Atypical Neurological Presentation Research Articles

Neuroschistosomiasis in Young Filipino Patient Presenting with Seizure.

Diagnostic challenge and insightThis case highlights the importance of considering a broad differential diagnosis in patients with atypical neurological presentations, particularly in those from regions where parasitic infections are endemic. The internist plays a crucial role in integrating clinical, serological, and imaging data to arrive at a correct diagnosis, in this case, neuroschistosomiasis.Tailored treatment strategyThe case illustrates the value of personalized patient care. Initiating empirical treatment based on a combination of clinical suspicion, high-risk patient demographics, and imaging findings, without invasive procedures, demonstrates the internist's role in managing complex cases where patient preferences must be balanced with medical necessity.

Atypical neurological presentation in Gaucher disease type 3C

Atypical neurological presentation in Gaucher disease type 3C

A Typical Aortic Intramural Hematoma With an Atypical Neurological Presentation

A Typical Aortic Intramural Hematoma With an Atypical Neurological Presentation

The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review.

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy (TMA) related to a severe ADAMTS13 deficiency, the specific von Willebrand factor (VWF)-cleaving protease. This deficiency is often immune-mediated (iTTP) and related to the presence of anti-ADAMTS13 autoantibodies that enhance its clearance or inhibit its VWF processing activity. iTTP management may be challenging at extreme ages of life. International cohorts of people with TTP report delayed diagnoses and misdiagnoses in children and elderly people. Child-onset iTTP shares many features with adult-onset iTTP: a female predominance, an idiopathic presentation, and the presence of neurological disorders and therapeutic strategies. Long-term follow-ups and a transition from childhood to adulthood are crucial to preventing iTTP relapses, in order to identify the occurrence of other autoimmune disorders and psychosocial sequelae. In contrast, older iTTP patients have an atypical clinical presentation, with delirium, an atypical neurological presentation, and severe renal and cardiac damages. They also have a poorer response to treatment and prognosis. Long-term sequelae are highly prevalent in older patients. Prediction scores for iTTP diagnoses are not used for children and have a lower sensitivity and specificity in patients over 60 years old. ADAMTS13 remains the unique biological marker that is able to definitely confirm or rule out the diagnosis of iTTP and predict relapses during follow-ups.

Atypical Neurological Presentation in a Patient with Uncontrolled Diabetes

Chorea is a disorder characterized by irregular and involuntary hyperkinetic movements associated with malfunctioning the basal ganglia. One of the rarest acquired causes of unilateral or bilateral chorea is hyperglycemia. This is a case of an elderly female diabetic who presented With 3 days history of unilateral involuntary movements of the upper limb and lower limb, irrelevant talk, and altered sensorium. She had a random blood glucose level of 418 mg/dl with hemoglobin A1C (HbA1c) of 15% and normal ketones. Magnetic resonance imaging brain demonstrated T1 hyperintense and T2 flair hypointense right basal ganglia. Diagnosis of nonketotic hyperglycemic chorea was considered. The patient was treated with insulin, antipsychotics, and benzodiazepines and the chorea resolved after proper control of glucose levels. This condition can be reversible with early identification and treatment. This disorder can be prevented with good glycemic control.

