Clinical characteristics and neuroimaging findings of seven patients with Dyke Davidoff Masson syndrome

Abstract Background DDMS is a rare disease diagnosed by clinical and radiological characteristics. But the complexity of radiological and clinical manifestations of DDMS has become a challenge diagnostically. To date, the reported cases with DDMS had highly varied clinical manifestations including seizures, contralateral hemiplegia/hemiparesis, facial asymmetry, mental retardation, etc. In addition to typical clinical findings, some new characteristics have been recently added to the spectrum of DDMS. However, few cases have been reported to be associated with neuropsychiatric symptoms according to the literature. This study aimed to investigate the neuropsychiatric manifestations associated with Dyke-Davidoff-Masson syndrome (DDMS) and related imaging findings. Methods This study included 7 patients diagnosed with DDMS between 2014 and 2020. The clinical characteristics, neuropsychiatric manifestations, and radiological results were retrospectively evaluated. Results Seven patients (five males and two females) with a mean age of 28.0 ± 9.73 (range 15.0–41.0) years were included. Five patients were admitted to the psychiatric unit due to psychological and behavioral disorders. Two patients were referred to the neurology unit mainly due to epilepsy. Six patients had epileptic seizures, 4 had hemiplegia, 3 had mental retardation, 2 patients had external ear deformities, and 2 had facial asymmetry. Neuropsychiatric symptoms were presented in 6 (85.7 %) cases. Cases 2–6 developed affective disorders. Deficits in verbal communication, impairment of social interaction, lack of insight, adulia and hypobulia appeared in cases 1–4. Schizophrenia with apathy, and epileptic schizoid psychosis were observed in cases 4 and 5 respectively. Case 6 had behavioral disorders, hyperactivity, tic disorder, mental retardation, anxiety, catatonic symptoms and suicidal tendency. Case 7 had seizures and mental retardation, and no psychiatric symptoms were presented. Radiological examinations showed unilateral cerebral atrophy, enlarged lateral ventricles, and various compensatory hypertrophy of the skull in all cases. The midline structure has shifted to the affected side in 5(71.4 %) cases. Atrophy of the basal ganglia or brain stem was observed in 4(57.1 %) cases. Conclusions The hallmark imaging manifestations of DDMS facilitated the diagnosis in most cases. This study illustrated that a variety of psychoneurotic disorders and ear abnormalities were correlated with DDMS.

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“organic Personality Disorder” as Part of Early Neuropsychiatric Manifestations in Huntington’s Disease - a Case Report

European Psychiatry ◽

10.1016/s0924-9338(09)71313-6 ◽

2009 ◽

Vol 24 (S1) ◽

pp. 1-1

Author(s):

J. Maia

Keyword(s):

Huntington's Disease ◽

Huntington’S Disease ◽

Psychiatric Symptoms ◽

Age Of Onset ◽

Neuropsychiatric Symptoms ◽

Personality Change ◽

Psychotic Symptoms ◽

Autosomal Dominant Disorder ◽

Neuropsychiatric Manifestations ◽

Psychiatric Manifestations

Huntington's Disease (HD) is an inherited autosomal dominant disorder characterized by motor, cognitive and psychiatric symptomatology, being considered a paradigmatic neuropsychiatric disorder that includes all three components of the "Triadic Syndromes": dyskinesia, dementia and depression.Firstly described in 1872 as an "Hereditary Chorea" by George Huntington only in 1993 was its responsible gene identified. A person who inherits the HD gene will sooner or later develop the disease. the age of onset, early signs and rate of disease progression vary greatly from person to person.Neuropsychiatric symptoms are an integral part of HD and have been considered the earliest markers of the disease, presenting sometimes more than 10 years before a formal diagnosis is done. Patients may experience dysphoria, mood swings, agitation, irritability, hostile outbursts, psychotic symptoms and deep bouts of depression with suicidal ideation. Personality change is reported in 48% of the cases, with the paranoid subtype being described as the most prevalent. the clinical case presented illustrates a case of HD which started with insidious psychiatric symptoms and an important personality change.Despite a wide number of medications being prescribed to help control emotional, movement and behaviour problems, there is still no treatment to stop or reverse the course of the disease. Furthermore, psychiatric manifestations are often amenable to treatment, and relief of these symptoms may provide significant improvement in patient's and caregivers quality of life.A greater awarness of psychiatric manifestations of HD is essential to an earlier diagnosis and an optimized therapeutic approach.

