Unusual Neurological Manifestations Research Articles

Bilateral blindness following Russell's viper bite - a rare clinical presentation: a case report.

IntroductionRussell’s viper (Daboia russelii) is one of the most common medically important snakes reported in Sri Lanka. Its envenomation leads to significant mortality and morbidity with local, hematological, neurological and renal complications. Here we report the case of a patient who presented with bilateral blindness secondary to a bilateral posterior circulation ischemic stroke instead of the usual neurological manifestations of Russell’s viper envenomation. There were no reported cases of cortical blindness following a Russell’s viper bite. Only a few reported cases of ischemic strokes following a Russell’s viper bite were found in the literature.Case presentationA 54-year-old Sri Lankan woman developed bilateral blindness due to a posterior circulation infarct following Russell’s viper envenomation.ConclusionIschemic stroke following a Russell’s viper bite is very rare and cortical blindness is not reported as the clinical presentation. Also, we emphasize the importance of considering the possibility of ischemic stroke in patients who develop unusual neurological manifestations following Russell’s viper envenomation.

Dengue fever presenting with acute cerebellitis: a case report

BackgroundThe incidence of dengue fever is on the rise in tropical countries. In Sri Lanka, nearly 45,000 patients were reported in 2012. With the increasing numbers, rare manifestations of dengue are occasionally encountered. We report a patient who presented with bilateral cerebellar signs as the presenting feature of dengue.Case presentationA 45-year-old previously healthy female from the suburbs of Colombo, Sri Lanka presented with an acute febrile illness associated with unsteadiness of gait. Clinical examination revealed a scanning dysarthria and marked horizontal nystagmus with bilateral dysmetria, dysdiadokokinesia and incordination more prominent on the right. Her gait was wide-based and ataxic with a tendency to fall to the right more than to the left. Dengue nonstructural protein antigen 1 test and IgM antibody testing both became positive indicating acute dengue infection. She recovered from the febrile episode within 9 days since the onset of fever but cerebellar symptoms outlasted the fever by one week. The magnetic resonance imaging of brain was normal and cerebellar signs resolved spontaneously by day 17 of the illness.ConclusionsCerebellar syndrome in association with dengue fever has been reported in only four instances and our patient is the first reported case of dengue fever presenting with cerebellitis as the first manifestation of disease. This case report is intended to highlight the occurrence of acute cerebellitis as a presenting syndrome of the expanding list of unusual neurological manifestations of dengue infection.

Unusual clinical manifestations of dengue infection in children in a tertiary care hospital in northeast Thailand

Abstract Background: Dengue virus infection has been a public health concern in Thailand. In the past decades, there has been recent interest concerning unusual clinical manifestations in both dengue fever (DF) and dengue hemorrhagic fever (DHF). Objective: We described the unusual clinical manifestations and outcomes of children with dengue admitted to a tertiary care hospital in northeast Thailand. Materials and Methods: A study was conducted on the 73 patients with serologically confirmed dengue infection admitted to Srinagarind Hospital, a tertiary care facility in northeast Thailand between January 2007 and August 2011. Results: Of the 73 children examined, 42 (57%) were boys and 31 were girls. Their age ranged from 8 months to 14 years (median 11 years). Nine patients developed neurological symptoms, 6 patients had altered consciousness, and 3 patients convulsion. Among 9 patients with neurological symptoms, 1 patient had acute kidney injury, 1 had hepatic failure, and 1 had kidney and liver involvement, mostly associated with fluid resuscitation or prolonged shock. Apart from neurological symptoms, one patient developed infection associated hemophagocytic syndrome and was treated with intravenous immunoglobulin. Two patients died from multiple organ failure, and 1 patient was brought back home in a moribund condition. The other patients recovered completely. Conclusion: Altered consciousness was the most commonly observed unusual neurological manifestation. Patients who did not develop acute kidney injury or liver failure had mild clinical courses and recovered from neurological symptoms without sequelae. Acute kidney injury was associated with fluid overload and/or prolonged shock. Careful fluid management and close monitoring for complications resulted in favorable outcomes.

