Goose Bumps and Memory Loss

2021 ◽  
pp. 96-97
Author(s):  
Jeffrey W. Britton ◽  
Bhavya Narapureddy ◽  
Divyanshu Dubey
Keyword(s):  
Mycophenolate Mofetil ◽  
Cognitive Dysfunction ◽  
Immunoglobulin G ◽  
Oral Prednisone ◽  
Memory Loss ◽  
Oligoclonal Bands ◽  
Fluid Analysis ◽  
Autoimmune Epilepsy ◽  
Fluid Attenuated Inversion Recovery ◽  
Interictal Discharges

A 46-year-old man had an episode of loss of awareness while driving home. He was found in a cul de sac by his neighbor and was acting confused. He was brought to the emergency department; while there, he started having recurrent episodes of goose bumps (goose flesh) involving half of his body associated with a “wavelike” sensation that would typically begin in the lower extremities and spread upward. He also had some speech difficulty. Physical and neurologic examinations were unremarkable, except for mild cognitive deficits. Video electroencephalographic monitoring showed frequent independent left and right temporal ictal and interictal discharges. Magnetic resonance imaging of the brain showed fluid-attenuated inversion recovery hyperintensity in the bilateral hippocampi. Cerebrospinal fluid analysis showed a mildly increased total protein concentration but no supernumerary oligoclonal bands and normal nucleated cell count and immunoglobulin G index. Serum autoimmune epilepsy evaluation was remarkable for leucine-rich, glioma-inactivated protein 1-immunoglobulin G seropositivity. The patient was diagnosed with leucine-rich, glioma-inactivated protein 1- immunoglobulin G antibody–associated autoimmune seizures presenting with pilomotor seizures. Before autoimmune work-up, the patient had been treated with a gradual escalation of antiseizure medications. The seizures continued. He was subsequently started on intravenous methylprednisolone. He was seizure free and was transitioned to an oral prednisone taper. He was subsequently started on a 6-week regimen of intravenous immunoglobulin. He was also started on mycophenolate mofetil. On follow-up clinic visits, the patient had no recurrence of seizures and disclosed no cognitive dysfunction except for mild inattention. Three years after the patient’s initial episode, the antiseizure medications and mycophenolate mofetil were gradually tapered, without recurrence. Leucine-rich, glioma-inactivated protein 1-immunoglobulin G–associated autoimmunity is typically seen among older patients (>50 years), more commonly men. Symptoms commonly include seizures and cognitive dysfunction, presenting as memory loss and disorientation.

Personality Changes, Cognitive Decline, and Jerking Movements

2021 ◽  
pp. 80-82
Author(s):  
A. Sebastian Lopez Chiriboga
Keyword(s):  
Cognitive Decline ◽  
Oral Prednisone ◽  
Clonic Seizure ◽  
Substantial Improvement ◽  
Fluid Analysis ◽  
Epileptiform Discharges ◽  
Abnormal Movements ◽  
Positron Emission ◽  
Personality Changes ◽  
Fluid Attenuated Inversion Recovery

A 64-year-old man had development of abnormal movements characterized by grimacing of the left hemiface and posturing of the ipsilateral arm. He had a generalized tonic-clonic seizure, followed by cognitive decline requiring assistance for most activities of daily living. He was readmitted to the hospital for neurologic evaluation. His heart rate was 120 beats/min, he was afebrile. On neurologic examination, he had a Mini-Mental State Examination (MMSE) score of 16/30. The patient was oriented to person only, and he had multiple, frequent, left hemibody jerks during examination. Cerebrospinal fluid analysis indicated increased protein concentration but otherwise normal findings. Laboratory abnormalities included a serum sodium and antibodies to leucine-rich, glioma-inactivated protein 1. Electroencephalography revealed diffuse slowing and bilateral temporal lobe epileptiform discharges. Magnetic resonance imaging of the brain showed T2/fluid-attenuated inversion recovery hyperintensities in the bilateral hippocampi and amygdalae and gadolinium enhancement in the same regions. Brain 18F-fludeoxyglucose–positron emission tomography demonstrated hypermetabolism in the mesiotemporal lobes bilaterally. A diagnosis of limbic encephalitis associated with leucine-rich, glioma-inactivated protein 1-immunoglobulin G antibodies was. The unilateral facial and arm movements were consistent with faciobrachial dystonic seizures and are pathognomonic of the disorder. The patient’s seizure frequency did not improve with antiepileptic drug therapy. The patient was treated with intravenous methylprednisolone, followed by plasmapheresis. This resulted in substantial improvement of his cognitive dysfunction and decrease in the faciobrachial dystonic seizures frequency. Therapy was transitioned to oral prednisone and the patient was discharged from the hospital. This patient had classic features of leucine-rich, glioma-inactivated protein 1 antibody encephalitis: faciobrachial dystonic seizures, personality changes, subacute cognitive decline, hyponatremia, and improvement with immunotherapy.

