Delayed post-hypoxic leukoencephalopathy: Case report

IntroductionDelayed post-hypoxic leukoencephalopathy (DPHL) is an underrecognized syndrome of delayed demyelination, where patients manifest neuropsychiatric symptoms after a period of 2–40 days of apparent recovery from a cerebral hypo-oxygenation episode.ObjectivesWe report a case of a patient who successfully recovered from an overdose of heroin, but then suffered a delayed abrupt neurological deterioration.AimsTo improve assessment and recognition of DPHL.MethodsAn adequate retrospective collection of clinical data and nonsystematic review of the literature was performed.ResultsA 43-year-old male with schizoaffective disorder who attempted suicide with an overdose of heroin, was successfully revived and return to his previously mental status, but 3 weeks after, he abruptly developed progressive cognitive impairment with akinetic mutism and ataxia. He was admitted to our acute psychiatric unit after brain CT and chemistry analyses were unremarkable. Brain MRI showed diffusely symmetric hyperintensity in the white matter (WM), pronominally the periventricular WM, on FLAIR and T2 weighted sequences. At 16 weeks postoverdose, he presented improvement both cognitive and motor symptoms, lasting deficits in frontal-executive functions.DiscussionDPHL is characterized by similar clinical and neuroimaging features regardless of the initial insult. The mean lucid interval coincides with the replacement half-life for myelin related lipids and proteins. Prolonged mild-to-moderate hypo-oxygenation of WM is thought to disrupt myelin turnover. It appears probable that these were responsible for DPHL in our patient rather than a direct toxicity.ConclusionDPHL can be diagnosed when clinical history, laboratory assessments and MRI findings are concordant. DPHL requires extensive support care and carries a relatively good prognosis.Disclosure of interestThe authors have not supplied their declaration of competing interest.

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The squeezing snake, a psychiatric presentation of epilepsy: A case report

European Psychiatry ◽

10.1016/j.eurpsy.2017.02.317 ◽

2017 ◽

Vol 41 (S1) ◽

pp. S347-S347

Author(s):

M. Mangas ◽

L. Bravo ◽

Y. Martins ◽

A. Matos Pires

Keyword(s):

Psychiatric Disorders ◽

Medical History ◽

Psychiatric Symptoms ◽

Neuropsychiatric Symptoms ◽

Brain Mri ◽

Outpatient Service ◽

Clinical History ◽

Depressive Mood ◽

Night Time ◽

Competing Interest

IntroductionEpilepsy is considered a complex neurological disorder with a great variety of clinical presentations that can resemble psychiatric disorders.ObjectivesDisclose an unusual clinical case with psychiatric symptoms as the presentation of epilepsy.MethodsPsychiatric assessments and retrospective review of the clinical file and literature research.ResultsA 40-years-old Romanian woman presented to the psychiatry outpatient service with a history of persistent depressive mood and disturbed sleep for the past 3 years, complaining of a feeling that she described as “a snake squeezing around her body, starting in her left leg and spreading to the rest of her body up to the neck” associated with a sense of pins and needles, occurring during night time. She attended general practice, neurosurgery and psychiatry appointments. Her medical history included “gastritis” and lower left leg fracture and a pituitary microadenoma revealed in brain CT-scan. Blood work, including endocrine tests and brain-MRI were normal. Her symptoms initially led to diagnosis of: anxiety, somatization, Ekbom syndrome and depression. She was treated with antidepressives, antipsychotics and anxiolytics, without response. After careful reconstruction of the clinical history and further analyses of her complaints, the diagnosis of focal sensory jacksonian seizure was made. Levetiracetam introduction led to symptomatic remission.ConclusionEpilepsy includes a variety of neuropsychiatric symptoms. This case illustrates that epileptic patients may experience non-convulsive seizures that might be mistaken as primary psychiatric disorders. Neurologists and psychiatrists must be aware of this varied presentation while obtaining the medical history in order to investigate and manage this patient effectively.Disclosure of interestThe authors have not supplied their declaration of competing interest.