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Neuropsychiatric manifestations of COVID-19 can be clustered in three distinct symptom categories

Scientific Reports ◽

10.1038/s41598-020-78050-6 ◽

2020 ◽

Vol 10 (1) ◽

Author(s):

Fatemeh Sadat Mirfazeli ◽

Atiye Sarabi-Jamab ◽

Amin Jahanbakhshi ◽

Alireza Kordi ◽

Parisa Javadnia ◽

...

Keyword(s):

Clinical Symptoms ◽

Neuropsychiatric Symptoms ◽

Clinical Manifestations ◽

Neuropsychiatric Symptom ◽

Neuropsychiatric Complications ◽

Neuropsychiatric Manifestations ◽

Clustering Approach ◽

Speech Problem ◽

Force Reduction ◽

Underlying Mechanisms

AbstractSeveral studies have reported clinical manifestations of the new coronavirus disease. However, few studies have systematically evaluated the neuropsychiatric complications of COVID-19. We reviewed the medical records of 201 patients with confirmed COVID-19 (52 outpatients and 149 inpatients) that were treated in a large referral center in Tehran, Iran from March 2019 to May 2020. We used clustering approach to categorize clinical symptoms. One hundred and fifty-one patients showed at least one neuropsychiatric symptom. Limb force reductions, headache followed by anosmia, hypogeusia were among the most common neuropsychiatric symptoms in COVID-19 patients. Hierarchical clustering analysis showed that neuropsychiatric symptoms group together in three distinct groups: anosmia and hypogeusia; dizziness, headache, and limb force reduction; photophobia, mental state change, hallucination, vision and speech problem, seizure, stroke, and balance disturbance. Three non-neuropsychiatric cluster of symptoms included diarrhea and nausea; cough and dyspnea; and fever and weakness. Neuropsychiatric presentations are very prevalent and heterogeneous in patients with coronavirus 2 infection and these heterogeneous presentations may be originating from different underlying mechanisms. Anosmia and hypogeusia seem to be distinct from more general constitutional-like and more specific neuropsychiatric symptoms. Skeletal muscular manifestations might be a constitutional or a neuropsychiatric symptom.

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Neuropsychiatric manifestations in CADASIL

Dialogues in Clinical Neuroscience ◽

10.31887/dcns.2007.9.2/hchabriat ◽

2007 ◽

Vol 9 (2) ◽

pp. 199-208 ◽

Cited By ~ 2

Keyword(s):

White Matter ◽

Psychiatric Symptoms ◽

Small Vessel Disease ◽

Cerebral Atrophy ◽

White Matter Lesions ◽

Cerebral Lesions ◽

Motor Disabilities ◽

Neuropsychiatric Manifestations ◽

Artery Disease ◽

Exact Origin

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited small-artery disease of mid-adulthood caused by mutations of the NOTCH3 gene. The disease is responsible for widespread white-matter lesions associated with lacunar infarctions in various subcortical areas. The disease is responsible for migraine with aura and ischemic strokes, and is associated with various degrees of cognitive impairment and with mood disturbances. CADASIL is considered as a unique model to investigate what is known as "subcortical ischemic vascular dementia." Recent data suggest that the number of lacunar infarctions and severity of cerebral atrophy are the main magnetic resonance imaging markers associated with cognitive and motor disabilities in this disorder. Mood disturbances are reported in 10% to 20% of patients, most often in association with cognitive alterations. Their exact origin remains unknown; the presence of ischemic lesions within the basal ganglia or the frontal white matter may promote the occurrence of these symptoms. Further studies are needed to better understand the relationships between cerebral lesions and both cognitive and psychiatric symptoms in this small-vessel disease of the brain.

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Frequency and Clinical Aspects of Neurological and Psychiatric Symptoms in Patients with Non-Celiac Wheat Sensitivity

Nutrients ◽

10.3390/nu13061971 ◽

2021 ◽

Vol 13 (6) ◽

pp. 1971

Author(s):

Antonio Carroccio ◽

Maurizio Soresi ◽

Marta Chiavetta ◽

Francesco La Blasca ◽

Stella Compagnoni ◽

...