Progressive Leukodystrophy-Like Demyelinating Syndromes with MOG-Antibodies in Children: A Rare Under-Recognized Phenotype

2021 ◽  
Author(s):  
Elise Yazbeck ◽  
Hélène Maurey ◽  
Carole Leroy ◽  
Philippe Horellou ◽  
Silvia Napuri ◽  
...  
Keyword(s):  
Brain Magnetic Resonance Imaging ◽  
Brain Biopsy ◽  
Active Immunotherapy ◽  
Oligoclonal Bands ◽  
Second Child ◽  
Fluid Analysis ◽  
Appropriate Treatment ◽  
Neurological Prognosis ◽  
Mog Antibodies

AbstractAcquired demyelinating syndromes (ADS) are frequently associated with myelin oligodendrocytes glycoprotein (MOG) antibodies in children. Clinical phenotypes are heterogeneous and may delay the diagnosis, especially when they relapse and are atypical, mimicking diseases such as multiple sclerosis or neuromyelitis optica spectrum disorders . Here, we describe two children: one with a progressive cognitive and behavioral deterioration with seizures after only one relapse and the other with similar clinical impairments associated with multiple relapses. Brain magnetic resonance imaging revealed a subsequent progressive leukodystrophy-like lesion with diffuse bilateral white matter injuries in both patients. Cerebrospinal fluid analysis showed pleiocytosis, increased level of proteins with no oligoclonal bands. Metabolic and inflammatory blood markers were all negative. Brain biopsy was performed in the second child and nonspecific inflammatory lesions with no argument for histiocytosis or tumor were observed. Clinical and radiological stabilization were obtained after active immunotherapy. Retrospective analysis of anti-MOG antibodies in these two children was positive at the earlier stage of the disease and turned negative after treatment and during follow-up. Leukodystrophy-like ADS with anti-MOG-antibodies may display distinct progressive phenotype and have a severe neurological prognosis. Early diagnosis and appropriate treatment may improve outcome in these children.

A Septuagenarian With Progressive Hemiparesis

2021 ◽  
pp. 48-50
Author(s):  
Roman Kassa ◽  
B. Mark Keegan
Keyword(s):  
Multiple Sclerosis ◽  
Late Onset ◽  
Progressive Multiple Sclerosis ◽  
Oligoclonal Bands ◽  
Hyperintense Lesion ◽  
Fluid Analysis ◽  
Hemiparetic Gait ◽  
Magnetic Resonance Imaging Mri ◽  
Disease Modifying Agents ◽  
The Right

A 78-year-old man with no pertinent medical history sought care for an 18-month history of progressive right lower extremity weakness, gait impairment, and falls. On neurologic examination, he had a hemiparetic gait. He had normal higher cognitive function and cranial nerve function. Motor examination showed decreased bulk over the right hand with no fasciculations, mild spasticity over the right leg, and right hemiparesis with an upper motor neuron pattern. Deep tendon reflexes were brisk throughout his limbs, and he had an extensor plantar reflex on the right side. He had impaired vibratory sense at the toes, with otherwise normal sensory and coordination examinations. Magnetic resonance imaging (MRI) of the brain showed ovoid periventricular and punctate subcortical and deep white matter T2 hyperintense foci. Some of these had corresponding T1 hypointensity. MRI of the cervical spine showed 1 eccentrically located T2 hyperintense lesion over the right lateral aspect of C2. Cerebrospinal fluid analysis showed no pleocytosis, an increased protein concentration of 66 mg/dL, and 4 unique oligoclonal bands. A diagnosis of primary progressive multiple sclerosis, very late onset, was made. With any diagnosis of late-onset multiple sclerosis, a decision about whether multiple sclerosis disease-modifying agents are indicated should be carefully considered. Our older patient had a progressive disease course, and neuroimaging studies did not reveal evidence of active disease. Based on this, a decision was made to monitor him clinically and radiologically. Management of spasticity with regular daily stretching exercises was discussed with him. A first clinical manifestation of multiple sclerosis can occur at a later-than-typical age. Most studies consider an onset at age 50 years or older to be late-onset multiple sclerosis, whereas first symptoms occurring at age 60 years or older are commonly referred to as very late–onset MS.

