scholarly journals Susac syndrome with cognitive impairment - case report of 31-year-old woman

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Michał Terpiłowski ◽  
Barbara Terpiłowska ◽  
Anna Orzeł ◽  
Dominika Szlichta ◽  
Marcin Łata ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cognitive Impairment ◽  
Case Report ◽  
Clinical Symptoms ◽  
Psychiatric Symptoms ◽  
Neuropsychiatric Symptoms ◽  
Disease Onset ◽  
Diagnostic Process ◽  
Susac Syndrome ◽  
The Brain

Abstract Introduction: Susac syndrome (SuS) is a rare immune-mediated disease caused by occlusions of microvessels in the brain, retina and inner ear. It is characterized by the clinical triad of encephalopathy, visual disturbances and hearing loss. The diagnosis of SuS is based mainly on the clinical symptoms and the supporting tests in which characteristic changes occur in the MRI. Case report: Here, we present a case of a patient with possible SuS with psychiatric symptoms. A 31-year-old woman was admitted to the Department of Psychiatry due to deteriorating mental health for several weeks manifested as a negative mood and suicidal thoughts. During hospitalization, a neurological consultation was conducted, in which the patient was identified as conscious, psychomotor sluggish, with impaired verbal contact and persistent hearing and vision impairment. Cross-sectional assessment of cognitive functions revealed that the patient had a generalized syndrome of neuropsychological deficits, which confirms the diagnosis of dementia. Discussion: This case summary provides an example of a woman diagnosed with SuS manifested as a cognitive impairment with associated vision and hearing deterioration. It is worth emphasizing the fact that such presentation of the triad of at disease onset is rare. The characteristics of the organic changes in the brain described in the MRI probably explain the symptoms described in that case. The prognosis of SuS depends on early diagnosis and treatment. Conclusions: A diagnosis of SuS should always be considered in the presence of nonspecific neuropsychiatric symptoms and progressive multifocal neurological symptoms, hearing loss, and visual impairment. An important fact is that the typical triad of SuS symptoms in most cases does not occur simultaneously, which makes the diagnostic process very difficult and may lead to misdiagnosis.

scholarly journals Susac syndrome. Case report.

2021 ◽  
Author(s):  
Joseph Bruno Bidin Brooks ◽  
Fábio César Prosdócimi ◽  
Gabriela Yumie Mota Kazama ◽  
Thayná Emily Barboza de Carvalho
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Sensorineural Hearing Loss ◽  
Symptomatic Treatment ◽  
Sensorineural Hearing ◽  
Compulsive Disorder ◽  
Susac Syndrome ◽  
Central Nervous System Dysfunction ◽  
Clinical Triad ◽  
The Brain

Context: Susac Syndrome, was first described in 1979, by neurologist John Susac, and is characterized by a clinical triad consisting of: central nervous system dysfunction, visual changes and sensorineural hearing loss. Although the pathophysiology is not yet fully understood, the description occurs with microangiopathy involving arteries of the brain, retina and cochlea. The manifestation of the clinical triad has a monophasic and self-limiting course.This case report was approved by the Ethics Committee of Universidade Metropolitana de Santos. Case Report: The present case refers to a female patient, 40 years old, caucasian, and presented a sudden decrease in the visual acuity of the left eye, with visual blurring and dyschromatopsia. After 3 days, he sought medical assistance from an ophthalmologist, already presenting amaurosis of his left eye. Occlusion of distal branches of the retinal artery of the left eye was confirmed. He had a personal history of recurrent major depression and obsessive-compulsive disorder with sub-optimal response to drug treatment. Evaluated with neurology, referring to a decrease in asymmetric auditory acuity. Bilateral sensorineural hearing loss confirmed. Skull MRI was performed and showed multiple images of central lesions in the corpus callosum (“snow ball lesions”), as well as lesions in the white matter of the brain. Conclusions: The diagnosis of Susac syndrome was established and symptomatic treatment was performed, presenting with clinical stabilization.