Keyword(s):

Clinical Presentation ◽

Psychiatric Symptoms ◽

Neuropsychiatric Symptoms ◽

Duodenal Mucosa ◽

Clinical Aspects ◽

Double Blind ◽

Duration Of Symptoms ◽

Neuropsychiatric Manifestations ◽

Wheat Sensitivity ◽

Clinical Records

Background: Non-Celiac Wheat Sensitivity (NCWS) is characterized by both intestinal and extra-intestinal symptoms. The study aims to investigate the frequency of neuropsychiatric manifestations in NCWS patients and identify their clinical and demographic characteristics. Methods: 278 clinical records of NCWS patients, diagnosed by a double-blind placebo-controlled wheat challenge between 2006 and 2020, were retrospectively revised. Fifty-two patients with Celiac Disease (CD) and 54 patients with Irritable Bowel Syndrome (IBS) served as controls. Results: 87% of the NCWS patients had an IBS-like clinical presentation. The NCWS group showed a longer duration of symptoms, a higher frequency of positive serum anti-nuclear antibodies than CD and IBS patients, and a higher frequency of DQ2/DQ8 haplotypes and duodenal mucosa lymphocytosis than IBS controls. In addition, 50% of NCWS patients showed neuropsychiatric manifestations, while lower percentages were observed in CD (25%) and IBS (28%) controls. Neuropsychiatric symptoms in NCWS were more frequently associated with the male sex, longer duration of symptoms, and IBS-diarrhea-like clinical presentation. Conclusions: Our data suggest that in patients with IBS-like symptoms and neuropsychiatric manifestations of unknown cause, it could be useful to investigate a correlation of these symptoms with wheat ingestion to identify NCWS patients with this ‘atypical’ manifestation.

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Stage-Specific Gender Differences in Cognitive and Neuropsychiatric Manifestations of Vascular Dementia

American Journal of Alzheimer s Disease & Other Dementias® ◽

10.1177/1533317512454712 ◽

2012 ◽

Vol 27 (6) ◽

pp. 433-438 ◽

Cited By ~ 12

Author(s):

Yi Xing ◽

Cuibai Wei ◽

Changbiao Chu ◽

Aihong Zhou ◽

Fang Li ◽

...

Keyword(s):

Gender Differences ◽

Vascular Dementia ◽

Neuropsychiatric Symptoms ◽

Clinical Manifestations ◽

Male Predominance ◽

Gender Comparisons ◽

Verbal Recall ◽

Severe Stage ◽

Neuropsychiatric Manifestations ◽

Male Patients

Studies on gender differences in the clinical manifestations of vascular dementia (VaD) are still lacking. In the present study, gender comparisons of cognitive and neuropsychiatric profiles were conducted separately for mild and moderate-to-severe VaD in a total of 467 patients with VaD. There were no significant gender differences in cognitive manifestations, except that females performed better on immediate verbal recall than males in mild stage. Women were more likely to exhibit delusions (15.5% vs 7.4%), hallucinations (9.5% vs 3.4%), and depression (43.1% vs 27.3%) in mild stage. The predominance of male patients was observed in apathy at moderate-to-severe stage (50.5% vs 34.8%). To conclude, gender differences existed in neuropsychiatric symptoms of VaD and were especially pronounced in mild stage. Delusions, hallucinations, and depression were more prevalent in females in mild VaD, with the male predominance only in apathy in the later stage.

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Study of Prevalence of Neuropsychiatric Symptoms in Elderly Dementia Patients

JOURNAL FOR CLINICAL AND DIAGNOSTIC RESEARCH ◽

10.7860/jcdr/2021/44440.14510 ◽

2021 ◽

Author(s):

Mohan Mahale ◽

Pradeep Behal ◽

Nitul M Bewal ◽

Vivek Aggarwal ◽

Anuj Singhal ◽

...