500: DIAGNOSIS OF WERNICKE ENCEPHALOPATHY IN A PATIENT WITH UNDERLYING PSYCHIATRIC ILLNESS

Introduction: Wernicke’s encephalopathy (WE) is an acute neuropsychiatric syndrome caused by thiamine deficiency. Common causes include alcohol use, bariatric surgery, and hemodialysis. Description: Our case involves a 65-year-old female who initially presented with seizure-like activity. Subsequent presentations over months included dysphagia, behavioral changes, and confusion. Initial workup with EEG, CT head, MRI brain, CT soft tissue neck, and endoscopy was unremarkable. Further history revealed that after her mother’s death four months prior, she began to self-isolate and reduce oral intake. Patient was evaluated by psychiatry and was diagnosed with conversion disorder. The clinical course was complicated by worsening neurological symptoms and respiratory distress requiring mechanical ventilation. Repeat MRI brain showed increased T2 FLAIR signal in the periaqueductal gray matter and along the dorsomedial thalamus consistent with WE. Patient was started on thiamine and showed symptomatic improvement. Discussion: WE is a triad of oculomotor dysfunction, ataxia, and confusion. In an observational study by Chamorro et al, 92.7% of cases were due to excessive alcohol intake, 47.1% due to malnutrition, and 5.9% due to psychiatric illness. The diagnosis of WE is primarily clinical, yet the classic triad is reported in only 10% of cases. The most consistent characteristic is a change in mental status. Caine et al developed the clinical diagnostic criteria, which include dietary deficiencies, oculomotor abnormalities, cerebellar dysfunction, and altered mentation or mild memory impairment. A case series based on this criteria found that clinical features of WE vary between alcohol and non–alcohol-related etiologies. The classic triad was more common in alcoholics than in non-alcoholics. One of the main challenges is the rate of underdiagnosis, which can be due to a lack of clinical suspicion or atypical presentation. Hence, MRI is a useful test to support the diagnosis of WE with a sensitivity of 53% and specificity of 93%. MRI may demonstrate lesions with increased symmetrical signal intensity in the periaqueductal region, medullary bodies, medial thalami, and the tectum of the midbrain. Diagnosis of WE should be considered in patients with psychiatric illnesses and atypical neurologic presentation.

ATYPICAL MANIFESTATIONS OF SUBACUTE SCLEROSING PANENCEPHALITIS: CASE SERIES

Subacute Sclerosing Panencephalitis (SSPE) is a rare, progressive degenerative disease of the brain caused by reactivation of aberrant measles virus. Classical symptoms of SSPE are described as behavioral changes, declining scholastic performance, myoclonic jerks etc. However, at times it presents with varied atypical manifestations, which makes diagnosis difcult. Hereby we report two patients with unusual manifestations of SSPE. In rst case, 14-year-old boy presented with recurrent falls of 9 months duration. Initially, it was 1-2 times per week, progressing over 9 months to frequency of 40-50 falls per day. In second case, 21-year-old gentleman presented with behavioral changes and memory disturbance of 1-year duration. However, his family members noticed worsening of symptoms 1 month before presentation as he started developing involuntary jerky movements involving the right upper and lower limb. In both of them electroencephalogram (EEG) was showing long interval periodic discharges and cerebrospinal uid was showing elevated titers of anti-measles antibody overall suggesting SSPE. Hence it needs much attention for atypical presentations and also considering SSPE in differential diagnosis of unsolved atypical neurological presentations.

Clinical spectrum and predictors of severity of dengue among children in 2019 outbreak: a multicenter hospital-based study in Bangladesh

BackgroundThe mosquito-borne arboviral disease dengue has become a global public health concern. However, very few studies have reported atypical clinical features of dengue among children. Because an understanding of various spectrums of presentation of dengue is necessary for timely diagnosis and management, we aimed to document the typical and atypical clinical features along with predictors of severity among children with dengue during the largest outbreak in Bangladesh in 2019.MethodsWe conducted a cross-sectional study between August 15 and September 30, 2019. in eight tertiary level hospitals in Dhaka city. Children (aged < 15 years) with serologically confirmed dengue were conveniently selected for data collection through a structured questionnaire. Descriptive, inferential statistics, and multivariable logistic regression were used to analyze data.ResultsAmong the 190 children (mean age 8.8 years, and male-female ratio 1.22:1) included in the analysis, respectively 71.1 and 28.9% children had non-severe and severe dengue. All children had fever with an average temperature of 103.3 ± 1.2 F (SD). Gastrointestinal symptoms were the most common associated feature, including mostly vomiting (80.4%), decreased appetite (79.5%), constipation (72.7%), and abdominal pain (64.9%). Mouth sore, a less reported feature besides constipation, was present in 28.3% of children. Atypical clinical features were mostly neurological, with confusion (21.3%) being the predominant symptom. Frequent laboratory abnormalities were thrombocytopenia (87.2%), leucopenia (40.4%), and increased hematocrit (13.4%). Age (AOR 0.86, 95%CI 0.75–0.98, p = 0.023), mouth sore (AOR 2.69, 95%CI 1.06–6.96, p = 0.038) and a decreased platelet count (< 50,000/mm3) with increased hematocrit (> 20%) (AOR 4.94, 95%CI 1.48–17.31, p = 0.01) were significant predictors of severity.ConclusionsDengue in children was characterized by a high severity, predominance of gastrointestinal symptoms, and atypical neurological presentations. Younger age, mouth sores, and a decreased platelet with increased hematocrit were significant predictors of severity. Our findings would contribute to the clinical management of dengue in children.