Voltage-gated potassium channel limbic encephalitis presenting as functional cognitive impairment

2020 ◽  
Vol 13 (12) ◽  
pp. e233179
Author(s):  
Eric Garrels ◽  
Fawziya Huq ◽  
Gavin McKay
Keyword(s):  
Cognitive Impairment ◽  
Case Report ◽  
Potassium Channel ◽  
Limbic Encephalitis ◽  
Psychiatric Symptoms ◽  
Differential Diagnoses ◽  
Visual Hallucinations ◽  
Voltage Gated ◽  
History Of ◽  
Impaired Cognition

Limbic encephalitis is often reported to present as seizures and impaired cognition with little focus on psychiatric presentations. In this case report, we present a 49-year-old man who initially presented to the Psychiatric Liaison Service with a several month history of confusion with the additional emergence of visual hallucinations and delusions. Due to the inconsistent nature of the symptoms in the context of a major financial stressor, a provisional functional cognitive impairment diagnosis was made. Investigations later revealed a positive titre of voltage-gated potassium channel (VGKC) antibodies, subtype leucine-rich glioma inactivated 1 accounting for his symptoms which dramatically resolved with steroids and immunoglobulins. This case highlighted the need for maintaining broad differential diagnoses in a patient presenting with unusual psychiatric symptoms.

scholarly journals A case report of unilateral conductive hearing loss as a consequence of malignant sinonasal melanoma: the importance of completing a full clinical history and examination

2020 ◽  
Vol 36 (1) ◽  
Author(s):  
Victoria Blackabey ◽  
Olivia Kenyon ◽  
Rishi Talwar
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Clinical Symptoms ◽  
Symptomatic Relief ◽  
Clinical History ◽  
Histological Diagnosis ◽  
Unusual Presentation ◽  
Sinus Surgery ◽  
Cancer Management ◽  
The Right

Abstract Background Sinonasal melanoma is a rare head and neck tumour. It is associated with a poor prognosis, high rates of loco-regional recurrence and distant metastasis. Treatment of the disease is therefore complicated, and because of limited data regarding the cancer, management is frequently tailored to the individual patient. We describe an unusual presentation of sinonasal melanoma with relevant histology, radiology and clinical photography. Case presentation The case report describes the presentation of a 64-year-old man to the Ear, Nose and Throat department with progressive right-sided hearing loss. A thorough history highlighted other clinical symptoms including unilateral nasal obstruction and epistaxis. Clinical examination showed a right middle ear effusion with a polypoidal lesion in the right nasal cavity. Relevant imaging demonstrated a destructive process that required further assessment. An endoscopic sinus procedure was performed to obtain histological diagnosis as well as providing symptomatic relief. Histology confirmed malignant mucosal melanoma. The patient underwent maxillectomy and orbital exenteration (due to further progression of disease) at a tertiary centre with a plan for subsequent immunotherapy. This however has been delayed due to further surgery to excise a metastatic lesion to the right femur. Conclusions This case report highlights the importance of a thorough clinical history and examination. An unusual presentation of a sinonasal tumour can easily be missed leading to a significant delay in treatment. The case report also describes the use of functional endoscopic sinus surgery in order to obtain histological diagnosis and to debulk the tumour, providing symptomatic relief. The current literature regarding management will be discussed as well as current developments guiding future treatment.

scholarly journals Mood symptoms Associated with CADASIL Syndrome: A Case Report

CNS Spectrums ◽  
2021 ◽  
Vol 26 (2) ◽  
pp. 144-144
Author(s):  
Asad Shaikh ◽  
Joel Idowu
Keyword(s):  
Case Report ◽  
Clinical Symptoms ◽  
Psychiatric Symptoms ◽  
Small Vessel Disease ◽  
Altered Mental Status ◽  
Hereditary Disease ◽  
African American Female ◽  
Psychiatric Disturbance ◽  
Rare Type ◽  
Parietal Lobes