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Different MRI patterns in MS worsening after stopping fingolimod

Neurology Neuroimmunology & Neuroinflammation ◽

10.1212/nxi.0000000000000566 ◽

2019 ◽

Vol 6 (4) ◽

pp. e566 ◽

Cited By ~ 5

Author(s):

Caterina Lapucci ◽

Damiano Baroncini ◽

Maria Cellerino ◽

Giacomo Boffa ◽

Ilaria Callegari ◽

...

Keyword(s):

Clinical Features ◽

Brain Mri ◽

Activity Level ◽

Lesion Volume ◽

Mri Findings ◽

Volume Increase ◽

Mri Features ◽

Disability Status ◽

The Mean

ObjectiveTo analyze MRI images in patients with MS who experienced worsening of neurologic status (WNS) after stopping fingolimod (FTY).MethodsIn this retrospective study, demographic, clinical, and radiologic data of patients with MS who experienced WNS after stopping FTY were retrospectively collected. We introduced the “δExpanded Disability Status Scale (EDSS)-ratio” to identify patients who, after FTY withdrawal, showed an inflammatory flare-up exceeding the highest lifetime disease activity level. Patients with δEDSS-ratio > 1 were enrolled in the study.ResultsEight patients were identified. The mean (SD) age of the 8 (7 female) patients was 35.3 (4.9) years. The mean FTY treatment duration was 3.1 (0.8) years. The mean FTY discontinuation–WNS interval was 4 (0.9) months. The 4 patients with δEDSS-ratio ≥ 2 developed severe monophasic WNS (EDSS score above 8.5), characterized by clinical features and MRI findings not typical of MS, which we classified as “tumefactive demyelination pattern” (TDL) and “Punctuated pattern” (PL). Conversely, patients whose δEDSS-ratio was between 1 and 2 had clinical features and brain MRI compatible with a more typical, even if aggressive, MS relapse. In patients with TDL and PL, the flare-up of inflammatory activity led to severe tissue damage resulting in T2 but also T1 lesion volume increase at 6-month follow-up.ConclusionsPeculiar MRI features (TDL and PL), different from a typical MS flare-up, might occur in some patients who experienced WNS after stopping FTY. Further studies, also involving immunologic biomarkers, are necessary to investigate TDL or PL pathophysiology.

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Posterior Reversible Encephalopathy Syndrome Presenting with Atypical Findings: Report of Two Cases

Case Reports in Neurological Medicine ◽

10.1155/2018/7835415 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5

Author(s):

Paolo Cerrone ◽

Patrizia Sucapane ◽

Rocco Totaro ◽

Simona Sacco ◽

Antonio Carolei ◽

...

Keyword(s):

Posterior Reversible Encephalopathy Syndrome ◽

Vision Loss ◽

Brain Mri ◽

Asymmetric Distribution ◽

Mri Findings ◽

Posterior Reversible Encephalopathy ◽

Atypical Symptoms ◽

Severe Impairment ◽

Reversible Encephalopathy ◽

Weighted Sequences

Background. Posterior reversible encephalopathy syndrome (PRES) is characterized by a variable association of symptoms including headache, consciousness impairment, visual disturbances, seizures, and focal neurological signs. Treating the underlying cause usually leads to partial or complete resolution of symptoms within days or weeks. Brain MRI findings include hyperintensities on T2-weighted sequences and their reversibility on follow-up exams. We describe two patients, one with an atypical clinical presentation characterized by a severe and prolonged impairment of consciousness and the other with atypical neuroimaging findings. Case Presentation. The first patient was a 42-year-old woman, with a negative medical history, presenting with seizures, lethargy, and left hemiparesis, 60 hours after uncomplicated delivery. Brain MRI showed an atypical pattern of alterations, with patchy asymmetric distribution in all lobes. Symptoms completely resolved after twelve days. The second patient was a 59-year-old woman with a history of hypertension, presenting with severe impairment of consciousness, vision loss, and seizures. Symptoms partially resolved after three weeks. Conclusion. PRES is characterized by reversible symptoms and radiological findings. Brain MRI usually shows widespread oedema in white matter with typical patterns. The cases we described suggest that PRES may presents with atypical symptoms and radiological manifestations, mimicking other neurological conditions.

