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Brain and Nerve = Shinkei Kenkyu No... Mar 2024We report a case of anti-NMDAR encephalitis and residual mutism in a 23-year-old woman who presented with neuroleptic intolerance. Admission to our department for...
We report a case of anti-NMDAR encephalitis and residual mutism in a 23-year-old woman who presented with neuroleptic intolerance. Admission to our department for investigation of her abnormal behavior revealed cerebrospinal fluid (CSF) positivity for anti-NMDAR antibodies, and the patient underwent immunotherapy. However, generalized tonic seizures developed, requiring mechanical ventilation in the intensive care unit. Antipsychotic drugs were also administered for involuntary movements and insomnia. Thereafter, a malignant syndrome of severe hyperCKemia (Max: 191,120 IU/L) and shock developed, requiring resuscitation and three sessions of hemodialysis. Subsequent rituximab therapy led to improvement, except for mutism, which had newly developed during resuscitation. Seven months after initial admission, the patient was discharged with independent gait. However, her mutism still persists. Temporary mutism has been reported to occur in this type of encephalitis, albeit rarely. The fact that remission was not observed in this case may have been due to cerebellar infarction occurring during resuscitation, but the true cause remains unclear. Malignant syndrome or rhabdomyolysis, as seen in this patient, has also sometimes been reported in this form of encephalitis when antipsychotic agents, especially dopamine receptor blockers, have been administered. Therefore, such agents should be administered with caution in patients with anti-NMDAR encephalitis. (Received August 17, 2023; Accepted October 24, 2023; Published March 1, 2024).
Topics: Humans; Female; Young Adult; Adult; Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Antipsychotic Agents; Mutism; Seizures; Receptors, N-Methyl-D-Aspartate; Receptors, Amino Acid
PubMed: 38514109
DOI: 10.11477/mf.1416202598 -
Brain & Spine 2024Autism spectrum disorder (ASD) is characterized by deficits in social communication, repetitive behaviors, and can be accompanied by a spectrum of psychiatric symptoms,...
INTRODUCTION
Autism spectrum disorder (ASD) is characterized by deficits in social communication, repetitive behaviors, and can be accompanied by a spectrum of psychiatric symptoms, such as schizophrenia and catatonia. Rarely, these symptoms, if left untreated, can result in spinal deformities.
RESEARCH QUESTION AND CASE DESCRIPTION
This case report details the treatment of a 16-year-old male ASD patient with catatonic schizophrenia and mutism, presenting with neck pain, left-rotated torticollis, and fever. MRI revealed atlantoaxial rotational instability and spinal cord compression from a dislocated dens axis. After inconclusive biopsies, empirical antibiotics, hard collar and halo fixation treatment, persistent instability necessitated C1/2 fusion. The ongoing catatonia was addressed with electroconvulsive therapy. Concurrently, he developed severe subaxial hyperkyphosis. The report examines the decision-making between conservative and surgical management for an adolescent with significant psychiatric comorbidity and progressive spinal symptoms against a backdrop of uncertain etiology.
MATERIALS AND METHODS
A case report and review of the literature.
RESULTS
Posterior C1-C7 stabilization was successfully executed, effectively restoring cervical sagittal alignment, which was maintained throughout a two-year follow-up. Concurrently, the catatonia resolved.
DISCUSSION AND CONCLUSION
To our knowledge, this is the third reported case of severe cervical deformity associated with fixed posture in a psychiatric patient. This case report emphasizes the critical importance of multidisciplinary collaboration in managing the interplay between neuropsychiatric disorders and severe spinal deformities. It showcases the practicality and efficacy of surgical intervention for persistent cervical deformity in pediatric schizophrenia patients, highlighting the necessity for a comprehensive risk-benefit analysis.
PubMed: 38510616
DOI: 10.1016/j.bas.2024.102747 -
Archives de Pediatrie : Organe Officiel... May 2024Cerebellar mutism syndrome (CMS) occurs in 8-29 % of children undergoing posterior fossa tumor surgery. Its main symptoms are mutism and emotional lability. Although it...
