Onset Of Neurological Signs Research Articles

Head tilt as a clinical sign of cervical spinal or paraspinal disease in dogs: 15 cases (2000-2021).

To characterise head tilt as a rare clinical sign of cervical spinal or paraspinal disease in dogs. Retrospective single-centre case-series study of dogs with head tilt and cervical spinal or paraspinal disease in the absence of intracranial abnormalities. Descriptive statistics were used. Fifteen dogs met the inclusion criteria of this study. Median age at onset was 6 years (range 2.5 to 12 years). Onset of neurological signs was mainly chronic (9/15, 60%). Most common presenting complaints included head tilt (9/15, 60%) and cervical hyperaesthesia (8/15, 53%). Most common neurological findings included head tilt (15/15, 100%), generalised proprioceptive ataxia and tetraparesis (6/15, 40%) and cervical hyperaesthesia (8/15, 53%). Diagnoses included post-operative complication of C2 spinal nerve root mass removal (2/15, 13%), C3-C4 intervertebral disc extrusion (2/15, 13%), cervical paraspinal myositis (2/15, 13%) and one of each: C2 vertebral malformation, C2 spinal nerve root mass, C1-C2 meningioma, C2 vertebral fracture, C4-C5 intervertebral disc extrusion, C4 vertebral body mass, C5-C7 osseous-associated cervical spondylomyelopathy, and concurrent C5-C6 and C6-C7 intervertebral disc protrusions. Two dogs were euthanased shortly after diagnosis and two of 15 were dogs lost to follow-up. No post-mortem examination was performed for these cases. For the 11 of 15 remaining dogs, head tilt resolved in eight of 15 (53%) dogs after treatment of the underlying condition and in three of 15 (20%) dogs, it remained static. Head tilt can be a rare clinical sign of cervical spinal or paraspinal disease in dogs.

Central nervous system syndrome associated with Marek’s disease in Hubbard broilers

Marek’s disease (MD) has a high economic impact in poultry production worldwide. MD consists of several pathologic syndromes of which the most commonly encountered are lymphoproliferative syndromes such as acute MD. Sporadically, MD leads to central nervous system syndromes characterized by non-neoplastic brain pathology. In this case report, an onset of neurological signs on two different broiler farms, housing ‘slow-growing’ Hubbard JA 757 broilers, is described. On the first farm, clinical signs were observed from 42 days of age onwards and on the second farm, respectively from 42 (stable 1) and 30 days (stable 2) of age onwards. The neurological symptoms consisted of acute central nervous system signs, which started with flaccid neck paralysis. Histopathological examination revealed multifocal perivascular cuffing of CD3 positive mononuclear cells in the brain tissues. These findings pointed towards the development of transient paralysis. qPCR analysis confirmed the diagnosis. In broiler flocks, which only have a limited life span, vaccination is not common practice. In flocks of slow-growing breeds, outbreaks of MD might become more important again. Therefore, vaccination of slow-growing broilers against MD seems recommended.

Does surgical timing affect the rapidity of recovery in deep pain-entire non-ambulatory dogs with thoracolumbar intervertebral disk extrusion?

To investigate whether the delay between onset of neurological signs and spinal cord decompression affects the time to recovery in non-ambulatory paraparetic/paraplegic dogs with deep pain perception affected by thoracolumbar intervertebral disc extrusion. Data on non-ambulatory dogs with preserved deep pain perception in both hindlimbs and surgically treated for thoracolumbar intervertebral disc extrusion were prospectively collected from three referral hospitals. Cox proportional hazards regression was used to explore the relationship of time until restoration of independent ambulation with age, weight, preoperative use of anti-inflammatory drugs, delay between onset of inability to walk and arrival at the clinic, time between presentation and spinal surgery and surgery time. One hundred and fifty-one dogs were included. Median time from admission to surgery, including imaging, was 180 (interquartile range, 65.4 to 240) minutes. All dogs were operated within 72 hours. Follow-up was available for all 151 dogs and ranged from 1 to 21 months. All but three dogs, which were all grade IV at presentation, recovered the ability to ambulate. In both univariable and multivariable models, only duration of surgery and neurological (Frankel) grade at presentation were significantly associated with the rapidity of recovery of ambulation. Delay between onset of clinical signs and presentation and time between presentation and spinal surgery was not associated with the rapidity of recovery of ambulation in dogs surgically treated for thoracolumbar intervertebral disc extrusion. These results should be evaluated in the context of a limited range of delay since only 14.5% dogs had a delay ≥6.5hours. Duration of surgery and neurological grade at presentation were significantly associated with rapidity of recovery of ambulation.