Severe Monocular Vision Loss Followed by Gait Difficulty

2021 ◽  
pp. 35-38
Author(s):  
Brian G. Weinshenker
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Immunoglobulin G ◽  
Vision Loss ◽  
Position Sense ◽  
Aquaporin 4 ◽  
Resonance Imaging ◽  
Gadolinium Enhancement ◽  
Fluid Analysis ◽  
Severe Impairment

A 56-year-old woman sought care for painless vision loss over 24 hours in the superior field of her right eye, which progressed to total vision loss. Intravenous corticosteroids were administered over 5 days, and she recovered well. Approximately 6 months later, paresthesias and sensory loss of her legs developed, which was sufficiently severe that she was unable to walk. She had severe impairment of vibration sense and lesser impairment of position sense, as well as proximal weakness of both legs. She recovered but had persistent burning dysesthesias of the legs. Magnetic resonance imaging of the orbits at the onset of symptoms showed gadolinium enhancement extending from the middle of the orbit posteriorly, almost to the level of the optic chiasm. Magnetic resonance imaging of the spine at the time of acute myelitis revealed a long spinal cord lesion extending from the lower cervical cord to the conus which was central and homogeneous on T2 images and exhibited patchy gadolinium enhancement. Cerebrospinal fluid analysis showed 37 leukocytes/µL with lymphocyte predominance and negative tests for oligoclonal bands. On serologic analysis, she was positive for aquaporin-4-immunoglobulin G antibodies by enzyme-linked immunosorbent assay. The patient was diagnosed with neuromyelitis optica spectrum disorder, aquaporin-4-immunoglobulin G seropositive. The patient was started on rituximab treatment. After 2 years, she decided to discontinue treatment because she was concerned about potential adverse effects. Six months after discontinuing rituximab, severe weakness of her right leg developed, which improved with intravenous corticosteroids. Rituximab therapy was restarted, 2 doses of 1 g each, but she had 1 further episode of myelitis 2 weeks after receiving rituximab. Six months later, her visual acuity in the right eye was only counting fingers, and she had a flaccid paraplegia with areflexia of the lower extremities and total loss of sensory function below T4.

Progressive Spasticity With a Single Magnetic Resonance Imaging Lesion

2021 ◽  
pp. 20-21
Author(s):  
Samantha A. Banks ◽  
Eoin P. Flanagan
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Sclerosis ◽  
Magnetic Resonance ◽  
Oligoclonal Bands ◽  
Resonance Imaging ◽  
Fluid Analysis ◽  
Skin Cancers ◽  
Clinical Episode ◽  
History Of ◽  
The Right

A 59-year-old White man with a history of excised basal and squamous cell skin cancers was evaluated for gait difficulties. He had erectile dysfunction but no bowel or bladder dysfunction. He also reported fatigue. He began using a cane for ambulation 2 weeks before evaluation at our facility. His medications included vitamin D and sildenafil. He was a lifelong nonsmoker and had no family history of multiple sclerosis. Neurologic examination at the time of our evaluation 3 years after onset was notable for a positive Hoffman sign on the right and mild weakness of the right triceps but preserved strength elsewhere. He had a spastic gait with moderate spasticity in both lower extremities, hyperreflexic patellar and ankle jerks bilaterally, and bilateral positive Babinski sign. The remainder of the examination was essentially normal. Magnetic resonance imaging of the brain showed a single lesion at the cervicomedullary junction and medullary pyramids, more prominent on the right. There was also some accompanying atrophy that was also visible on cervical spine magnetic resonance imaging. Results of cerebrospinal fluid analysis showed a normal white blood cell count, increased protein concentration (108 mg/dL), and positive oligoclonal bands. The progressive nature of his symptoms, spinal fluid results, and lesion appearance were all consistent with a diagnosis of progressive solitary sclerosis. At the time this patient was seen, no immunomodulatory medications for progressive solitary sclerosis were approved, so no immunomodulatory medication was tried. Ongoing symptomatic management was recommended. Progressive solitary sclerosis is a rare variant of multiple sclerosis in which patients have a single central nervous system demyelinating lesion and development of motor progression attributable to that lesion. Patients can initially have a clinical episode followed by progression or can have a progressive course without an initial relapse.