AbstractObjectiveTo discuss the psychiatric symptoms that are associated with CADASIL syndrome Abstract Cerebral:Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a rare type of hereditary disease involving the small cerebral vessels. The clinical symptoms are various and include recurrent ischemic strokes, migraine with aura, seizures with epilepsy, psychiatric problems such as mood disturbances, and progressive cognitive decline leading to dementia. This disease needs awareness amongst the psychiatrists even though it is discussed much more in neurology literature. Psychiatric symptoms are seen in 20–41% of patients with CADASIL syndrome (1, 2). Psychiatric symptoms are actually the initial presentation in 15% of the cases. (3) The psychiatric disturbance most reported are mood disturbances (9–41%) especially depression. Here a 42-year-old African American female was brought to the hospital emergency room after she was found wandering in the streets. Psychiatry was consulted for altered mental status. Upon evaluation by the psychiatric consult service she was only oriented to person, depressed, anxious and complaining of headaches. Initial CT scan showed marked small vessel disease and old lacunar infarcts in the basal ganglia and right corona radiata. Magnetic Resonance Imaging (MRI) of the brain showed acute infarcts in the right posterior frontal and right parietal lobes along with old infarcts. Her symptoms and findings on imaging were consistent with CADASIL syndrome. Once the diagnosis was confirmed and prior records were obtained patient was resumed on an antidepressant and anxiolytic.ConclusionThe purpose of this case report was to discuss psychiatric symptoms associated with CADASIL syndrome. Although there has been research showing a relationship between vascular disease and depression, a review of the literature suggests that there needs to be more research done to explore other psychiatric disturbances that may be seen with this syndrome. Psychiatric symptoms that are untreated can have the potential to further impact the quality of life therefore psychiatrists need to be aware of this syndrome in order to treat these patients promptly.References1 https://bmcmedicine.biomedcentral.com/articles/10.1186/s12916-017-0778-8 2 http://dx.doi.org/10.32474/OJNBD.2018.01.000101 3 https://pdfs.semanticscholar.org/47f6/5952ee3c5dcf2a61345f704914b17fa8dc0d.pdf

scholarly journals Neuropsychological profile of a male psychiatric patient with a Morgagni-Stewart-Morel syndrome

2014 ◽  
Vol 27 (1) ◽  
pp. 60-64 ◽  
Author(s):  
Aksel Hansen ◽  
Liliana Engelhardt ◽  
Wolfgang Pleschutznig ◽  
Gerhard Dammann ◽  
Stephanie Vietze
Keyword(s):  
Case Report ◽  
Psychiatric Patient ◽  
Neuropsychological Tests ◽  
Psychiatric Symptoms ◽  
Case Reports ◽  
Neuropsychiatric Symptoms ◽  
Pain Syndrome ◽  
Admission Diagnosis ◽  
Neuropsychological Profile ◽  
Definition Of

In 1765 Giovanni Morgagni described a syndrome consisting of hyperostosis frontalis interna (HFI), obesity and hirsutism. In 1928 Stewart and in 1930 Morel added neuropsychiatric symptoms, e.g. depression and dementia, which led to the definition of the Morgagni-Stewart-Morel Syndrome (MSM). Although mostly women were characterized in literature no gender specifity is demanded. This case report presents the rare case of a 66 year old male psychiatric patient with Morgagni-Stewart-Morel Syndrome. The patient complained of loss of concentration and difficulties with activities of daily living. Admission diagnosis was an opioid misuse on the basis of a chronic pain syndrome. In this case report we are describing clinical features, the patient history and technical (MRI) and neuropsychological tests. Although severe psychiatric symptoms and neuropsychological deficits are commonly seen in these patients, our patient showed only mild symptoms. This case reports shows the possibility of a male patient with MSM. If MSM is a separate entity or just an epiphenomena of hormone dysregulation should be investigated in further studies.

scholarly journals Ekbom Syndrome associated with Lewy Body Dementia: A case report

2020 ◽  
Vol 14 (1) ◽  
pp. 83-87
Author(s):  
Felippe José Pontes de Mendonça ◽  
Ivan Abdalla Teixeira ◽  
Valeska Marinho
Keyword(s):  
Case Report ◽  
Lewy Body ◽  
Psychiatric Symptoms ◽  
Neuropsychiatric Symptoms ◽  
Correct Diagnosis ◽  
Lewy Body Dementia ◽  
Rare Condition ◽  
Adequate Treatment ◽  
Thought Disorders ◽  
Ekbom Syndrome

ABSTRACT Ekbom Syndrome, also known as parasitosis delusion or psychogenic parasitosis, is a rare condition in which patients present with a fixed belief of being infested by parasites, vermin or small insects, along with tactile hallucinations (such as pruritus or sensations of the parasites crawling over or under the skin). The syndrome may occur idiopathically or be associated with other medical conditions and drug use. This case report describes the occurrence of Ekbom syndrome in a patient diagnosed with Lewy Body Dementia (LBD), a neurodegenerative disease that commonly presents with sensory perception and thought disorders and other neuropsychiatric symptoms. Although visual hallucination is considered a core diagnostic criterion, other modalities of psychiatric symptoms can also occur posing a further challenge for correct diagnosis. Proper recognition allows early diagnosis and adequate treatment, preventing hazardous antipsychotic use in these patients.