Prevalence and Clinicopathologic Features of Canine Metastatic Melanoma Involving the Central Nervous System: A Retrospective Analysis and Comparative Review.

BackgroundCentral nervous system (CNS) involvement is the leading cause of death in malignant melanoma. Rodent models, while vital to mechanistic investigation, have had limited success identifying effective therapies for melanoma brain metastases. The companion dog with de novo melanoma is a promising complementary model for developmental therapeutic investigation, as these tumors occur in an immunologically outbred host that has shared environmental exposures with humans. However, relatively little is known regarding the prevalence and clinicopathological features of canine melanoma metastasis to the CNS. To further validate the dog as an appropriate model for human metastatic melanoma, the aims of this study were to determine the rate of CNS metastasis and associated clinicopathologic features in canine malignant melanoma.MethodsMedical records of dogs diagnosed with malignant melanoma from 1985-2019 at the University of California Davis Veterinary Medical Teaching Hospital were assessed retrospectively. Clinicopathologic features were compared between dogs with CNS metastasis (CNS+) and dogs without CNS metastasis (CNS-). Site of CNS involvement and associated neurological signs were analyzed via Wilcoxon-Mann-Whitney rank sum and Fisher’s exact tests. Survival data were analyzed via Kaplan-Meier estimates.ResultsCNS metastasis was identified in 38% of dogs in this study (20/53). The oral cavity was the most common site of primary melanoma in both groups [CNS+: n=12 (60%) vs. CNS-: n=22 (67%); p>0.99]. The total burden of metastatic disease was higher in the CNS+ group (CNS+: 4, 95% CI 3-5 vs. CNS-: 3, 95% CI 1-3; p<0.001). The cerebrum was the most common site of CNS metastasis (n=15, 75%) and seizures were the most observed neurological sign (n=9, 64%). There was no difference in overall survival between CNS+ and CNS- groups. However, the median survival time following onset of neurological signs was 9.5 days (95% CI 1-43), with 5 dogs euthanized within 24 hours of the onset of neurological signs.ConclusionsCanine and human MM patients share similar rates of CNS metastasis and clinical presentation. This study will guide clinical management of canines with malignant melanoma and inform future studies using dogs with spontaneously occurring melanoma as a preclinical model for human melanoma brain metastases.

Neuroimaging findings in hospitalized patients with COVID-19

BackgroundVariable neuroimaging findings have been reported in patients with coronavirus disease 2019 (COVID-19). In addition to respiratory symptoms, many neurologic manifestations of COVID-19 are increasingly reported and variable neuroimaging findings have been observed in patients with COVID-19. Our aim was to describe findings observed in hospitalized patients with COVID-19, presenting with acute neurologic manifestations and undergoing computed tomography (CT) or magnetic resonance imaging (MRI) of the brain.MethodsWe performed a retrospective study involving patients with laboratory-confirmed SARS-COV-2 infection, admitted to our hospital between July 1 and December 30, 2020. Patients who presented with acute neurologic symptoms and required neuroimaging were only included in the study. Neuroimaging examinations were evaluated for the presence of, infarction, hemorrhage and encephalopathy. The frequency of these findings was correlated with clinical variables, including presence of comorbidities, requirement for intensive care unit admission, and duration between admission and onset of neurologic signs and symptoms as documented in the hospital medical records.ResultsA total of 135 patients underwent at least one cross-sectional imaging of the brain, the median age of these patients was 63 years, and 72% were men. Disturbed level of consciousness was the most common neurologic symptom (80.7%). Acute neuroimaging findings were found in 34 patients (25.2%) including; acute ischemic infarcts (16/135; 11.9%), intracranial hemorrhages (9/135, 6.7%), cerebral venous thrombosis (2/135; 1.5%), posterior reversible encephalopathy syndrome (1/135; 0.7%), and hypoxic-ischemic encephalopathy (6/135, 4.4%). There was no statistically significant difference in patient age (p = 0.062), sex (0.257), presence of comorbidities (p = 0.204), intensive care unit admission (p = 0.326) and duration between admission and onset of neurologic signs and symptoms (p = 0.755), in patients with positive versus negative neuroimaging studies.ConclusionsOur study showed that cerebrovascular complications, ischemic and hemorrhagic were the most frequent imaging finding in hospitalized patients with COVID-19. Knowledge about these potentially serious complications can help optimize management for these patients.