Juvenile Systemic Lupus Erythematosus and Glioblastoma:A Case Report and Literature Review

This is a case report of 11-years-old Chadian female, diagnosed with juvenile systemic lupus erythematosus based on five out of eleven criteria of the American College of Rheumatology. Her disease was under control on oral prednisolone, azathioprine and hydroxychloroquine. One year after diagnosis, she presented to the emergency department complaining of severe headache, vomiting and deterioration in the level of consciousness. Urgent brain computed tomography scan showed large brain tumour in the left parietooccipital region with significant midline shifting. Craniotomy and excisional biopsy of the mass was performed by a neurosurgeon. Histopathology reports a high grade malignant glioblastoma multiforme. Three cases were reported in the literature as space occupying lesion in the brain in juvenile systemic lupus erythematosus. To our knowledge, glioblastoma multiforme is the first case to report in juvenile systemic lupus erythematosus. Thus, severe headache and unusual neurological manifestations in juvenile systemic lupus erythematosus may be related to space occupying lesion, and should be evaluated immediately for early medical and surgical management.

Behavior Disorder After Encephalitis Caused by Dengue

Behavior Disorder After Encephalitis Caused by Dengue

Neurobrucellosis with bilateral sensorineural hearing loss and ataxia

Summary Brucellosis is a zoonotic infection and has endemic characteristics. Neuro­ brucellosis is an uncommon complication of this infection. In neurobru­ cellosis, various clinical and neuroradiological signs and symptoms can be confused with other neurological diseases. In inhabitants or visitors of e ndemic areas, neurobrucellosis should be kept in mind in cases which have unusual neurological manifestations. We present a case of a 50­year­old f emale who presented with headache, bilateral hearing loss for 1 year with ataxia and vertigo for 2 months. The case is reported because of the d iagnostic dilemma and its rarity.

Multidrug-Resistant Typhoid Fever With Neurologic Findings on the Malawi-Mozambique Border

Salmonella enterica serovar Typhi causes an estimated 22 million cases of typhoid fever and 216 000 deaths annually worldwide. We investigated an outbreak of unexplained febrile illnesses with neurologic findings, determined to be typhoid fever, along the Malawi-Mozambique border. The investigation included active surveillance, interviews, examinations of ill and convalescent persons, medical chart reviews, and laboratory testing. Classification as a suspected case required fever and ≥1 other finding (eg, headache or abdominal pain); a probable case required fever and a positive rapid immunoglobulin M antibody test for typhoid (TUBEX TF); a confirmed case required isolation of Salmonella Typhi from blood or stool. Isolates underwent antimicrobial susceptibility testing and subtyping by pulsed-field gel electrophoresis (PFGE). We identified 303 cases from 18 villages with onset during March-November 2009; 214 were suspected, 43 were probable, and 46 were confirmed cases. Forty patients presented with focal neurologic abnormalities, including a constellation of upper motor neuron signs (n = 19), ataxia (n = 22), and parkinsonism (n = 8). Eleven patients died. All 42 isolates tested were resistant to ampicillin, chloramphenicol, and trimethoprim-sulfamethoxazole; 4 were also resistant to nalidixic acid. Thirty-five of 42 isolates were indistinguishable by PFGE. The unusual neurologic manifestations posed a diagnostic challenge that was resolved through rapid typhoid antibody testing in the field and subsequent blood culture confirmation in the Malawi national reference laboratory. Extending laboratory diagnostic capacity, including blood culture, to populations at risk for typhoid fever in Africa will improve outbreak detection, response, and clinical treatment.

Detection of the dengue non-structural 1 antigen in cerebral spinal fluid samples using a commercially available enzyme-linked immunosorbent assay

The involvement of the central nervous system in dengue infections has been reported in countries where the disease in endemic. The purpose of this study was to determine whether an enzyme-linked immunosorbent assay kit designed to detect the dengue NS1 antigen in serum was able to detect this antigen in cerebral spinal fluid (CSF) samples from patients with fatal outcomes. To evaluate the sensitivity of the kit, 26 dengue-positive CSF samples were used. The Pan-E Dengue Early kit was able to detect the NS1 antigen in 13 of 26 dengue-positive CSF samples, resulting in a sensitivity of 50% (95% confidence interval, 29.9–70.1%) and specificity of 100% (95% confidence interval, 75.3–100%). The kit was able to detect the NS1 antigen in CSF of individuals who had died of dengue. When used in combination with IgM, the detection rate rose to 92.3%. This study reports a method for rapidly detecting the dengue virus in CSF, thereby increasing the diagnosis of dengue fever cases with unusual neurological manifestations.

Primary antiphospholipid antibody syndrome presenting with encephalopathy, psychosis and seizures

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by recurrent thrombotic events, miscarriages and thrombocytopenia with persistently positive antiphospholipid antibodies.( 1,2 ) APS may be isolated (primary APS) or associated to a connective tissue disease, most often systemic lupus erythematosus (SLE).( 1,2 ) APS usually affects young patients before the fifth decade( 3 ) with stroke being the commonest neurological manifestation.( 4 ) Various other neurological manifestations are being recognized in patients with APS including migraine, epilepsy, multi-infarct dementia and chorea.( 2 ) The pathological process underlying the neurological manifestations remains obscure.( 1,2 ) Herein we report a case of primary APS presenting with a group of unusual neurological manifestations in a 68-year-old woman.