BACKGROUND
Cerebellar mutism syndrome (CMS) occurs in 8-29 % of children undergoing posterior fossa tumor surgery. Its main symptoms are mutism and emotional lability. Although it is always transient, recovery time can be lengthy with long-term cognitive sequelae. There is no approved drug treatment for CMS, but some drugs are used in everyday medical practice. One of these is fluoxetine, which has been used for many years in our institution. The main objective of this study was to establish the safety profile of fluoxetine in this condition.
MATERIALS AND METHODS
The records of patients admitted to the pediatric intensive care unit after brain surgery at Angers University Hospital from 2010 to 2020 were reviewed. Children aged 2 years and older who underwent a posterior fossa tumor surgery and were diagnosed with CMS were included. Data on patient characteristics, prescription of fluoxetine treatment, side effects if any, and complete mutism duration were collected.
RESULTS
Among 246 patients admitted to the pediatric intensive care unit for brain surgery during the study period, 23 had CMS and eight were prescribed fluoxetine. No serious adverse event related to fluoxetine was reported. Complete mutism duration did not differ significantly between the fluoxetine group and the non-fluoxetine group(p = 0.22). However, the treatment was initiated after recovery from complete mutism in half of the treated patients.
CONCLUSION
This study suggests a positive safety profile of fluoxetine used in postoperative CMS. It does not answer the question of whether the treatment is effective for this indication. A randomized controlled trial based on a syndrome severity scale should be conducted to provide a more reliable assessment of the efficacy and safety of fluoxetine.
Topics: Humans; Fluoxetine; Mutism; Male; Child; Female; Child, Preschool; Postoperative Complications; Retrospective Studies; Selective Serotonin Reuptake Inhibitors; Infratentorial Neoplasms; Cerebellar Diseases; Adolescent; Syndrome; Neurosurgical Procedures
PubMed: 38485568
DOI: 10.1016/j.arcped.2023.10.010 -
Journal of Neurology Jun 2024Primary angiitis of the central nervous system (PACNS) is a rare form of vasculitis solely affecting the vessels of the brain, spinal cord, and leptomeninges. A range of... (Review)
Review
BACKGROUND AND OBJECTIVE
Primary angiitis of the central nervous system (PACNS) is a rare form of vasculitis solely affecting the vessels of the brain, spinal cord, and leptomeninges. A range of magnetic resonance imaging (MRI) features have been associated with PACNS, including cerebral infarction, hemorrhage, and parenchymal or leptomeningeal contrast enhancement.
METHODS AND RESULTS
We describe a 51-year-old man with a case of PACNS manifesting as akinetic mutism with progressive leukoencephalopathy.
DISCUSSION
Progressive leukoencephalopathy has not been well defined as a manifestation of PACNS. We review a small number of cases with comparable features, providing additional context on this PACNS manifestation with consideration of clinical subtypes.
Topics: Humans; Male; Middle Aged; Brain; Leukoencephalopathies; Magnetic Resonance Imaging; Vasculitis, Central Nervous System
PubMed: 38478031
DOI: 10.1007/s00415-024-12283-7 -
Journal of the College of Physicians... Mar 2024To assess the clinical spectrum, treatment, and outcome of children with autoimmune encephalitis (AE).
OBJECTIVE
To assess the clinical spectrum, treatment, and outcome of children with autoimmune encephalitis (AE).
STUDY DESIGN
Descriptive study. Place and Duration of the Study: Department of Paediatrics, The Aga Khan University Hospital, Karachi, Pakistan, from January 2017 to December 2021.
METHODOLOGY
Medical records of children with a diagnosis of AE were reviewed for clinical features, treatment details, and outcomes. Outcome was defined as good (0-2) or poor (3-6) based on a modified Rankin Scale (mRS) score at 3-month follow-up. Descriptive statistics were reported and logistic regression was used to assess the prognostic factors associated with outcome.