Canine Tick-Borne Encephalitis: Clinical Features, Survival Rate and Neurological Sequelae: A Retrospective Study of 54 Cases (1999-2016).

Tick-borne encephalitis (TBE) is one of the most important infectious diseases of the central nervous system in dogs from endemic areas. While in humans survival rate and long-term outcomes are well described, these data are lacking in veterinary literature. The aim of the present paper is to characterize the clinical aspects of TBE and to investigate fatality rate, long-term outcome and the long-term neurological sequelae in a population of dogs infected with TBE. We performed a retrospective analysis of 54 dogs diagnosed with TBE at the veterinary hospital of the University of Zurich between 1999 and 2016. Medical data such as signalment, clinical presentation, results of diagnostic procedures, treatment and outcome were collected and analyzed. Statistical analysis including a cox proportional hazard model using a backward stepwise regression approach was performed. In 62% of the TBE cases unspecific signs were described before the onset of neurological signs, resembling a biphasic appearance that is well known in human TBE. Case fatality rate was 33% and all dogs died within the first 4 months after diagnosis. Long-term neurological sequalae were detected in 17% of the TBE cases. For each day of clinical signs before hospital entry the odds of sequalae increased by a factor of 1.88 (CI 1.04–3.15). Older dogs and dogs presented with seizure activity had an increased hazard risk of death (Hazard ration = 1.2, p = 0.03; and 9.38, p = 0.001, respectively). In conclusion, despite TBE being a life-threatening disease with severe clinical signs, the survival rate in our study was 67%. However, long-term sequalae can be of concern especially in dogs with longer clinical course.

IFN-γ is a therapeutic target in paraneoplastic cerebellar degeneration.

Paraneoplastic neurological disorders result from an autoimmune response against neural self-antigens that are ectopically expressed in neoplastic cells. In paraneoplastic disorders associated to autoantibodies against intracellular proteins, such as paraneoplastic cerebellar degeneration (PCD), current data point to a major role of cell-mediated immunity. In an animal model, in which a neo-self-antigen was expressed in both Purkinje neurons and implanted breast tumor cells, immune checkpoint blockade led to complete tumor control at the expense of cerebellum infiltration by T cells and Purkinje neuron loss, thereby mimicking PCD. Here, we identify 2 potential therapeutic targets expressed by cerebellum-infiltrating T cells in this model, namely α4 integrin and IFN-γ. Mice with PCD were treated with anti-α4 integrin antibodies or neutralizing anti-IFN-γ antibodies at the onset of neurological signs. Although blocking α4 integrin had little or no impact on disease development, treatment using the anti-IFN-γ antibody led to almost complete protection from PCD. These findings strongly suggest that the production of IFN-γ by cerebellum-invading T cells plays a major role in Purkinje neuron death. Our successful preclinical use of neutralizing anti-IFN-γ antibody for the treatment of PCD offers a potentially new therapeutic opportunity for cancer patients at the onset of paraneoplastic neurological disorders.

