scholarly journals An Atypical Presentation of CLIPPERS, a Challenging Diagnosis of Reversible Early-Onset Dementia

2020 ◽  
Vol 12 (3) ◽  
pp. 307-313
Author(s):  
Mirella Russo ◽  
Alberto Piermartiri ◽  
Claudia Carrarini ◽  
Fedele Dono ◽  
Maria Vittoria De Angelis ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Brain Mri ◽  
Antidepressant Drugs ◽  
Laboratory Research ◽  
Inflammatory Disorder ◽  
Atypical Presentation ◽  
Ataxic Gait ◽  
Laboratory Findings ◽  
Early Onset Dementia ◽  
Fluid Analysis

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory disorder featured by pontocerebellar dysfunctions and, in some cases, later cognitive disturbances. Here, we describe an atypical presentation of CLIPPERS, characterized by clinical onset with neuropsychiatric and cognitive symptoms. A 45-year-old man was referred to our Memory Clinic due to difficulties at work for over a month, caused by confusion and asthenia. Furthermore, insomnia and mood changes appeared. These disturbances were unresponsive to antipsychotic and antidepressant drugs. At admission, the patient presented also with severe cognitive impairment, urinary incontinence, ataxic gait, and limitation of lateral conjugate gaze. During the hospitalization, the patient underwent cerebrospinal fluid analysis, serum systemic autoimmune disorders laboratory research, neoplastic markers analysis, and brain MRI scan. The radiological and laboratory findings were compatible with the diagnosis of CLIPPERS. The sudden clinical and radiological improvement of the patient’s conditions, after only a week of steroid therapy, further confirmed our clinical suspicion. The present case enhances the necessity to consider CLIPPERS in the differential diagnosis of pre-senile cognitive impairment, even in the absence of early pontocerebellar neurological signs. Before the spreading of the neuroinflammatory and degenerative processes, CLIPPERS represents one among the few possible reversible causes of cognitive decline.

Haemiballism/haemichorea: an atypical presentation of ischaemic stroke

2021 ◽  
Vol 14 (6) ◽  
pp. e240439
Author(s):  
Raman Nohria ◽  
Stacey Bennett ◽  
Yasmin Ali O'Keefe
Keyword(s):  
Brain Mri ◽  
Acute Onset ◽  
Cerebral Peduncle ◽  
Atypical Presentation ◽  
Cerebrospinal Fluid Analysis ◽  
Medication Therapy ◽  
Fluid Analysis ◽  
Ct Head ◽  
The Right ◽  
Distressing Condition

A 76-year-old man was admitted to the hospital with acute onset of involuntary movements of the left side of his body. His neurological examination revealed he was oriented only to himself, and aforementioned movements of his left arm and leg. CT head demonstrated old infarcts in his right aspect of his pons and basal ganglia. Cerebrospinal fluid analysis was unremarkable. He initially had a normal blood glucose with an elevated anion gap and elevated creatine kinase. Brain MRI showed a small lacunar-type ischaemic infarct within the anteromedial aspect of the right cerebral peduncle, which localised to his haemiballism. To prevent worsening rhabdomyolysis associated with his haemiballism, the primary team initiated both tetrabenazine and diazepam. His movements improved after 1 week of medication therapy. This report discusses a thorough workup for this movement disorder and when to intervene for this distressing condition.

Posterior reversible encephalopathy syndrome following Miller-Fisher syndrome

2021 ◽  
Vol 14 (7) ◽  
pp. e242231
Author(s):  
Catarina Bernardes ◽  
Cristiana Silva ◽  
Gustavo Santo ◽  
Inês Correia
Keyword(s):  
Intravenous Immunoglobulin ◽  
Posterior Reversible Encephalopathy Syndrome ◽  
Brain Mri ◽  
Clonic Seizure ◽  
Miller Fisher Syndrome ◽  
Fisher Syndrome ◽  
Posterior Reversible Encephalopathy ◽  
Fluid Analysis ◽  
Immunoglobulin Treatment ◽  
Reversible Encephalopathy

A 71-year-old woman presented to the emergency room with dysphonia, diplopia, dysphagia and generalised weakness since that day. Neurological examination revealed eye adduction limitation, ptosis, hypoactive reflexes and gait ataxia. Blood and cerebrospinal fluid analysis and brain CT were normal. Electromyography revealed a sensory axonal polyneuropathy. She was diagnosed with Miller-Fisher syndrome (MFS) and started on intravenous immunoglobulin. Two days after intravenous immunoglobulin treatment was completed, she developed a sustained hypertensive profile and presented a generalised tonic-clonic seizure. Brain MRI was suggestive of posterior reversible encephalopathy syndrome (PRES) and supportive treatment was implemented with progressive improvement. PRES may be a possible complication of MFS not only due to autonomic and inflammatory dysfunctions, but also as a consequence of its treatment. Patients with MFS should be maintained under close surveillance, especially in the first days and preferably in intermediate care units.

scholarly journals Meningoencephalitis in intensive care unit: Do Japanese Encephalitis cases go unsuspected?