Neurological Complications of Pulmonary Embolism: a Literature Review.

Purpose of ReviewThe present review discusses in-depth about neurological complications following acute venous thromboembolism (VTE).Recent FindingsIntracranial hemorrhage, acute ischemic cerebrovascular events, and VTE in brain tumors are described as central nervous system (CNS) complications of PE, while peripheral neuropathy and neuropathic pain are reported as peripheral nervous system (PNS) sequelae of PE. Syncope and seizure are illustrated as atypical neurological presentations of PE.SummaryMounting evidence suggests higher risk of venous thromboembolism (VTE) in patients with neurological diseases, but data on reverse, i.e., neurological sequelae following VTE, is underexplored. The present review is an attempt to explore some of the latter issues categorized into CNS, PNS, and atypical complications following VTE.

Hyperacute relapse of Lewis-Sumner syndrome during influenza A (H1N1) virus infection

BackgroundLewis-Sumner Syndrome (LSS) is considered an asymmetric sensory-motor variant of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), mostly affecting the limbs distally, with electrophysiological evidence of multifocal motor conduction blocks. Cranial nerve involvement is present in a minority. Various well-known infectious agents, directly or via the host’s immune responses, may trigger or exacerbate acute and chronic peripheral neuropathies, which may manifest clinically through a multitude of signs and symptoms.Case presentationWe present the case of a 57-year-old male with Lewis-Sumner Syndrome, whose clinical course was quite stable over many years. He developed severe hyperacute relapse of his neuropathic disease in the context of active pneumonia due to influenza A (H1N1) virus infection. During this exacerbation, besides the obvious worsening of the previous asymmetric limb involvement, the patient also manifested left peripheral facial palsy and dysphagia that rapidly evolved over minutes, mimicking a stroke. The patient also showed rapid recovery, with marked improvement of the acute neuropathic dysfunction, immediately after initiation of treatment with oseltamivir. Our hypothesis is that the direct modulation of Na + ion channel activity in the host’s peripheral nerve cell by H1N1 viral proteins could cause acute and potentially reversible dysfunction in the conduction of nerve action potentials. Direct viral neuritis could also have been the cause. Immunomodulatory agents, namely IVIg, were not administered due to the swift clinical improvement noticed in the following days.ConclusionsWe aim to raise awareness of the possibility of atypical neurological presentations of viral infections, especially relevant in the context of the pandemic the world is now facing.

Immune thrombotic thrombocytopenic purpura in older patients: prognosis and long-term survival

AbstractOlder age is associated with increased mortality in immune thrombotic thrombocytopenic purpura (iTTP). Yet, data are scarce regarding iTTP occurring among older patients. To assess clinical features and long-term impact of iTTP on mortality in older patients (>60 years old), characteristics and prognoses of adult iTTP patients enrolled in the French Reference Center for Thrombotic Microangiopathies registry between 2000 and 2016 were described according to age (<60 years old or ≥60 years old). Long-term mortality of iTTP older survivors was compared with that of non-iTTP geriatric subjects. Comparing, respectively, older iTTP patients (N = 71) with younger patients (N = 340), time from hospital admission to diagnosis was longer (P < .0001); at diagnosis, delirium (P = .034), behavior impairment (P = .045), renal involvement (P < .0001), and elevated troponin level (P = .025) were more important whereas cytopenias were less profound (platelet count, 22 × 103/mm3 [9-57] vs 13 × 103/mm3 [9-21], respectively [P = .002]; hemoglobin level, 9 g/dL [8-11] vs 8 g/dL [7-10], respectively [P = .0007]). Short- and mid-term mortalities were higher (P < .0001) and increased for every 10 years of age range. Age ≥60 years, cardiac involvement, increased plasma creatinine level, and total plasma exchange volume were independently associated with 1-month mortality. Compared with a non-iTTP geriatric population, older survivors showed an increased long-term mortality (hazard ratio = 3.44; P < .001). In conclusion, older iTTP patients have atypical neurological presentation delaying the diagnosis. Age negatively impacts short-term but also long-term mortality.