scholarly journals Bilateral Retrobulbar Optic Neuritis as the First Manifestation of Neuro-Behçet Disease

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Mohsen Jari ◽  
Taiiebeh Mohammadi ◽  
Ensiyeh Taheri
Keyword(s):  
Optic Neuritis ◽  
Brain Magnetic Resonance Imaging ◽  
Human Leukocyte ◽  
Oligoclonal Bands ◽  
Behçet Disease ◽  
Normal Brain ◽  
Behcet Disease ◽  
Fluid Analysis ◽  
Acute Optic Neuritis ◽  
Retrobulbar Optic Neuritis

Background. Behçet disease (BD) is a polygenic and chronic autoinflammatory multisystem vasculitis. Acute optic neuritis has been rarely reported in patients with BD, especially in children. Case Presentation. We reported an 8-year-old girl with a sudden visual loss and color vision impairment. The patient had a history of recurrent oral aphthous ulcers, genital ulcers, and chronic abdominal pain. On ophthalmic examination, anterior and posterior chambers and funduscopy of both eyes were normal. The results of laboratory tests for infectious and rheumatic diseases were normal. Brain magnetic resonance imaging and the result of cerebrospinal fluid analysis for oligoclonal bands and auto-antibodies were also normal. Pathergy skin test and human leukocyte antigens (HLA) B5 and HLA-B51 were positive. The patient was recognized as a case of BD-related bilateral retrobulbar optic neuritis and was treated by corticosteroids, azathioprine, colchicine, and infliximab. Conclusion. Retrobulbar optic neuritis may be the first manifestation of neuro-BD.

scholarly journals Dementia with Leukoencephalopathy in Systemic Lupus Erythematosus

1991 ◽  
Vol 18 (3) ◽  
pp. 344-348 ◽  
Author(s):  
Andrew Kirk ◽  
Andrew Kertesz ◽  
Marsha J. Polk
Keyword(s):  
White Matter ◽  
Lupus Erythematosus ◽  
Memory Loss ◽  
Cerebral Atrophy ◽  
Hepatic Function ◽  
Subcortical White Matter ◽  
Oligoclonal Bands ◽  
Mri Findings ◽  
Recent Onset ◽  
Blood Brain Barrier Disruption

ABSTRACT:Neurologic manifestations, afflicting up to 70% of SLE patients, include psychosis, seizures, chorea, neuropathies, and stroke. MRI is useful in evaluation of lupus patients and several reports have documented cerebral atrophy or focal hyperintensities. We report an unusual MRI appearance in a 56-year-old woman with SLE, diagnosed on the basis of pleuritis, lymphopenia, anti-DNA antibodies, and neurologic involvement. She reported recent onset of Raynaud's phenomenon and generalized macular rash. She presented after two months of gradual deterioration with memory loss, flattened affect, dysphagia, dysarthria, anomia, and somnolence, without focal neurologic signs. Investigations included elevated ESR, reduced complement, normal CSF without oligoclonal bands, negative viral serology, normal hormone and vitamin levels, normal renal and hepatic function. Neuropsychologic testing showed widespread impairment (WAIS-R: FSIQ-63; WMS-69; DRS-98; RCPM-14; WAB AQ-78.8). CT was normal but MRI showed strikingly symmetric, confluent hyperintensities extensively involving cerebral and cerebellar white matter on Tl and T2 weighted scans. Basal ganglia and subependymal and subcortical white matter were spared. Treated with prednisone, the patient made a gradual, but incomplete, recovery. These MRI findings may reflect widespread vasculopathy or direct immunologic brain insult with or without imunologic blood-brain barrier disruption.

scholarly journals Neuropsychological Dysfunction in the Absence of Structural Evidence for Cerebral Ischemia after Uncomplicated Carotid Endarterectomy

Neurosurgery ◽  
2006 ◽  
Vol 58 (3) ◽  
pp. 474-480 ◽  
Author(s):  
Eric J. Heyer ◽  
Robert DeLaPaz ◽  
Hadi J. Halazun ◽  
Anita Rampersad ◽  
Robert Sciacca ◽  
...  
Keyword(s):  
Carotid Endarterectomy ◽  
Cognitive Dysfunction ◽  
Neuropsychological Tests ◽  
High Signal ◽  
Neurocognitive Dysfunction ◽  
Neuropsychological Dysfunction ◽  
Cognitive Evaluation ◽  
Apparent Diffusion ◽  
Fluid Attenuated Inversion Recovery ◽  
Apparent Diffusion Coefficient Maps