Unusual neurologic manifestations of varicella zoster virus infection with the absence of rash in a kidney transplant recipient

M. Assi, S. Abou Antoun. Unusual neurologic manifestations of varicella zoster virus infection with the absence of rash in a kidney transplant recipient. Transpl Infect Dis 2011: 13: 545–547. All rights reserved

Neurobrucellosis: clinical, diagnostic, therapeutic features and outcome. Unusual clinical presentations in an endemic region

Brucellosis is a zoonotic infection and has endemic characteristics. Neurobrucellosis is an uncommon complication of this infection. The aim of this study was to present unusual clinical manifestations and to discuss the management and outcome of a series of 18 neurobrucellosis cases. Initial clinical manifestations consist of pseudotumor cerebri in one case, white matter lesions and demyelinating syndrome in three cases, intracranial granuloma in one case, transverse myelitis in two cases, sagittal sinus thrombosis in one case, spinal arachnoiditis in one case, intracranial vasculitis in one case, in addition to meningitis in all cases. Eleven patients were male and seven were female. The most prevalent symptoms were headache (83%) and fever (44%). All patients were treated with rifampicin, doxycycline plus trimethoprim-sulfamethoxazole or ceftriaxone. Duration of treatment (varied 3-12 months) was determined on basis of the CSF response. In four patients presented with left mild sequelae including aphasia, hearing loss, hemiparesis. In conclusion, although mortality is rare in neurobrucellosis, its sequelae are significant. In neurobrucellosis various clinical and neuroradiologic signs and symptoms can be confused with other neurologic diseases. In inhabitants or visitors of endemic areas, neurobrucellosis should be kept in mind in cases that have unusual neurological manifestations.

Neurobrucellosis: clinical, diagnostic, therapeutic features and outcome. Unusual clinical presentations in an endemic region

Brucellosis is a zoonotic infection and has endemic characteristics. Neurobrucellosis is an uncommon complication of this infection. The aim of this study was to present unusual clinical manifestations and to discuss the management and outcome of a series of 18 neurobrucellosis cases. Initial clinical manifestations consist of pseudotumor cerebri in one case, white matter lesions and demyelinating syndrome in three cases, intracranial granuloma in one case, transverse myelitis in two cases, sagittal sinus thrombosis in one case, spinal arachnoiditis in one case, intracranial vasculitis in one case, in addition to meningitis in all cases. Eleven patients were male and seven were female. The most prevalent symptoms were headache (83%) and fever (44%). All patients were treated with rifampicin, doxycycline plus trimethoprim-sulfamethoxazole or ceftriaxone. Duration of treatment (varied 3-12 months) was determined on basis of the CSF response. In four patients presented with left mild sequelae including aphasia, hearing loss, hemiparesis. In conclusion, although mortality is rare in neurobrucellosis, its sequelae are significant. In neurobrucellosis various clinical and neuroradiologic signs and symptoms can be confused with other neurologic diseases. In inhabitants or visitors of endemic areas, neurobrucellosis should be kept in mind in cases that have unusual neurological manifestations.

Neurosjögren : une présentation inhabituelle

Neurological manifestations in Sjögren's syndrome are variable. The peripheral nervous system is generally involved. We report a rare case of an unusual central neurological manifestation. A 54-year-old woman was admitted with headache and tetraparesia. The physical examination revealed a tetrapyramidal syndrome and a bilateral parotidomegaly. The patient's general condition was nevertheless quite good. Brain MRI showed an heterogeneous pontine lesion with multiple nodular formations in the periventricular white matter. Blood tests revealed anti-SSA and anti-SSB antibodies. A labial salivary gland biopsy was grade IV in Chisholm scoring system and Schirmer's test was positive. A latent Sjögren's syndrome can lead to a wide variety of focal brain MRI abnormalities and should be evoked when the etiology is not clear.