Atypical Presentation of Glioblastoma Multiforme.

BackgroundGlioblastoma multiforme (GBM) is a highly malignant glial tumour classified by the World Health Organization (WHO) as a stage IV astrocytoma. It varies in shape and size and can be cystic, vascular and necrotic. It often appears as a ring-enhancing lesion on magnetic resonance imaging (MRI). The most common symptoms of GBM, such as headache, vomiting and seizures, are due to increased intracranial pressure. The objective of this case report is to describe an atypical presentation of GBM.Case ReportA 53-year-old woman of Italian origin presented with a 2-week history of lack of coordination in her hands and some difficulty in speech. Electromyography for assessment of her arms and cranial bulbar function was normal. However, 2 days later, the patient presented to the emergency department with progressive weakness in her left arm and leg as well as difficulty in speech. Mild left facial asymmetry was noted. A brain MRI revealed a right frontal mass. Stereotactic surgical resection was performed 2 days later, and biopsy confirmed the diagnosis of GBM. Although headache and other features of raised intracranial pressure are the most common initial symptoms of GBM, any atypical neurological or psychiatric presentation in an adult patient should raise suspicion for this tumour.ConclusionCareful analysis of an adult with atypical signs and symptoms along with thorough review of radiological tests will facilitate early diagnosis of dangerous tumours such as GBM.LEARNING POINTAn adult patient with symptoms that do not conform to a neurological condition should be investigated for a brain tumour.Careful history taking and examination are essential for reaching the correct diagnosis as soon as possible.Meticulous review of radiological images in order to detect subtle changes in brain anatomy is essential.

Atypical Presentation of Glioblastoma Multiforme

Background: Glioblastoma multiforme (GBM) is a highly malignant glial tumour classified by the World Health Organization (WHO) as a stage IV astrocytoma. It varies in shape and size and can be cystic, vascular and necrotic. It often appears as a ring-enhancing lesion on magnetic resonance imaging (MRI). The most common symptoms of GBM, such as headache, vomiting and seizures, are due to increased intracranial pressure. The objective of this case report is to describe an atypical presentation of GBM. Case Report: A 53-year-old woman of Italian origin presented with a 2-week history of lack of coordination in her hands and some difficulty in speech. Electromyography for assessment of her arms and cranial bulbar function was normal. However, 2 days later, the patient presented to the emergency department with progressive weakness in her left arm and leg as well as difficulty in speech. Mild left facial asymmetry was noted. A brain MRI revealed a right frontal mass. Stereotactic surgical resection was performed 2 days later, and biopsy confirmed the diagnosis of GBM. Although headache and other features of raised intracranial pressure are the most common initial symptoms of GBM, any atypical neurological or psychiatric presentation in an adult patient should raise suspicion for this tumour. Conclusion: Careful analysis of an adult with atypical signs and symptoms along with thorough review of radiological tests will facilitate early diagnosis of dangerous tumours such as GBM.

Human Usutu Virus Infection with Atypical Neurologic Presentation, Montpellier, France, 2016.

Infection with Usutu virus (USUV) has been recently associated with neurologic disorders, such as encephalitis or meningoencephalitis, in humans. These findings indicate that USUV is a potential health threat. We report an acute human infection with USUV in France putatively associated with a clinical diagnosis of idiopathic facial paralysis.

Reversible myelopathy: an uncommon accompaniment of hypothyroidism

Atypical neurological presentations of hypothyroidism include myelopathy which has been seldom reported as presenting feature in patients. This case was one of those atypical presentations of hypothyroidism in which patient presented with neurological complication viz myelopathy which was timely diagnosed and treated. The symptoms including neurological findings reversed with thyroxin replacement. The case highlights the varied and vivid manifestations of hypothyroidism which treating physicians should be aware of while treating patients.