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Psychiatric Symptoms ◽

Neuropsychiatric Symptoms ◽

Tertiary Care ◽

Primary Caregiver ◽

Ageing Population ◽

Night Time ◽

Neuropsychiatric Manifestations ◽

B12 Deficiency

Introduction: Prevalence of dementia is increasing with rapidly ageing population. Neuropsychiatric Symptoms (NPS) are common in certain types of dementia and increases with duration of dementia in Alzheimer’s disease and are causes significant psychosocial and management issues. Aim: To study the prevalence and type of NPS in patients of dementia presenting to a Tertiary Care Hospital and to find out the association between NPS and the type of dementia and to assess the association of the caregiver stress in this setting. Materials and Methods: The present cross-sectional observational study was conducted at Tertiary Care Government Hospital of Western Maharashtra. All patients with suspected dementia were initially screened by a Mini-Cog questionnaire and patients with a score of three or less were assessed with Hindi Mental Status Examination (HMSE). The inclusion criteria were patients with HMSE of less than or equal to 23. All the diagnosed patients with dementia were screened for common psychiatric symptoms using Neuropsychiatric Inventory (NPI). The presence of NPS was noted based on either presenting complaint of the patient or as per the history given by the primary caregiver for these symptoms in last one month. These questions were administered in focused group discussion form to the primary caregiver by the physician. The data was collected and analysed using descriptive statistics and Chi-square test with SSPS 20. Results: The most common neuropsychiatric manifestation was sleep and night-time behavioural disorder (51.5%). It was followed by depression (44%). Neuropsychiatric manifestations were much more common in demented patients of probable Dementia with Lewy Bodies (DLB) and dementia with B12 deficiency. More than 50% of the patients had two or more psychiatric symptoms. The most common psychiatric symptom in patients with probable Alzheimer’s disease was sleep and night-time disturbance in 54.9%. Common neuropsychiatric manifestations seen in DLB were visual hallucinations (100%), followed by irritability (88%). More than 90% of the caregivers were not aware of the psychiatric manifestations of dementia and did not know how to cope up with these symptoms. Conclusion: NPS were more common in patients with DLB dementia and dementia with B12 deficiency, with depression been more common symptom in patients with Parkinson’s related dementia. It is therefore, very important to look for NPS in all elderly patients which can help us to diagnose dementia earlier.

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Dyke-Davidoff-Masson Syndrome with crossed cerebellar atrophy

Nepal Journal of Neuroscience ◽

10.3126/njn.v18i3.37163 ◽

2021 ◽

Vol 18 (3) ◽

pp. 73-75

Author(s):

Rohit Kumar ◽

Deepak Kumar ◽

Himanshu Mishra ◽

Sanjay Kumar Suman ◽

Umakant Prasad

Keyword(s):

Early Childhood ◽

Mental Retardation ◽

Frontal Sinus ◽

Cerebellar Atrophy ◽

Cerebral Atrophy ◽

Facial Asymmetry ◽

Mri Findings ◽

Neurological Condition ◽

Mri Brain ◽

Cerebral Insult

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare neurological condition characterised clinically by recurrent seizures, facial asymmetry, hemiplegia and mental retardation likely due to foetal or early childhood cerebral insult. We describe the MRI findings of DDMS in a 10-year-old male child. MRI brain revealed right cerebral atrophy, ipsilateral thickening of calvarium, right lateral ventricular dilatation, hyper-pneumatisation of frontal sinus, and contralateral cerebellar atrophy which are consistent with DDMS.

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Neuropsychiatric Lupus in an Adolescent Presenting with Bipolar Disorder with Catatonic Features

European Psychiatry ◽

10.1016/s0924-9338(09)70803-x ◽

2009 ◽

Vol 24 (S1) ◽

pp. 1-1

Author(s):

A. Alao ◽

C. Chung

Keyword(s):

Family History ◽

Pediatric Patients ◽

Psychiatric Symptoms ◽

Neuropsychiatric Symptoms ◽

Psychiatric Disease ◽

African American Female ◽

Neuropsychiatric Lupus ◽

Psychiatric Medication ◽

Bipolar I Disorder ◽

Neuropsychiatric Manifestations

Case presentation:We describe a 15-year-old African American female with a family history positive for Bipolar I disorder and schizophrenia who presented with symptoms consistent with an affective disorder. The patient was diagnosed with Bipolar I disorder with catatonic features and required multiple hospitalizations for mood disturbance. Two years after her initial presentation, the patient was noted to have a malar rash and subsequently underwent a full rheumatologic workup, which revealed cerebral vasculitis. Neuropsychiatric lupus (NPSLE) was diagnosed and, following treatment with steroids, the patient improved substantially and no longer required further psychiatric medication or therapy.Conclusion:Neuropsychiatric manifestations of lupus (NPSLE) have been shown to occur in 80-91% of adults and up to 95% of pediatric patients with SLE. In the case we described, diagnosis was hindered by the patient's presentation of what appeared to be isolated psychiatric symptoms and her strong family history of psychiatric disease. Thus, given the especially high prevalence of NPSLE in pediatric patients with lupus, it is important for clinicians to recognize that neuropsychiatric symptoms in an adolescent may indeed be the initial manifestations of SLE.