The Grisel's syndrome: A non-traumatic subluxation of the atlantoaxial joint

IntroductionGrisel's syndrome consists in rotational subluxation of C1–C2 following ENT infection or surgery. There is no consensus on management. We present 2 cases requiring surgical treatment in our center. Case reportsTwo 10-year-old patients presented torticollis with cervical pain resistant to medical treatment, with onset a few months after tonsillectomy. In both cases, radiological assessment, comprising CT scan and MRI, showed Fielding–Hawkins type-3 C1–C2 rotational subluxation, without ligament lesion. After failure of conservative treatment, posterior reaming, realignment, C1–C2 arthrodesis using lateral masses and pars interarticularis screws and bone graft achieved good fusion and immediate spinal stability in all planes of the atlantoaxial complex. DiscussionGrisel's syndrome consists in non-traumatic subluxation of the atlantoaxial joint with intact atlantoaxial ligaments. Initial pharyngeal inflammation spreads to the prevertebral fascia via direct connections between the periodontoidal venous plexus and pharyngovertebral veins, inducing fasciitis that leads to abnormal relaxation of the atlantoaxial ligaments and reactional muscle contraction with ankylosis. This phenomenon, appearing gradually and insidiously over a period of a few weeks, creates a frozen joint with ankylosis. Medical treatment with NSAIDs, muscle relaxants, and immobilization is usually sufficient; cervical traction may be needed. Surgical treatment by C1–C2 arthrodesis is indicated in case of failure of medical management or onset of neurologic signs. ConclusionClose collaboration between pediatricians, ENT surgeons and neurosurgeons is essential for early diagnosis and management, which is the main prognostic factor for successful medical treatment, avoiding surgery.

Canine primary encephalopathies: a retrospective study of 48 cases (2008-2012)

A retrospective study was performed on 48 dogs with primary encephalopathies, admitted during a 5-year period (2008-2012). Their age ranged from 2 months to 19 years, the majority being older than 10 years. Genders were almost equally distributed and most animals were purebreds or crossbreds. Onset of neurological signs was acute (11/48), subacute (12/48) or chronic (24/48). Lesion localization was focal in 31 and multifocal in 17 dogs. The cerebrum and brainstem were the most common focal localizations, while cerebellar lesions were recorded only in 4 dogs. An aetiologic diagnosis was established in 29 dogs and included primary (8/29) or metastatic neoplasia (2/29), encephalitis of variable etiology (9/29), congenital or inherited diseases (6/29), senile cognitive dysfunction (3/29) and ischaemic encephalopathy (1/29). In the remaining 19 dogs, aetiology of intracranial dysfunction was not determined; however a tentative diagnosis was speculated in eight of these cases and included breed specific and viral encephalitis, neoplasia, ischaemic encephalopathy and inherited disease. Most dogs were euthanized due to the debilitating neurological signs (24/48) or died from complications of their illness (13/48). Six animals are still alive with symptomatic medical treatment and supportive nursing care and 5 were lost to follow up.

Colite necrohemorrágica causada por Entamoeba histolytica em um cão

Background: Intestinal amebiasis with morphological lesions and clinical manifestations is uncommon in dogs. The disease is caused by the protozoan Entamoeba histolytica, which is commonly observed in its natural hosts, humans and some non-human primates. It is occasionally found in the company of animals, usually associated with contact with infected humans. Thus, the objective here is to describe a case of necro-hemorrhagic colitis caused by E. histolytica in a dog infected with the canine distemper virus, in order to characterize the epidemiological and clinicopathological aspects of the disease.Case: An adult, mixed-breed bitch displaying anorexia and ataxia was referred to the veterinary hospital for treatment. Clinical evaluation showed a cachectic animal with 12% dehydration, ocular discharge, and bilateral purulent nasal dis-charge. A clinical diagnosis of distemper was made, and treatment was instituted. The dog’s signs progressed to walking in circles, aimlessly, with lethargy and blindness. After three days of the onset of neurological signs, the dog developed diarrhea with hematochezia. With no improvement noted, we elected to euthanize the dog. At necropsy, edema was present in subcutaneous tissues, and the lungs had yellow areas in the cranio-ventral portions, which the court was flowing purulent discharge. In the large intestine, segmental distention of the distal portion of the descending colon was observed. The segment was approximately 15 cm in length and consisted of irregular reddish areas. There was also slight thickening of the wall with edematous mucosa containing blood clots, fibrin, and multiple areas of ulceration. Microscopically showed necro-hemorrhagic colitis associated with rounded structures, approximately 15 μm in size, containing abundant eosinophilic cytoplasm that was slightly granular or vacuolated. They also contained nuclei and nucleoli that were central or slightly eccentric. These organisms were consistent with amoeba trophozoites. There was also demyelinating encephalitis associated with malacia, corpuscular intranuclear eosinophilic inclusions and / or intracytoplasmic inclusions in ependymal cells, astrocytes, and gemistocytes, characteristic of infection with canine distemper virus. Using immunohistochemistry with polyclonal anti-E. histolytica antibodies in the dilution of 1:1000, trophozoites were immunomarked, confirming the suspected amebiasis.Discussion: The diagnosis of intestinal amebiasis was based on clinical signs and by morphological characteristics on gross and microscopic examination, and was confirmed as E. histolytica by immunohistochemistry. Limited information on theepidemiology and pathological findings of infection with Entamoeba sp. has been reported in the literature, as it is relatively uncommon in pets. Affected animals are usually asymptomatic, but immunosuppression caused by canine distemper virus may have triggered the clinical manifestations of the disease in this dog. Enteritis due Entamoeba sp. should be considered in dogs with chronic weight loss and bloody diarrhea. It should also be included in the differential diagnoses for weight loss and hemorrhagic gastroenteritis, such as canine parvovirus, canine adenovirus 1, Pythium insidiosum, and Giardia sp.