2017 ◽  
Vol 7 (1) ◽  
pp. 7-10
Author(s):  
Ashish Ghimire ◽  
Balkrishna Bhattarai ◽  
Basudha Khanal ◽  
Suchana Marhatta ◽  
Gopendra Prasad Deo
Keyword(s):  
Intensive Care Unit ◽  
Intensive Care ◽  
Adult Patients ◽  
Medical Emergency ◽  
Real Problem ◽  
Cerebrospinal Fluid Analysis ◽  
Laboratory Findings ◽  
Fluid Analysis ◽  
Intensive Approach ◽  
Serology Testing

Background: Meningoencephalitis (ME) is a medical emergency. Acute infective encephalitis is usually viral. Nepal has the second highest prevalence of JE in South East Asia. About one third of the JE cases results in death. The records of the JE in the laboratory of B.P. Koirala Institute of Health Sciences (BPKIHS) showed 760 samples to have the evidence of JE infection out of 3352 tested during the period of 2001-2011 AD. The present study aimed to conduct an audit on meningoencephalitis cases admitted in the ICU of our hospital in an attempt to find its burden.Methods: Medical records of all the adult patients with the provisional diagnosis of Meningoencephalitis at BPKIHS over the period of 2009-2011 AD were examined. Laboratory findings of all the patients diagnosed with ME who were admitted to the eight bedded general intensive care unit (ICU) were also reviewed. The diagnosis was made based on the findings of medical history, clinical examination and cerebrospinal fluid analysis for biochemistry and cytology, results of anti JE IgM test performed on CSF and/or serum.Results: Altogether 127 samples of CSF were obtained from adult patients admitted in different inpatient department of BPKIHS with the clinical diagnosis of meningo encephalitis between 2009-2011 AD. Out of them, 25 (19.68%) were tested positive of JE. A total of 684 patients were admitted over the same period with different diagnoses in 8 bedded ICU of hospital. Fifteen (2.19%) were transferred to the ICU with the ME. In the ICU, 9(60%) were treated as viral encephalitis. Among them CSF of only 4 patients’ were sent for testing for JE and found positive in 2 cases. Seven (46.6%) patients expired while 4(26.6%) were taken home by the relatives against medical advice in critically ill conditions and remaining 4 (26.6%) improved and transferred to ward. Among the expired patients 5 (71.4%) had viral encephalitis.Conclusion: JE is a real problem but many cases are likely to have been gone undetected. Adoption of a more intensive approach with much liberal serology testing policy seems to be appropriate for better detecting JE cases in the setting.

scholarly journals Miller Fisher Syndrome- a Case Report and Review of Literature

2016 ◽  
Vol 32 (1) ◽  
pp. 47-51
Author(s):  
Imran Sarker ◽  
Maliha Hakim ◽  
Mahmudul Islam ◽  
Md Badrul Alam ◽  
Md Rafiqul Islam
Keyword(s):  
Treatment Options ◽  
Brain Mri ◽  
Clinical Findings ◽  
Miller Fisher Syndrome ◽  
Upper Respiratory Tract ◽  
Bilateral Ptosis ◽  
Laboratory Findings ◽  
Fisher Syndrome ◽  
Disease Modifying Treatment ◽  
Subsequent Sample

Background and Objective:: Miller Fisher syndrome (MFS) is a variant of Guillan Barre syndrome characterized by ophthalmoplegia, ataxia and areflexia. Although self-limiting disease course is expected, disease modifying treatment options for MFS are no different than for GBS and include intravenous immune globulin (IVIG) and plasmapheresis. Here, we report a case of MFS presented with bilateral ptosis, ophthalmoplegia, ataxia with quadriparesis and normal NCS. Patient Methods: A 14- year-old young boy was admitted to our hospital with the complaints of double vision, vertigo, difficulty in walking, imbalance. He had no diarrhea or upper respiratory tract infection prior to this illness. On neurological examination, he had limited ability to move his eyes up and out, had bilateral ptosis, ataxia. The muscle strength was mildly impaired. The plantar reflexes were flexor and the deep tendon reflexes were absent. Results: The blood laboratory, CT and brain MRI were normal. In the first sample of CSF, there was no change. Subsequent sample after 14 days revealed high protein with albuminocytological dissociation. The NCS and EMG were normal. Anti GQ 1b antibody was negative. He showed marked improvement with conservative management. Conclusion: MFS is a rare disease that must be diagnosed with the clinical findings and in the following days the diagnosis can be supported by the laboratory findings. Bangladesh Journal of Neuroscience 2016; Vol. 32 (1): 47-51