scholarly journals Rare Occurrence of Internal Auditory Canal Stenosis Accompanied With Congenital Facial Palsy in a 3-Month-Old Infant: A Case Report

2020 ◽  
Vol 44 (3) ◽  
pp. 256-259
Author(s):  
Se-Heum Park ◽  
Woo-Jin Kim ◽  
Yun-Jung Lim ◽  
Cheol-Won On ◽  
Ji-Ho Park ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Developmental Delay ◽  
Facial Palsy ◽  
Internal Auditory Canal ◽  
Magnetic Resonance Imaging Scan ◽  
Developmental Assessment ◽  
Canal Stenosis ◽  
The Brain ◽  
Tomography Scan

Internal auditory canal (IAC) stenosis with hypoplasia of the facial and vestibulocochlear nerves is a rare cause of congenital facial palsy. In this case report, a 3-month-old female infant was referred for a neurological developmental assessment for developmental delay and congenital facial palsy. Upon evaluation of developmental delay, hearing loss was detected. Following a magnetic resonance imaging scan of the brain and a computed tomography scan of the temporal bone, IAC stenosis with hypoplasia of facial and vestibular nerves was diagnosed. This is a rare case of IAC stenosis in an infant with initial presentations of left facial palsy and developmental delay associated with hearing loss in the left ear. We strongly suggest that IAC stenosis be considered a cause of congenital facial palsy in infants, especially in patients with developmental delay. In infants with congenital facial palsy, a thorough physical examination and neurological developmental assessment should be performed.

scholarly journals Convexity meningioma associated with depression: A case report

2014 ◽  
Vol 142 (9-10) ◽  
pp. 586-588 ◽  
Author(s):  
Nenad Zivkovic ◽  
Marko Markovic ◽  
Milan Spaic
Keyword(s):  
Case Report ◽  
Major Depression ◽  
Psychiatric Symptoms ◽  
Late Onset ◽  
Depression Symptoms ◽  
Total Resection ◽  
Long Time ◽  
Fibroblastic Meningioma ◽  
Slow Growing ◽  
The Brain

Introduction. Meningiomas are slow growing, extra-axial lesions, and can be neurologically silent for a long time and present only with depression. Case Outline. A 65-year-old woman developed major depression and was treated with antidepressants for two years. Depression failed to respond to drug treatment and there was no improvement. Two months before admission to hospital, due to the onset of epilepsy attack the patient underwent reinvestigation, and a large temporal convexity meningioma, which corresponded in position to the original electroencephalography focus, was diagnosed using the computer topography of the brain. The patient underwent osteoplastic craniotomy, and a left fronto-temporal convexity meningioma of 5 cm in diameter was completely removed with its attachment to the dura. Histological examination confirmed a fibroblastic meningioma. Conclusion. Total resection of convexity meningioma and decompression of the brain tissue in the region of limbic pathways that are involved, may contribute to a complete remission of depression symptoms. This case also illustrates the need for a prompt neuroimaging of the brain when patients present any atypical psychiatric symptoms, with late onset (>50 years old) of the first depressive episode or fast changes of the mental state.

scholarly journals CADASIL: case report

2012 ◽  
Vol 6 (3) ◽  
pp. 188-191
Author(s):  
Julio Cesar Vasconcelos da Silva ◽  
Emerson L. Gasparetto ◽  
Eliasz Engelhardt
Keyword(s):  
Cognitive Impairment ◽  
Case Report ◽  
Genetic Testing ◽  
Autosomal Dominant ◽  
Transient Ischemic Attacks ◽  
Imaging Studies ◽  
Notch 3 ◽  
Sensory Motor ◽  
Cerebral Arteriopathy ◽  
The Brain

ABSTRACT Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a hereditary cerebral arteriopathy caused by mutations in the Notch-3 gene. The diagnosis is reached by skin biopsy revealing presence of granular osmiophílic material (GOM), and/or by genetic testing for Notch-3. We report a case of a 52-year-old man with recurrent transient ischemic attacks (TIA), migraine, in addition to progressive sensory, motor and cognitive impairment. He was submitted to a neuropsychological assessment with the CERAD (Consortium to Establish a Registry for Alzheimer's Disease) battery along with other tests, as well as neuroimaging and genetic analysis for Notch-3, confirming the diagnosis. Executive function, memory, language and important apraxic changes were found. Imaging studies suggested greater involvement in the frontal lobes and deep areas of the brain.

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