Clinical presentation, diagnostic findings and outcome in dogs diagnosed withpresumptive spinal-only meningoen-cephalomyelitis of unknown origin.

ObjectivesTo summarise clinical presentation, diagnostic findings and long‐term outcome for dogs clinically diagnosed with meningoencephalomyelitis of unknown origin affecting the spinal cord alone.MethodsMedical records were reviewed for dogs diagnosed with presumptive spinal‐only meningoencephalomyelitis of unknown origin between 2006 and 2015.Results21 dogs were included; the majority presented with an acute (43%) or chronic (52%) onset of neurological signs. Ambulatory paresis was the most common neurological presentation (67%). Neurological examination most commonly revealed a T3‐L3 myelopathy, and spinal hyperaesthesia was a common finding (71%). A spinal cord lesion was visible in 90% of cases on magnetic resonance imaging. Eighteen lesions (86%) showed parenchymal contrast enhancement and 17 lesions (81%) showed contrast enhancement of overlying meninges. All dogs were treated with immunosuppressive doses of glucocorticosteroids, sometimes combined with cytosine arabinoside. At time of data capture, 10/21 dogs (48%) had died or been euthanased because of the condition. Overall median survival time was 669 days.Clinical SignificanceMeningoencephalomyelitis of unknown origin should be considered in the differential diagnosis of dogs presenting with a progressive myelopathy. Magnetic resonance imaging features can possibly help to distinguish presumptive meningoencephalomyelitis of unknown origin from other more common spinal diseases. Overall, long‐term survival is guarded, approximately 50% of dogs will die or be euthanased despite appropriate therapy.

Spontaneous ischaemic stroke lesions in a dog brain: neuropathological characterisation and comparison to human ischaemic stroke

BackgroundDogs develop spontaneous ischaemic stroke with a clinical picture closely resembling human ischaemic stroke patients. Animal stroke models have been developed, but it has proved difficult to translate results obtained from such models into successful therapeutic strategies in human stroke patients. In order to face this apparent translational gap within stroke research, dogs with ischaemic stroke constitute an opportunity to study the neuropathology of ischaemic stroke in an animal species.Case presentationA 7 years and 8 months old female neutered Rottweiler dog suffered a middle cerebral artery infarct and was euthanized 3 days after onset of neurological signs. The brain was subjected to histopathology and immunohistochemistry. Neuropathological changes were characterised by a pan-necrotic infarct surrounded by peri-infarct injured neurons and reactive microglia/macrophages and astrocytes.ConclusionsThe neuropathological changes reported in the present study were similar to findings in human patients with ischaemic stroke. The dog with spontaneous ischaemic stroke is of interest as a complementary spontaneous animal model for further neuropathological studies.

Guillain-Barré Syndrome During Ongoing Zika Virus Transmission - Puerto Rico, January 1-July 31, 2016.