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Atypical cognitive dysfunction due to brain damage: A case report

European Psychiatry ◽

10.1016/j.eurpsy.2016.01.1984 ◽

2016 ◽

Vol 33 (S1) ◽

pp. S536-S536

Author(s):

C.A. Welte-Santana ◽

A.F. Macedo de Queiroz ◽

N. Merola Fontoura ◽

C. Lima de Melo ◽

M. Ribeiro Garcia de Rezende ◽

...

Keyword(s):

Retrograde Amnesia ◽

Brain Mri ◽

Brain Lesion ◽

Clinical History ◽

Public Hospitals ◽

Patient Reported ◽

History Of ◽

Organic Brain ◽

Competing Interest ◽

One Year

IntroductionThe diagnosis of a primary psychiatric disorder requires the exclusion of an organic etiology. However, Brazilian public hospitals commonly lack resources. Diagnostic precision requires also the congruence of the clinical history and the natural history of the investigated disorder.ObjectivesThis study reports a rare case of hallucinations and retrograde amnesia, due to organic brain lesion but without other cognitive impairments.Case presentationFifty-three-years-old male Brazilian, was evaluated after one year in Brazil after being missing for 25 years in USA. Encountering his family, he did not recognize his mother, did not remember his life in Brazil, including his infancy, nationality and mother language. He was found as a homeless in poor hygiene. In the exam, he only presented retrograde amnesia, without any disturbances of fixation memory, intelligence, formal thought, affect or psychomotor function. Patient reported hallucinations. Blood tests showed no abnormalities. EEG showed diffuse slow rhythms. Brain MRI showed cortical and hippocampal atrophy. After weekly evaluations for 5 months, he remained stable despite lack of prescription. Some weeks after MRI, patient reported frequent alcohol and inhalant use when missing. No hints of secondary gain were found until present.DiscussionOrganic etiology was suspected due to atypical presentation: hallucinations, evocative amnesia, with no further cognitive and affect disturbances. This is not compatible with schizophrenia, dementia or dissociative disorder. The brain abnormalities and recent data highly suggest this etiological hypothesis.ConclusionSince this clinical presentation does not fit into any specific psychiatric category, the case will continue to be studied.Disclosure of interestThe authors have not supplied their declaration of competing interest.

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Psychotic symptoms in a patient diagnosed with temporal lobe epilepsy and schizoaffective disorder

European Psychiatry ◽

10.1016/j.eurpsy.2016.01.269 ◽

2016 ◽

Vol 33 (S1) ◽

pp. S150-S150

Author(s):

G. Martinez-Ales ◽

V. Baena ◽

I. Rubio ◽

B. Rodriguez-Vega ◽

V. Bonan ◽

...

Keyword(s):

Psychiatric Disorders ◽

Schizoaffective Disorder ◽

Neuropsychiatric Symptoms ◽

Clinical History ◽

Psychotic Symptoms ◽

Antiepileptic Medication ◽

Complex Motor ◽

History Of ◽

Competing Interest ◽

Symptomatic Remission

IntroductionEpilepsy is considered a complex neurological disorder, and its clinical picture can resemble many different cerebral dysfunctions, including those associated to major psychiatric disorders.Case reportWe report the case of a 52-year-old gentleman, with a 30-year history of schizoaffective disorder and of complex partial epilepsy with secondary generalization. He was admitted to an emergency room due to a voluntary overdose with 8 mg of clonazepam. The patient explained how he had recently experienced visual hallucinations and insomnia, symptoms that originally led to the psychotic diagnosis. He had previously presented these symptoms, along with stupor, delusions and lability, as a prodrome of complex motor epileptic decompensations. Thus, he took the overdose not to suffer seizures. After carefully reconstructing the clinical history, psychiatric admissions had shown seizures, and periods of clinical stability had been achieved by regulating antiepileptic medication. Eslicarbazepine and lamotrigine reintroduction, and quetiapine withdrawal, led to symptomatic remission.DiscussionEpilepsy and major psychiatric disorders show a high comorbidity. There has been an effort to even include epilepsy and psychosis in a unique diagnosis (alternant psychosis). Furthermore, polimorphism and restitutio ad integrum may resemble classic cycloid psychosis. In this case, chronological study showed all symptoms could be explained by one disorder.ConclusionEpilepsy includes a variety of neuropsychiatric symptoms. It can be difficult to withdraw psychiatric diagnoses from patients after years of follow-up. However, a carefully taken medical history clarifies temporal criteria.Disclosure of interestThe authors have not supplied their declaration of competing interest.