Jacksonian March Revisited? A Case of Local Tetanus with Generalized Spread

A 41 year-old male with no prior history of seizure disorder presented to the emergency department complaining of four “seizure” episodes which began a few hours prior to arrival. Within minutes of triage, one of the episodes was witnessed and inconsistencies with seizure activity including an absence of clonus, consciousness throughout the event and lack of a post-ictal state were noted. Further examination led to discovery of an untreated laceration on the patient’s left hand which had been sustained 1-week prior on a rusted metal fence. More extensive history also revealed recurrent episodes of muscle spasm in his left hand which preceding the generalized attacks. A presumptive diagnosis of wound tetanus with secondary, generalized manifestation was made and the patient was treated with local and intramuscular tetanus immunoglobulin as well as intravenous metronidazole and diazepam. The patient was admitted to the Neurologic Intensive Care Unit and, after 1 week of therapy, made a full recovery. While rare in the developed world, acute tetanus remains a disease associated with a significant morbidity and mortality (even in the United States). The high rate of lapsed adult immunization as well as the prevalence of insufficient effective antibodies even in those sufficiently immunized mandates vigilance on the part of the emergency physicians-particularly in patients with atypical neurological presentations.

Atypical neurological presentation of GSS: Case report

Introduction: Gougerot-Sjogren Syndrome (GSS) is an auto-immune exocrinopathy characterized by xerophthalmia and xerostomia. Neurological complications occur between 8.5 and 70%. Data concerning central nervous system (CNS) symptoms have been rarely described/INS;. We analyze the clinical, radiological and therapeutic aspects of a patient with CNS signs revealing primitive GSS and we discuss some particularities.

Paraplegia in a chiropractic patient secondary to atraumatic dural arteriovenous fistula with perimedullary hypertension: case report

Intracranial dural arteriovenous fistulas are abnormal communications between higher-pressure arterial circulation and lower-pressure venous circulation. This abnormal communication can result in important and frequently misdiagnosed neurological abnormalities.A case of rapid onset paraplegia following cervical chiropractic manipulation is reviewed. The patient’s generalized spinal cord edema, lower extremity paraplegia and upper extremity weakness, were initially believed to be a complication of the cervical spinal manipulation that had occurred earlier on the day of admission. Subsequent diagnostic testing determined the patient suffered from impaired circulation of the cervical spinal cord produced by a Type V intracranial arteriovenous fistula and resultant venous hypertension in the pontomesencephalic and anterior spinal veins.The clinical and imaging findings of an intracranial dural arteriovenous fistula with pontomesencephalic venous congestion and paraplegia are reviewed.This case report emphasizes the importance of thorough and serial diagnostic imaging in the presence of sudden onset paraplegia and the potential for error when concluding atypical neurological presentations are the result of therapeutic misadventure.

Fabry Disease With Atypical Neurological Presentation

Fabry disease (FD) is a rare, X-linked lysosomal storage disorder with multiorgan involvement. FD is caused by a partial or total deficit of α-galactosidase A enzyme, which is responsible for the accumulation of glycosphingolipids in a variety of cell types. Neurological complications include central nervous system involvement with cerebrovascular disease, peripheral neuropathy, and autonomic dysfunction. We report the case of a 47-year-old man with an atypical neurological presentation of FD, characterized by 48-hour consciousness alteration with amnesia, resembling a long-lasting episode of transient global amnesia. Our case expands the neurological presentations associated with FD.

Insulinoma presenting as refractory seizure disorder

Diagnosis of insulinoma may be delayed when symptoms are nonspecific. Rarely neuroglycopenic symptoms are the primary feature and these patients can be misdiagnosed as having epilepsy or neuropsychiatric disease. We report a case of insulinoma presenting as an adult-onset refractory seizure disorder. The time from onset of symptoms to diagnosis was 9 years. The atypical features of the episodes of hypoglycaemia, and the poor response to treatment led to a review of diagnosis. This case highlights the importance of considering hypoglycaemia in atypical neurological or psychiatric presentations. DOI: http://dx.doi.org/10.4038/sjdem.v2i1.4336 Sri Lanka Journal of Diabetes, Endocrinology and Metabolism 2012; 1 : 46-48