Guillain-Barré syndrome (GBS) is a postinfectious autoimmune disorder characterized by bilateral flaccid limb weakness attributable to peripheral nerve damage (1). Increased GBS incidence has been reported in countries with local transmission of Zika virus, a flavivirus transmitted primarily by certain Aedes species mosquitoes (2). In Puerto Rico, three arthropod-borne viruses (arboviruses) are currently circulating: Zika, dengue, and chikungunya. The first locally acquired Zika virus infection in Puerto Rico was reported in December 2015 (3). In February 2016, the Puerto Rico Department of Health (PRDH), with assistance from CDC, implemented the GBS Passive Surveillance System (GBPSS) to identify new cases of suspected GBS (4). Fifty-six suspected cases of GBS with onset of neurologic signs during January 1-July 31, 2016, were identified. Thirty-four (61%) patients had evidence of Zika virus or flavivirus infection; the median age of these patients was 55 years (range=21-88 years), and 20 (59%) patients were female. These 34 patients were residents of seven of eight PRDH public health regions. All 34 patients were hospitalized and treated with intravenous immunoglobulin G (IVIg), the standard treatment for GBS; 21 (62%) required intensive care unit admission, including 12 (35%) who required endotracheal intubation and mechanical ventilation. One patient died of septic shock after treatment for GBS. Additionally, 26 cases of neurologic conditions other than GBS were reported through GBPSS, including seven (27%) in patients with evidence of Zika virus or flavivirus infection. Residents of and travelers to Puerto Rico and countries with active Zika virus transmission should follow recommendations for prevention of Zika virus infections.* Persons with signs or symptoms consistent with GBS should promptly seek medical attention. Health care providers in areas with ongoing local transmission seeing patients with neurologic illnesses should consider GBS and report suspected cases to public health authorities.

Variable Infection Dynamics in Four Peromyscus Species Following Experimental Inoculation with Baylisascaris procyonis.

Wild rodents such as Peromyscus spp. are intermediate hosts for the zoonotic ascarid Baylisascaris procyonis (raccoon roundworm), and previous studies indicate Peromyscus leucopus (white-footed mouse) likely serves an important role in parasite ecology. Natural infections have been sporadically identified in a few Peromyscus spp., but no data are available on differences in susceptibility among the many other species. We compared survival and infection dynamics of B. procyonis in 4 species ( P. leucopus , Peromyscus maniculatus [deer mouse], Peromyscus californicus [California mouse], Peromyscus polionotus [Oldfield mouse]) from regions of varying habitat types as well as B. procyonis prevalence in raccoons. Six captive-bred mice of each species were inoculated per os with 1 of 3 biologically-relevant doses of embryonated B. procyonis eggs (∼10, ∼50, or ∼500). Animals were monitored twice daily for clinical signs and behavioral abnormalities and were euthanized at the onset of neurological signs or extensive (≥20%) weight loss, or at 45 days post-infection if no disease developed. Larvae were counted in the brain via microscopic examination and in skeletal muscle and visceral organs via artificial digestion. In the high-dose group, all but 1 mouse developed severe neurologic disease and were euthanized. In the medium-dose group, survival was variable and ranged from 33-85% across species. Little to no disease was observed in the low-dose group, although 1 P. maniculatus developed disease and was euthanized. Survival analysis reveals P. leucopus had a longer time until clinical disease onset versus the other species, which did not differ significantly from each other. Interestingly, larval recovery relative to dose was nearly identical across species and doses; however, larvae were differentially distributed in skeletal muscle, visceral organs, and brain among species. These data indicate that P. leucopus may be more resilient toward severe baylisascariasis compared to the other species and that even closely-related rodents may experience differential mortality. This variation in tolerance may have ecological implications for the different species as B. procyonis intermediate hosts, although more work is needed to put these experimental findings into context.

Dengue virus induces apoptosis in SH-SY5Y human neuroblastoma cells.

Dengue is a human disease caused by a virus with the same name, which is transmitted by the bite of Aedes mosquitoes. The infection has a wide range of clinical presentations ranging from asymptomatic to fatal cases, with the pediatric population being the most susceptible. According to the new classification of the disease, the neurological manifestations are considered a criterion for the diagnosis of severe dengue. To evaluate the possible mechanisms involved in the onset of neurological signs in a cell line of human neurons as a model of infection with dengue virus type 2 (DENV-2). Susceptibility and permissiveness of the SH-SY5Y line to infection by DENV-2 was analyzed, showing that the proportions of viral infection and production are similar to those of primate cells used as positive control for infection. Infection induced a cytopathic effect on the neuroblastoma line characterized by apoptotic cell death process, increasing the proportion of annexin V and TUNEL positive cells and an upregulation of TNF-α. Treatment with anti-TNF-α antibody increased slightly cell survival of infected cells. The addition of exogenous TNF-α to the infected cultures enhanced cell death. These results as a whole suggest that the upregulation of TNF-α could be part of the process that induces cell damage and death in cases of dengue encephalitis.