RESULTS
Thirty-three patients were identified with AE. Thirteen (39.3%) were antibody positive. Anti-N-methyl-D-aspartate receptor (NMDAR) antibody was seen in 92% of positive cases. Behavioural abnormalities (87.8%), seizures (81.8%), movement disorders (66.6%), psychiatric symptoms (63.6%), and mutism (33.3%) were the prominent symptoms. Thirty (91%) patients received first-line immunotherapy. Good outcome was seen in 14 (48.2%) patients. Univariable analysis showed that the odds of having poor outcome were 2.5 (95% confidence interval [CI] 0.37-16.88, p=0.34) in patients with chorea. In addition, an elevated cerebrospinal fluid (CSF) protein had an odds ratio (OR) of 8.6 (CI 0.88-84.83, p=0.064) and positive CSF antibodies had an OR of 3.7 (CI 0.79-17.72, p=0.095) for a poor outcome. Mortality was seen in 4 (12.1%) patients.
CONCLUSION
A very low threshold is needed for the diagnosis of AE in children presenting with behavioural symptoms and chorea. Although the odds for poor prognosis were higher in patients with chorea, elevated CSF protein and positive CSF antibodies, the p-value did not come out significant.
KEY WORDS
Autoimmune encephalitis, Antibodies, NMDAR, Immunotherapies, mRS score, Outcome.
Topics: Humans; Child; Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Chorea; Retrospective Studies; Autoantibodies; Treatment Outcome; Encephalitis; Hashimoto Disease
PubMed: 38462869
DOI: 10.29271/jcpsp.2024.03.323 -
Cureus Feb 2024Oral health is a vital part of overall health, particularly for children with special healthcare requirements. The terms "dumb" and "mute" are frequently linked with...
Oral health is a vital part of overall health, particularly for children with special healthcare requirements. The terms "dumb" and "mute" are frequently linked with the term "deaf" due to the connection between hearing loss and speech impairment. A hearing and speech-impaired child may be unable to express completely because of the communication barriers. It is important to treat special children with utmost care and safety. This case report describes the dental management of an 8-year-old special child reported with multiple carious lesions under general anesthesia as she was not well acquainted with sign language. In a hospital setting under general anesthesia (GA), all necessary treatments are carried out in a single appointment. Since it is monitored by a multi-disciplinary team it can safely provide effective care to a child with hearing impairment and mutism.
PubMed: 38455789
DOI: 10.7759/cureus.53685 -
Neuropsychopharmacology Reports Jun 2024Catatonia, a psychomotor disorder characterized by diverse clinical signs, including stupor and mutism, remains elusive in its causes and a challenge to diagnose....
INTRODUCTION
Catatonia, a psychomotor disorder characterized by diverse clinical signs, including stupor and mutism, remains elusive in its causes and a challenge to diagnose. Moreover, it is often underrecognized due to its resemblance to disorders of consciousness. However, when diagnosing catatonia, an antipsychotic medication may exacerbate the condition. The first-line treatment typically includes benzodiazepines and/or electroconvulsive therapy (ECT).
CASE REPORT
A 60-year-old woman with systemic lupus erythematosus (SLE) and epilepsy presented with catatonic stupor. Despite stable treatment, she experienced an acute deterioration in consciousness, requiring hospitalization. Her condition improved markedly following a benzodiazepine challenge, as documented on EEG. This improvement was short-lived, but a second benzodiazepine challenge restored her from E1V1M1 (stupor) to E4V5M6 within minutes, as documented by a video recording. The patient was treated with lorazepam 1.5 mg/day orally and did not experience further relapses.
DISCUSSION
The diagnosis of catatonia had been based on her scores on the Bush-Francis Catatonia Rating Scale (BFCRS; Screening, 6/14; Severity, 19), despite meeting only two DSM-5 criteria for catatonia (stupor and mutism). The diagnosis was supported by EEG and video documentation, excluding other potential differential diagnoses such as nonconvulsive status epilepticus and encephalopathy. Additional quantitative EEG analyses indicated that benzodiazepine administration increased brainwide alpha and beta band power significantly, suggesting that the benzodiazepine normalized attention, consciousness, and long-range synchronization. This report additionally emphasizes the significance of video recordings in managing catatonia, and it helps in accurately tracking symptoms, documenting comprehensively, and improving patient understanding, which is crucial for treatment adherence.