Manifestation of cognitive impairments after a prior coronavirus infection COVID-19 in a patient with ApoE ε4 genotype

2021 ◽  
Vol 26 (2) ◽  
pp. 25-29
Author(s):  
M. S. Novikova ◽  
V. V. Zakharov ◽  
N. V. Vakhnina
Keyword(s):  
Cognitive Impairment ◽  
Mild Cognitive Impairment ◽  
Small Vessel Disease ◽  
Brain Mri ◽  
Periventricular White Matter ◽  
Everyday Activity ◽  
Apoe Ε4 ◽  
Coronavirus Infection ◽  
Novel Coronavirus

Nowadays, the novel coronavirus infection (COVID-19) pandemic is one of the most important global health problems. There is increasing evidence that COVID-19 affects central and peripheral nervous system as well. The paper presents a clinical case of a 47 old patient with the ApoE ε4 haplotype and family history of Alzheimer’s disease who developed cognitive impairment after acute COVID-19. Before the infection the patient has no cognitive complaints and preserved everyday activity. After novel coronavirus infection, which was observed in mild form, the patient had started to complain on constant excessive forgetfulness. Neuropsychological assessment confirmed the presence of pre-mild cognitive impairment of predominantly single domain amnestic type. However, brain MRI showed only subtle periventricular white matter changes usually attributed to small vessel disease. Memory complaints were observed for 3 months of follow up despite intensive cognitive training, optimization of lifestyle and therapy with choline alphoscerate. Probable links between coronavirus infectious and cognitive impairment manifestation are discussed. There is data that ApoE ε4 haplotype is associated with increase of microglia mediated neuro-inflammation and it can be significant for accelerating of progression of neurodegenerative diseases after COVID-19. Further follow up of the patient is necessary for determination of nosological diagnosis explaining manifested predominantly amnestic type pre-mild cognitive impairment.

Abstract 193: Hemophagocytic lymphohistiocytosis following kawasaki disease:Differential Diagnosis in IVIG Refractory Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Hyun Ok Jun ◽  
Eun Kyung Cho ◽  
Jeong Jin Yu ◽  
So Yeon Kang ◽  
Chang Deok Seo ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Coronary Artery ◽  
Kawasaki Disease ◽  
Skin Rash ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Symptoms ◽  
Early Recognition ◽  
Cervical Lymphadenopathy ◽  
Inflammatory Disorder ◽  
Laboratory Findings

Introduction: Hemophagocytic lymphohistiocytosis(HLH) is a systemic inflammatory disorder characterized by uncontrolled histiocytic proliferation, hemophagocytosis and up-regulation of inflammatory cytokines. Thus, both HLH and Kawasaki disease(KD) are characterized by prolonged fever, and are diagnosed by a clinical and laboratory scoring system, concurrent manifestation of HLH and KD has been described in the literature. We describe two cases of children who diagnosed as KD initially, but after intravenous gamma globulin(IVIG) failed to produce clinical response, were found to have HLH. Case report: A 3-year-old boy who had previous KD history 5 months ago was admitted for 9day fever and skin rash. His symptoms were fulfilled KD criteria, and echocardiography showed dilated right coronary artery of 4.2mm. He was treated with 2 cycles of IVIG until fever subsided. However, 2 days later, he got fever again and cytopenia(Hb<9.0), hypertriglyceridemia, high level of ferritin was shown and had splenomegaly on physical examination. In the suspicion of HLH, bone marrow biopsy was done and revealed hemophagocytosis, consistent with HLH. A second case of 11-month-old boy admitted for 8-day fever with Kawasaki feature. Although, he showed incomplete feature(fever, skin rash, conjunctival injection, cervical lymphadenopathy), echocardiography showed dilated left main coronary artery(3.5mm) and treated with IVIG. However, 2days after IVIG administration, he was still pyrexial. The laboratory findings fulfilled 5 diagnostic criteria of HLH; bicytopenia(anemia, thrombocytopenia), hypofibrinogenemia, hyperferritinemia, hemophagocytosis in bone marrow, raised level of soluble IL-2 receptor. In both cases, the patients treated according to the HLH protocol 2004, and after that clinical symptoms and laboratory findings were improved. Several causes of febrile illness, EBV, CMV, rubella, parvo-viral infection, for example, were excluded. Comment: There is considerable overlap between the clinical syndromes of KD and HLH and early recognition and treatment of these two disease entity is imperative to avoid fatal outcomes in severe cases. Thus, these should both be considered and excluded in any child with unremitting fever and rash.