Acute Quadriplegia Caused by Idiopathic Spontaneous Epidural Haematoma in Young Adult-Case Report and Review of Literature

Introduction: Authors tried to emphasize the importance of diagnosing and rapidly treating spontaneous spinal epidural hematoma (SSEH), a rare idiopathic condition which if not recognized early can have catastrophic consequences. The definition and pathophysiology of this condition remains controversial. Usual management consists of urgent MRI imaging followed by immediate surgical decompression. Clinical presentation: A 22 year young adult presented with the symptoms of sudden severe chest pain, followed by weakness and numbness over the all four extremities, progressing to intense paraparesis, anesthesia and consecutively quadriplegia and sphincter control loss. MR imaging demonstrated acute epidural hematoma of cervico-thoracic spinal segments (C7-Th2) with cord compression. Idiopathic SSEH was diagnosed and prompt decompressive laminectomy with hematoma evacuation was performed. During the operation cause of bleeding was not identified. Postoperatively CTA and DSA were performed which came back negative. After 2 weeks, there was no residual weakness/parasthaesias, sphincter control was fully obtained and full motor power was required. Discussion: SSEH in its true idiopathic form is a rare pathologic entity which can be disabling or fatal. According to the literature, most patients present with severe back and/or neck pain, often with a radicular component, followed by motor and/or sensory deficits which are symptoms to be aware of. Prompt diagnosis should be made with MRI, and evacuation of the hematoma should be immediate, ideally before the onset of neurologic signs or symptoms. As evidenced outcome depends on prompt operation timing and prognosis is impacted by age and preoperative deficit. Because of the high risk of poor outcome without treatment, SSEH should always be a diagnostic consideration in patients whose presentation is even slightly suggestive. Rapid, appropriate surgical treatment can often lead to complete recovery of function, whereas any delay in appropriate treatment can be disastrous. The role of conservative management needs to be proven and should be tailored on an individual basis. Conclusion: SSEH is a rare and potentially fatal disease. Prompt diagnosis and surgical treatment is required to obtain good outcome results. Physicians should be aware of the early symptoms so the best treatment can be achieved.

Foodborne-Transmitted Prions From the Brain of Cows With Bovine Spongiform Encephalopathy Ascend in Afferent Neurons to the Simian Central Nervous System and Spread to Tonsils and Spleen at a Late Stage of the Incubation Period.

Protease-resistant prion protein (PrP(res)) accumulation in lymphoreticular tissues indicates prion infection. To date, tonsillectomy and appendectomy samples have been used in population prevalence surveys to detect clinically silent carriers of variant Creutzfeldt-Jakob disease (vCJD). However, the temporal sequence of prion spread in the human body is still not known. We therefore traced the temporal-spatial pattern of PrP(res) accumulation in the body of a simian vCJD model. Cynomolgus monkeys were fed brain of (eleven) cows with bovine spongiform encephalopathy, and some were euthanized before and some after onset of neurological signs. PrP(res) was detected in tissues by a paraffin-embedded tissue blot technique and a semiquantitative Western immunoblot assay. Bovine spongiform encephalopathy (BSE)-associated prions were preferentially transported from the gut to the central nervous system (CNS) along sensory nerve fibers and initially entered the simian CNS at lumbar spinal cord levels. In asymptomatic animals, we found BSE in 50% and 12% of gut- and tonsil-derived samples, respectively. Unlike in rodents and ruminants, foodborne BSE-associated prions entered the simian CNS via afferent neurons. From sites of initial CNS invasion, prions spread centrifugally to tonsils and spleen at an advanced stage of the incubation period, thus explaining why tonsil specimens were not reliable for detection of simian disease carriers before onset of clinical signs.

Toltrazuril does not show an effect against pigeon protozoal encephalitis.