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Clinical and Dopamine Depletion Patterns in Hyposmia- and Dysautonomia-Dominant Parkinson’s Disease

Journal of Parkinson s Disease ◽

10.3233/jpd-212747 ◽

2021 ◽

pp. 1-11

Author(s):

Han Soo Yoo ◽

Young-gun Lee ◽

Seong Ho Jeong ◽

Byoung Seok Ye ◽

Young H. Sohn ◽

...

Keyword(s):

Parkinson’S Disease ◽

Parkinson's Disease ◽

Clinical Characteristics ◽

Ventral Striatum ◽

Neuropsychiatric Symptoms ◽

Age At Onset ◽

Clinical Manifestations ◽

Rem Sleep Behavior Disorder ◽

Dopamine Depletion ◽

Prodromal Symptoms

Background: Olfactory or autonomic dysfunction is one of the earliest prodromal symptoms of Parkinson’s disease (PD). It has not been investigated whether PD patients have different phenotypes depending on the presence of these prodromal symptoms. Objective: To investigate whether hyposmia-dominant and dysautonomia-dominant patients with early PD have different clinical manifestations and nigrostriatal degeneration. Methods: This cross-sectional study recruited 168 drug-naive PD patients and 34 control subjects. PD patients were classified as patients without hyposmia and dysautonomia (PD–H–D–, n = 51), hyposmia-dominant patients (PD–H+D–, n = 36), dysautonomia-dominant patients (PD–H–D+, n = 33), and patients with hyposmia and dysautonomia (PD–H+D+, n = 48). We then compared the baseline clinical characteristics, striatal specific to non-specific binding ratio (SNBR), neuropsychological performance, and neuropsychiatric symptoms among the groups. Results: The PD–H+D–group had a lower SNBR in the ventral striatum (p = 0.013), a greater asymmetric index of striatal SNBRs, and higher prevalence of apathy (p = 0.021) than the PD–H–D+ group. The PD–H–D+ group had older age at onset (p = 0.043) and a higher prevalence of REM sleep behavior disorder (p = 0.041) than the PD–H+D–group. The PD–H+D+ group had higher motor deficits, lower cognitive function, and lower SNBRs in all striatal subregions than the PD–H–D–group. Decreased SNBRs in the anterior caudate, posterior caudate, and ventral striatum were associated with the presence of apathy. Conclusion: The present study suggests that hyposmia-dominant and dysautonomia-dominant PD have different clinical characteristics and patterns of striatal dopamine depletion.

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Misidentification of Acute Psychiatric Symptoms in the Emergency Room: Clinical Experience in China

Frontiers in Psychiatry ◽

10.3389/fpsyt.2020.579484 ◽

2021 ◽

Vol 11 ◽

Author(s):

Fang Liu ◽

Jianjun Chen ◽

Yang Du ◽

Wenxia Jiang ◽

Lei Gong ◽

...

Keyword(s):

Emergency Department ◽

Emergency Room ◽

Clinical Characteristics ◽

Sleep Disturbances ◽

Clinical Symptoms ◽

Psychiatric Symptoms ◽

Neurological Diseases ◽

Clinical Manifestations ◽

Neuropsychiatric Inventory ◽

Abnormal Behaviors

Introduction: Patients who come to the emergency department because of acute psychiatric symptoms are often not admitted to the correct department timely. The purpose of this study is to identify the clinical characteristics of patients with acute psychiatric symptoms in order to achieve early and correct triage in the emergency room.Methodology: We conducted a cross-analysis of inpatients who first came to the emergency department with acute psychiatric symptoms and then admitted to the department of neurology or psychiatry between years 2012 and 2018. Among them, 70 patients were rediagnosed and retransferred, with 38 patients to the department of neurology and 32 patients to the department of psychiatry. The clinical characteristics, laboratory examination, and Neuropsychiatric Inventory (NPI) were analyzed.Results: Patients who were rediagnosed with neurological diseases were more prone to have somatic symptoms (headache, dizziness) (P < 0.05). Because of the atypical early clinical manifestations in the emergency room, fever and positive neurological signs do not necessarily represent the diagnosis of neurological diseases. On the other hand, the absence of these manifestations does not guarantee the diagnosis of psychiatric illness. According to NPI, abnormal behaviors, changes in appetite, and sleep disturbances were more obvious in patients with neurological diseases (P < 0.05), whereas patients with psychiatric disorders often showed prominent irritability (P < 0.05).Conclusions: Acute psychiatric symptoms are usually complex and diverse. The triage and diagnosisshould be based on multiple factors. After triage, clinical symptoms should be dynamically observed.

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