Unusual presentations of nervous system infection by Cryptococcus neoformans

Nervous system infections by Cryptococcus neoformans may occur not only in congenital or acquired immunodeficiency syndromes, but also in immunocompetent hosts. Neurological manifestations of C. neoformans infection include meningitis and, less commonly, parenchymal CNS granulomatous disease. This paper provides detailed clinical descriptions of highly unusual neurological manifestations of cryptococcal nervous system infections. Medical records and diagnostic data including magnetic resonance imaging, histopathology, serology, and CSF analysis were reviewed. A conus medullaris abscess was found in a patient infected with the human immunodeficiency virus (HIV). A patient with Hodgkin's disease was diagnosed with cryptococcal meningitis and dermatitis mimicking ophthalmic zoster. An immunocompetent patient presented with recurrent cerebral infarctions in the setting of cryptococcal meningitis. Cryptococcal infections of the nervous system can cause severe neurological disability when diagnosis is delayed. Sensitive and specific tests are readily available and should be considered when an unusual clinical presentation is encountered.

Cholesterol Ester Storage Disease With Unusual Neurological Manifestations in Two Siblings: A Report From South India

Cholesterol ester storage disease is a rare autosomal recessive storage disorder resulting from lysosomal acid lipase deficiency. Two siblings manifested with hepatosplenomegaly, ptosis, and bilateral external ophthalmoplegia. Evaluation revealed hyperlipidemia and bilateral adrenal calcifications. Leukocyte acid lipase levels were significantly low in both the patients, compared with controls, suggesting a diagnosis of cholesterol ester storage disease. Ptosis and external ophthalmoplegia have hitherto not been reported in cholesterol ester storage disease.

Unusual neurologic manifestations (I): Parkinsonism in juvenile SLE

A girl with neuropsychiatric lupus demonstrated Parkinsonian features soon after commencing risperidone. The single photon emission computed tomography scan showed hyperperfusion of the basal ganglia. Symptoms abated with the addition of dopaminergic agents to immunosuppressive therapy. The literature on juvenile Parkinsonism in lupus has been reviewed.

Unusual neurologic manifestation of compressive radiculopathy of the first thoracic root (T1)

Unusual neurologic manifestation of compressive radiculopathy of the first thoracic root (T1)

Unusual presentations of neurocysticercosis

Neurocysticercosis (NCC) is the most common helminthic infection of the central nervous system. It is a major public health problem in developing countries, however, rarely reported in Saudi Arabia. As far as our knowledge, this is the first case report of NCC in Saudi Arabia. An increasing number of immigrants from endemic areas is the cause. Here, we report three interesting cases of NCC in three adult females with very unusual clinical presentation. These include: (case 1) progressive spasticity of the four limbs with severe pseudobulbar manifestations, precipitancy of micturition, cognitive impairment and no eye involvement; (case 2) dystonic movement of right upper and lower limbs together with weight gain, lethargy, mental dullness and goitre; and (case 3) depressive manifestations together with disturbance of writing, mild deep sensory loss, limb-kinetic apraxia, lack of coordination and dystonia of the right upper limb. The clinical details and possible mechanisms for the uncommon presentations are discussed. NCC should be kept in mind and raise a high level of suspicion for unusual neurological manifestations in Saudian patients particularly as there has been increasing percentage of immigration from endemic areas during the last few years.

Manifestaciones clínicas inusuales del dengue hemorrágico en niños.

Recently, dengue hemorrhagic fever with unusual neurological and hepatic manifestations has been reported in children, with a concomitant increase in morbidity and mortality. To describe unusual clinical manifestations of dengue hemorrhagic fever in children. These manifestations were systematically scrutinized in dengue cases occurring between 1992-2004 in the Pediatrics Department of the University Hospital of Santander, Bucaramanga, Colombia. Case reports were examined of 913 patients with clinical symptoms of dengue hemorrhagic fever. They were classified according to standard criteria for dengue hemorrhagic fever and grouped according to the main organs or systems that were affected. Clinical presentation, laboratory results and medical evolution were summarized. Of the 913 cases, 168 showed abnormal clinical manifestations; these were predominant in male school age children and associated mainly with grades III and IV of dengue hemorrhagic fever. The most frequent manifestations were as follows: hepatitis--53 cases (27%), neurological alterations--49 (25%), renal impairment--14 (7%), cardiac involvement--15 (8%), pulmonary alterations--18 (9%), alithiasic cholecistitis--18 (9%), haemophagocitic syndrome--5 (2.5%), pancreatitis--2 (1%), and acute abdominal pain--21 (11%). Ten patients died. The results were compared with data from other endemoepidemic countries of dengue. The significance and importance of abnormal dengue were explored, as well as the difficult retrospective diagnoses. Hepatic and neurological manifestations were recognized as frequently involved in elevated morbidity and mortality. Physicians working in endemic zones should be aware of them in order to recognize them.

Unusual neurologic manifestation of compressive radiculopathy of the first thoracic root (T1)

Unusual neurologic manifestation of compressive radiculopathy of the first thoracic root (T1)