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Preoperative localization of parathyroid glands

Nuklearmedizin ◽

10.1055/s-0038-1625596 ◽

2004 ◽

Vol 43 (03) ◽

pp. 85-90 ◽

Cited By ~ 27

Author(s):

E. Lopez Hänninen ◽

Th. Steinmüller ◽

T. Rohlfing ◽

H. Bertram ◽

M. Gutberlet ◽

...

Keyword(s):

Image Fusion ◽

Single Photon ◽

Imaging Techniques ◽

Photon Emission ◽

Parathyroid Glands ◽

Preoperative Localization ◽

Mri Findings ◽

99Mtc Mibi ◽

Weighted Sequences ◽

Mibi Scintigraphy

Summary Aim: Minimally invasive resection of hyperfunctional parathyroid glands is an alternative to open surgery. However, it requires a precise preoperative localization. This study evaluated the diagnostic use of magnetic resonance (MR) imaging, parathyroid scintigraphy, and consecutive image fusion. Patients, methods: 17 patients (9 women, 8 men; age: 29-72 years; mean: 51.2 years) with primary hyperparathyroidism were included. Examination by MRI used unenhanced T1- and T2-weighted sequences as well as contrast-enhanced T1-weighted sequences. 99mTc-MIBI scintigraphy consisted of planar and SPECT (single photon emission tomography) imaging techniques. In order to improve the anatomical localization of a scintigraphic focus, SPECT-data were fused with the corresponding MR-data using a modified version of the Express 5.0 software (Advanced Visual Systems, Waltham, MA). Results of image fusion were then compared to histopathology. Results: In 14/17 patients, a single parathyroid adenoma was found. There were 3 cases with hyperplastic glands. MRI detected 10 (71%), scintigraphy 12 (86%) adenomas. Both modalities detected 1/3 patients with hyperplasia. Image fusion improved the anatomical assignment of the 13 scintigraphic foci in five patients and was helpful in the interpretation of inconclusive MR-findings in two patients. Conclusions: Both MRI and 99mTc-MIBI scintigraphy sensitively detect parathyroid adenomas but are less reliable in case of hyperplastic glands. In case of a scintigraphic focus, image fusion considerably improves its topographic assignment. Furthermore, it facilitates the evaluation of inconclusive MRI findings.

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Brain MRI-findings in Ph - negative myeloproliferative disorders

Terapevticheskii arkhiv ◽

10.26442/00403660.2019.07.000329 ◽

2019 ◽

Vol 91 (7) ◽

pp. 29-34 ◽

Cited By ~ 2

Author(s):

M M Tanashyan ◽

A L Melikyan ◽

P I Kuznetsova ◽

A A Raskurazhev ◽

A A Shabalina ◽

...

Keyword(s):

Cerebrovascular Disease ◽

Sinus Thrombosis ◽

Cerebral Arteries ◽

Brain Mri ◽

Myeloproliferative Disorders ◽

Cerebral Venous Sinus Thrombosis ◽

Mri Findings ◽

Cerebral Lesions ◽

Cerebrovascular Pathology ◽

Underlying Mechanisms

Myeloproliferative disorders (MPD) are accompanied by a high proportion of thrombotic complications, which may lead to cerebrovascular disease (CVD). Aim. To describe MRI-findings in patients with Ph - negative MPD and evaluate any cerebrovascular disease. Materials and methods. We included 104 patients with Ph - negative MPD (age varied between 20 and 58) with clinical correlates of cerebrovascular pathology. Results. Brain MRI showed post - stroke lesions in 20% of patients (7 hemispheric infarcts due to thrombotic occlusion of one of the large cerebral arteries, 14 - cortical infarcts). 37 patients (36%) had vascular cerebral lesions. Cerebral venous sinus thrombosis occurred in 5 patients - in 7% (n=3) of patients with polycythemia vera and 5% (n=2) - in patients with essential thrombocythemia. The incidence of vascular cerebral lesions was associated with higher levels of the following: erythrocyte, platelet count, fibrinogen, and with the decrease in fibrinolytic activity, as well. Conclusion. The pioneering results of the study include the description and analysis of brain MRI-findings in patients with Ph - negative MPD. The underlying mechanisms of cerebrovascular pathology in these patients are associated with certain blood alterations (particularly, hemorheology) which present a major risk factor.