Topics: Humans; Female; Catatonia; Middle Aged; Electroencephalography; Stupor; Benzodiazepines; Video Recording; Lorazepam
PubMed: 38453164
DOI: 10.1002/npr2.12427 -
Pediatric Neurology May 2024Postoperative pediatric cerebellar mutism syndrome (CMS) may occur following a process affecting the posterior cranial fossa. Recent evidence demonstrates disabling and...
BACKGROUND
Postoperative pediatric cerebellar mutism syndrome (CMS) may occur following a process affecting the posterior cranial fossa. Recent evidence demonstrates disabling and potentially lasting motor components of this syndrome, including ataxia, hemiparesis, and oculomotor dysfunction. These impairments may contribute to vestibular deficits.
METHODS
This case series contributes data to quantify vestibular dysfunction in postoperative CMS. The pair consisted of one female and one male.
RESULTS
Vestibular testing demonstrated both peripheral and central dysfunction.
CONCLUSIONS
Given these findings, a thorough vestibular assessment may be indicated as part of a comprehensive evaluation following a postoperative CMS diagnosis. Further research is needed to understand the pathophysiology, treatment, and long-term outcomes of postoperative pediatric CMS.
Topics: Child; Humans; Male; Female; Mutism; Cerebellar Neoplasms; Postoperative Complications; Cerebellar Diseases; Cranial Fossa, Posterior; Syndrome
PubMed: 38447508
DOI: 10.1016/j.pediatrneurol.2024.01.024 -
Practical Neurology Feb 2024A 30-year-old woman had 5 days of visual hallucinations, nystagmus, memory impairment and mutism. On examination, she was disorientated with reduced attention span,...
A 30-year-old woman had 5 days of visual hallucinations, nystagmus, memory impairment and mutism. On examination, she was disorientated with reduced attention span, gaze-evoked nystagmus, paratonia and abnormal frontal reflexes. Cerebrospinal fluid (CSF) showed 80 cells, protein 0.41 g/L and glucose 3.2 mmol/L (plasma glucose 5.0 mmol/L). MR scan of the brain showed involvement of limbic and extra-limbic regions and brainstem. Commercial cell-based assays were negative, but tissue-based assays showed neuropil staining, and cell-based assays for anti-metabotropic glutamate receptor 5 (mGluR5) antibodies were positive in serum and CSF. Six months later, she was diagnosed with Hodgkin's lymphoma. This case emphasises the broader clinical spectrum of anti-mGluR5 encephalitis, challenging its initial characterisation as Ophelia syndrome. It underscores the significance of interpreting commercial cell-based assays and advocates for tissue-based assay testing followed by cell-based assay testing in serum and CSF for diagnosing rare autoimmune encephalitis.
PubMed: 38423754
DOI: 10.1136/pn-2024-004089 -
Cureus Jan 2024Bilateral medial medullary infarction (BMMI) is a rare stroke subtype that accounts for less than 1% of acute strokes. Common manifestations of this stroke include...
Bilateral medial medullary infarction (BMMI) is a rare stroke subtype that accounts for less than 1% of acute strokes. Common manifestations of this stroke include quadriparesis, bilateral hypoglossal palsy, bilateral sensory loss, and respiratory failure. We present the case of a 39-year-old male with deafness and mutism who was brought to the emergency department due to acute onset of altered mental status and generalized weakness, further decompensated, and was lately diagnosed with bilateral medial medullary infarction. This case hopes to illustrate a differential diagnosis to be considered and promptly managed when a patient presents with altered mental status and quadriparesis, especially in the acute setting where tissue plasminogen activator (tPA) can still be given.
PubMed: 38406117
DOI: 10.7759/cureus.52965