable, except for a grade 2/6 apical systolic murmur and palpable right inguinal nodes. Roentgenograms of the chest (Fig 1 and 2) demonstrate the subacute evolution of nodular masses within both lungs, with eventual cavitation. Between these two examinations, the patient received 1,000 rads in one week to both lungs. There was no discernible response

Cavitating pulmonary nodules complicating Hodgkin's disease

Dr. Hatfield: This 64-year-old man with a six-year history of Hodgkin's disease complained of pain in the right side of the chest of three days' duration. For six years he had received high doses of steroids, which had induced myopathy in his legs. He also had severe coronary artery disease with at least one myocardial infarct, angina, atrial fibrillation, and recurrent congestive failure. He first received melphalan (Alkeran) two years previously when he was admitted to the hospital with pain in the right side of the chest and lowgrade fever. Results of physical examination were unremarkable, except for a grade 2/6 apical systolic murmur and palpable right inguinal nodes. Roentgenograms of the chest (Fig 1 and 2) demonstrate the subacute evolution of nodular masses within both lungs, with eventual cavitation. Between these two examinations, the patient received 1,000 rads in one week to both lungs. There was no discernible response