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Comparisons of clinical characteristics, brain MRI findings, and responses to epidural blood patch between spontaneous intracranial hypotension and post-dural puncture headache: retrospective study

BMC Neurology ◽

10.1186/s12883-021-02279-5 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Gha-Hyun Lee ◽

Jiyoung Kim ◽

Hyun-Woo Kim ◽

Jae Wook Cho

Keyword(s):

Intracranial Hypotension ◽

Dural Puncture ◽

Epidural Blood Patch ◽

Brain Mri ◽

Spontaneous Intracranial Hypotension ◽

Vein Of Galen ◽

Mri Findings ◽

Post Dural Puncture Headache ◽

Straight Sinus ◽

Blood Patch

Abstract Background Spontaneous intracranial hypotension and post-dural puncture headache are both caused by a loss of cerebrospinal fluid but present with different pathogeneses. We compared these two conditions concerning their clinical characteristics, brain imaging findings, and responses to epidural blood patch treatment. Methods We retrospectively reviewed the records of patients with intracranial hypotension admitted to the Neurology ward of the Pusan National University Hospital between January 1, 2011, and December 31, 2019, and collected information regarding age, sex, disease duration, hospital course, headache intensity, time to the appearance of a headache after sitting, associated phenomena (nausea, vomiting, auditory symptoms, dizziness), number of epidural blood patch treatments, and prognosis. The brain MRI signs of intracranial hypotension were recorded, including three qualitative signs (diffuse pachymeningeal enhancement, venous distention of the lateral sinus, subdural fluid collection), and six quantitative signs (pituitary height, suprasellar cistern, prepontine cistern, mamillopontine distance, the midbrain-pons angle, and the angle between the vein of Galen and the straight sinus). Results A total of 105 patients (61 spontaneous intracranial hypotension patients and 44 post-dural puncture headache patients) who met the inclusion criteria were reviewed. More patients with spontaneous intracranial hypotension required epidural blood patch treatment than those with post-dural puncture headache (70.5% (43/61) vs. 45.5% (20/44); p = 0.01) and the spontaneous intracranial hypotension group included a higher proportion of patients who underwent epidural blood patch treatment more than once (37.7% (23/61) vs. 13.6% (6/44); p = 0.007). Brain MRI showed signs of intracranial hypotension in both groups, although the angle between the vein of Galen and the straight sinus was greater in the post-dural puncture headache group (median [95% Confidence Interval]: 85° [68°-79°] vs. 74° [76°-96°], p = 0.02). Conclusions Patients with spontaneous intracranial hypotension received more epidural blood patch treatments and more often needed multiple epidural blood patch treatments. Although both groups showed similar brain MRI findings, the angle between the vein of Galen and the straight sinus differed significantly between the groups.

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Fatigue in patients with systemic lupus erythematosus and neuropsychiatric symptoms is associated with anxiety and depression rather than inflammatory disease activity

Lupus ◽

10.1177/09612033211005014 ◽

2021 ◽

pp. 096120332110050

Author(s):

Rory C Monahan ◽

Liesbeth JJ Beaart-van de Voorde ◽

Jeroen Eikenboom ◽

Rolf Fronczek ◽

Margreet Kloppenburg ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Lupus Erythematosus ◽