Abstract OBJECTIVE: Neurocognitive dysfunction has been shown to occur in roughly 25% of patients undergoing carotid endarterectomy (CEA). Despite this, little is known about the mechanism of this injury. Recently, several groups have shown that new diffusion weighted imaging (DWI)-positive lesions are seen in 20% of patients undergoing CEA. We investigated to what degree neurocognitive dysfunction was associated with new DWI lesions. METHODS: Thirty-four consecutive patients undergoing CEA were subjected to pre- and postoperative cognitive evaluation with a battery of neuropsychological tests. Postoperative magnetic resonance imaging was performed in all patients within 24 hours of surgery. Lesions that showed high signal on DWI and restricted diffusion on apparent diffusion coefficient maps but no abnormal high signal on the fluid-attenuated inversion recovery images were considered hyperacute. RESULTS: Cognitive dysfunction was seen in eight (24%) patients. New hyperacute DWI lesions were seen in three (9%). Only one (13%) of the patients with cognitive dysfunction had a new DWI lesion. Two thirds of the new DWI lesions occurred in the absence of cognitive deterioration. Patients with cognitive dysfunction had significantly longer carotid cross-clamp times. CONCLUSION: Neurocognitive dysfunction after CEA does not seem to be associated with new DWI positive lesions.

scholarly journals Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation

2012 ◽  
Vol 4 (1) ◽  
pp. 5 ◽  
Author(s):  
Go Makimoto ◽  
Yasuhiro Manabe ◽  
Chizuru Yamakawa ◽  
Daiki Fujii ◽  
Yasuko Ikeda-Sakai ◽  
...  
Keyword(s):  
High Intensity ◽  
Skin Lesions ◽  
Pulse Therapy ◽  
Initial Manifestation ◽  
Diffusion Weighted Images ◽  
Fluid Analysis ◽  
Fluid Attenuated Inversion Recovery ◽  
Left Caudate ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

We report 2 cases that were considered to be neuro-Sweet disease. They initially manifested with meningoencephalitis and no skin lesions, and rapidly improved with corticosteroid therapy. In both cases, patients complained of meningitic symptoms such as fever and headache, and HLA-B54 and -Cw1 turned out to be positive over the clinical course. Cerebrospinal fluid analysis showed increased levels of lymphocytes and protein. In case #1, fluid-attenuated inversion recovery (FLAIR), magnetic resonance imaging (MRI) and diffusion-weighted images (DWI) showed high-intensity signals in the right dorsal medulla oblongata, bilateral dorsal midbrain, and left thalamus. In case #2, FLAIR and DWI showed high-intensity signals in the bilateral cerebellar cortex and left caudate nucleus. Symptoms and MRI images were markedly improved in both cases after corticosteroid pulse therapy. According to published diagnostic criteria, these 2 cases were considered possible neuro-Sweet disease. These cases suggest that the combination of meningoencephalitis and HLA specificity is important to consider the possibility of neuro-Sweet disease, even without skin lesions.

scholarly journals Dysfunction of bloodbrain barrier and intrathecal immune response in patients with clinically isolated multiple sclerosis syndrome

2019 ◽  
Vol 27 (2 (99)) ◽  
pp. 20-22
Author(s):  
Olga Shulga
Keyword(s):  
Multiple Sclerosis ◽  
Blood Brain Barrier ◽  
Immunoglobulin G ◽  
Clinically Isolated Syndrome ◽  
Brain Barrier ◽  
Oligoclonal Bands ◽  
Barrier Dysfunction ◽  
Intrathecal Synthesis ◽  
Blood Brain ◽  
Increased Risk

The study is devoted to the evaluation of blood-brain barrier dysfunction and intrathecal synthesis of immunoglobulin G in patients with clinically isolated syndrome (CIS) of multiple sclerosis. The presence of oligoclonal bands and immunoglobulin G indicates an increased risk of multiple sclerosis in patients with CIS, regardless of the presence of other risk factors. An observational study of an intranasal response in patients with CIS was performed for the fi rst time in Ukraine. Based on the evaluation of cerebrospinal fl uid and blood parameters in 19 adult patients, it was found that 68 % of patients had an impairment of permeability of the blood-brain barrier. Synthesis of intrathecal immunoglobulin G is present in two thirds of cases. Key words: clinically isolated syndrome, multiple sclerosis, blood-barrier, intrathecal synthesis, immunoglobulin G

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