The protozoan parasite Sarcocystis calchasi causes a severe neurologic disease in domestic pigeons (Columba livia f. dom.) named pigeon protozoal encephalitis. Recently, the parasite has also been reported in psittacines causing a virtually identical disease with fatal outcome. So far, an etiological treatment of S. calchasi infections in pigeons or psittacines is unknown. The present study evaluates the effectiveness of the anticoccidian drug toltrazuril against S. calchasi and the influence of the timepoint of treatment. Therefore, nine domestic pigeons were inoculated with 400 S. calchasi sporocysts and treated with toltrazuril (25 mg/kg) in groups of three pigeons each at dpi 10/11 and dpi 40/41 and on two consecutive days at the onset of neurologic signs. After euthanasia at dpi 73, tissue samples including brain and skeletal muscles were examined by histology and S. calchasi-specific real-time PCR. All pigeons independent of the group developed neurologic signs from dpi 49 onwards. Histology identified sarcocysts in the skeletal muscles and a granulomatous encephalitis in the brains. The relative amount of S. calchasi DNA was on a comparable level in all pigeons. Consequently, toltrazuril was demonstrated to be not effective against S. calchasi with the applied treatment regime. Longer treatment periods or agents other the toltrazuril may be considered for further investigations. So far, preventive measures like roofing of aviaries for prevention of infection and regular disinfection remain the most important factor in the control of S. calchasi infections.

Recent advances in the diagnosis and treatment of niemann-pick disease type C in children: a guide to early diagnosis for the general pediatrician.

Niemann-Pick disease (NP-C) is a lysosomal storage disease in which impaired intracellular lipid transport leads to accumulation of cholesterol and glycosphingolipids in various neurovisceral tissues. It is an autosomal recessive disorder, caused by mutations in the NPC1 or NPC2 genes. The clinical spectrum is grouped by the age of onset and onset of neurological manifestation: pre/perinatal; early infantile; late infantile; and juvenile periods. The NP-C Suspicion Index (SI) screening tool was developed to identify suspected patients with this disease. It is especially good at recognizing the disease in patients older than four years of age. Biochemical tests involving genetic markers and Filipin staining of skin fibroblast are being employed to assist diagnosis. Therapy is mostly supportive and since 2009, the first specific therapy approved for use was Miglustat (Zavesca) aimed at stabilizing the rate of progression of neurological manifestation. The prognosis correlates with age at onset of neurological signs; patients with early onset form progress faster. The NP-C disease has heterogeneous neurovisceral manifestations. A SI is a screening tool that helps in diagnostic process. Filipin staining test is a specific biomarker diagnostic test. Miglustat is the first disease-specific therapy.

Parasite distribution and early-stage encephalitis in Sarcocystis calchasi infections in domestic pigeons (Columba livia f. domestica)

Pigeon protozoal encephalitis is a biphasic, neurologic disease of domestic pigeons (Columba livia f. domestica) caused by the apicomplexan parasite Sarcocystis calchasi. Despite severe inflammatory lesions of the brain, associated parasitic stages have only rarely been identified and the cause of the lesions is still unclear. The aim of this study was therefore to characterize the tissue distribution of S. calchasi within pigeons between the two clinical phases and during the occurrence of neurological signs. For this purpose, a semi-quantitative real-time polymerase chain reaction (PCR) was developed. Forty-five domestic pigeons were infected orally (via a cannula into the crop) with 200 S. calchasi sporocysts and euthanized in groups of three pigeons at intervals of 2 to 10 days over a period of 61 days. Tissue samples including brain and skeletal muscle were examined by histology, immunohistochemistry, and PCR. Schizonts were detected in the liver of one pigeon at day 10 post infection. A mild encephalitis was detected at day 20 post infection, around 4 weeks before the onset of neurological signs. At the same time, immature sarcocysts were present in the skeletal muscle. In seven pigeons a few sarcocysts were identified in the brain, but not associated with any lesion. These results suggest that the encephalitis is induced at a very early stage of the S. calchasi lifecycle rather than in the chronic phase of pigeon protozoal encephalitis. Despite the increasing severity of lesions in the central nervous system, the amount of sarcocysts did not increase. This supports the hypothesis of a delayed-type hypersensitivity response as the cause of the encephalitis. The study also demonstrated that S. calchasi DNA is detectable in tissues negative by histological methods, indicating a higher sensitivity of the real-time PCR.