Behçet’s syndrome

2010 ◽  
pp. 3684-3687
Author(s):  
Hasan Yazici ◽  
Sebahattin Yurdakul ◽  
Izzet Fresko
Keyword(s):  
Clinical Laboratory ◽  
Central Nervous System Involvement ◽  
Arterial Occlusion ◽  
Inflammatory Disorder ◽  
Behçet’S Syndrome ◽  
Laboratory Findings ◽  
Behçet's Syndrome ◽  
Oral Ulcers ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Behçet’s syndrome is an inflammatory disorder of unknown aetiology that involves arteries and veins of all sizes. Most cases are from the countries around the Mediterranean basin, the Middle East and east Asia, with the highest prevalence in Turkey. Clinical features—the disease typically presents in the second and third decades with recurrent oral ulcers (98% of cases), genital ulcers (85%), acneiform lesions (85%), pathergy reaction (60% in some countries), erythema nodosum (50%), uveitis (50%), arthritis (50%), thrombophlebitis (30%), and less commonly with arterial occlusion/aneurysm, central nervous system involvement or gastrointestinal lesions. A relapsing/remitting course is usual. Disease is more severe and mortality is higher in men. The diagnosis is clinical, laboratory findings are nonspecific and there is no specific diagnostic test for Behçet’s syndrome....

scholarly journals Successful treatment of Yersinia pseudotuberculosis hepatitis in a cat presenting with neurological abnormalities

2019 ◽  
Vol 5 (1) ◽  
pp. 205511691985364 ◽  
Author(s):  
Dan Thompson
Keyword(s):  
Yersinia Pseudotuberculosis ◽  
Total Bilirubin ◽  
Brain Mri ◽  
In Situ Hybridisation ◽  
Clinical Signs ◽  
Reference Interval ◽  
Acute Onset ◽  
Fluid Analysis ◽  
Neurological Abnormalities ◽  
Veterinary Antimicrobials

Case summary A 3-year-7-month-old female neutered domestic shorthair cat was presented for further investigation of acute-onset neurological abnormalities, including marked decreased mentation, ataxia and abnormal cranial nerve responses, with concurrent marked pyrexia (40ºC). Initial blood testing was non-specific with mild-to-moderate increases in alanine aminotransferase (ALT) (194 IU/l; reference interval [RI] 17–62 IU/l), aspartate aminotransferase (AST; 150 IU/l [RI 0–51 IU/l]) and total bilirubin (20 µmol/l; RI 0–11 µmol/l), and neutropenia (1.17 ×109/l; RI 2.5–12.5 ×109/l). Brain MRI and cerebrospinal fluid analysis were unremarkable and Toxoplasma serology was negative. Worsening of hepatic biochemical parameters (ALT 265 IU/l, AST 205 IU/l, total bilirubin 42.9 µmol/l) led to further investigations for liver disease, including ultrasound, fine-needle aspirate cytology, histology, fluorescent in situ hybridisation and culture of liver tissue and bile, resulting in a diagnosis of Yersinia pseudotuberculosis hepatitis. The cat was treated with a combination of potentiated amoxicillin (62.5 mg PO q12h), marbofloxacin (5mg PO q24h) and combined s-adenosyl methionine (SAMe)/silybin (90 mg PO q24h), and made a full recovery. Follow-up over 14 months identified a persistent mild increase in ALT, despite no apparent ongoing disease. Relevance and novel information Yersinia pseudotuberculosis hepatitis should be considered as a differential diagnosis in cats presenting with acute-onset neurological signs, and, when diagnosed, can be successfully treated with a combination of marbofloxacin, potentiated amoxicillin and SAMe/silybin. This is the first such case treated successfully with licensed veterinary antimicrobials and the first instance where Y pseudotuberculosis hepatitis has presented with primarily neurological clinical signs.

Sign in / Sign up

Export Citation Format

Share Document