Neuropsychiatric Symptoms ◽

Anxiety And Depression ◽

Systemic Lupus ◽

Sf 36 ◽

Neuropsychiatric Sle ◽

Inflammatory Phenotype ◽

The Mean

Introduction We aimed to investigate risk factors for fatigue in patients with systemic lupus erythematosus (SLE) and neuropsychiatric symptoms in order to identify potential interventional strategies. Methods Patients visiting the neuropsychiatric SLE (NPSLE) clinic of the Leiden University Medical Center between 2007–2019 were included. In a multidisciplinary consensus meeting, SLE patients were classified as having neuropsychiatric symptoms of inflammatory origin (inflammatory phenotype) or other origin (non-inflammatory phenotype). Fatigue was assessed with the SF-36 vitality domain (VT) since 2007 and the multidimensional fatigue inventory (MFI) and visual analogue scale (VAS) since 2011. Patients with a score on the SF-36 VT ≥1 standard deviation (SD) away from the mean of age-related controls of the general population were classified as fatigued; patients ≥2 SD away were classified as extremely fatigued. Disease activity was measured using the SLE disease activity index-2000. The influence of the presence of an inflammatory phenotype, disease activity and symptoms of depression and anxiety as measured by the hospital anxiety and depression scale (HADS) was analyzed using multiple regression analyses corrected for age, sex and education. Results 348 out of 371 eligible patients filled in questionnaires and were included in this study . The majority was female (87%) and the mean age was 43 ± 14 years. 72 patients (21%) had neuropsychiatric symptoms of an inflammatory origin. Fatigue was present in 78% of all patients and extreme fatigue was present in 50% of patients with an inflammatory phenotype vs 46% in the non-inflammatory phenotype. Fatigue was similar in patients with an inflammatory phenotype compared to patients with a non-inflammatory phenotype on the SF-36 VT (β: 0.8 (95% CI −4.8; 6.1) and there was less fatigue in patients with an inflammatory phenotype on the MFI and VAS (β: −3.7 (95% CI: −6.9; −0.5) and β: −1.0 (95% CI −1.6; −0.3)). There was no association between disease activity and fatigue, but symptoms of anxiety and depression (HADS) associated strongly with all fatigue measurements. Conclusion This study suggests that intervention strategies to target fatigue in (NP)SLE patients may need to focus on symptoms of anxiety and depression rather than immunosuppressive treatment.

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Genetic Neonatal-Onset Epilepsies and Developmental/Epileptic Encephalopathies with Movement Disorders: A Systematic Review

International Journal of Molecular Sciences ◽

10.3390/ijms22084202 ◽

2021 ◽

Vol 22 (8) ◽

pp. 4202

Author(s):

Carlotta Spagnoli ◽

Carlo Fusco ◽

Antonio Percesepe ◽

Vincenzo Leuzzi ◽

Francesco Pisani

Keyword(s):

Movement Disorders ◽

Time Course ◽

Neonatal Period ◽

Age Of Onset ◽

Brain Mri ◽

Neonatal Seizure ◽

Mri Findings ◽

Epileptic Encephalopathies ◽

Pathogenic Variants ◽

Neonatal Onset

Despite expanding next generation sequencing technologies and increasing clinical interest into complex neurologic phenotypes associating epilepsies and developmental/epileptic encephalopathies (DE/EE) with movement disorders (MD), these monogenic conditions have been less extensively investigated in the neonatal period compared to infancy. We reviewed the medical literature in the study period 2000–2020 to report on monogenic conditions characterized by neonatal onset epilepsy and/or DE/EE and development of an MD, and described their electroclinical, genetic and neuroimaging spectra. In accordance with a PRISMA statement, we created a data collection sheet and a protocol specifying inclusion and exclusion criteria. A total of 28 different genes (from 49 papers) leading to neonatal-onset DE/EE with multiple seizure types, mainly featuring tonic and myoclonic, but also focal motor seizures and a hyperkinetic MD in 89% of conditions, with neonatal onset in 22%, were identified. Neonatal seizure semiology, or MD age of onset, were not always available. The rate of hypokinetic MD was low, and was described from the neonatal period only, with WW domain containing oxidoreductase (WWOX) pathogenic variants. The outcome is characterized by high rates of associated neurodevelopmental disorders and microcephaly. Brain MRI findings are either normal or nonspecific in most conditions, but serial imaging can be necessary in order to detect progressive abnormalities. We found high genetic heterogeneity and low numbers of described patients. Neurological phenotypes are complex, reflecting the involvement of genes necessary for early brain development. Future studies should focus on accurate neonatal epileptic phenotyping, and detailed description of semiology and time-course, of the associated MD, especially for the rarest conditions.

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