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Clinical Medicine Insights. Case Reports 2022Tacrolimus is a calcineurin inhibitor (CNI), an immunosuppressive agent used to prevent graft versus host disease following allogeneic hematopoietic cell transplantation...
Tacrolimus is a calcineurin inhibitor (CNI), an immunosuppressive agent used to prevent graft versus host disease following allogeneic hematopoietic cell transplantation (HCT). Side-effects of tacrolimus treatment include neuropsychiatric symptoms, for example, affective disturbances, psychosis, and akinetic mutism. The onset of side-effects is independent of tacrolimus blood concentration and can occur years after treatment initiation. To our knowledge, case-reports describing tacrolimus-induced neuropsychiatric symptoms following HCT are sparse. This article reports the case of a 60-year-old woman with T-cell prolymphocytic leukemia, who developed memory loss, affective disturbances, and delusions, 1-year after HCT, and tacrolimus treatmentinitiation. Upon hospital admission, she was motionless and mute, albeit easily roused. The routine physical examination was without pathological findings. Blood work and microbiological analyses of blood and cerebrospinal fluid were normal. The neuroimaging showed chronic structural changes without relation to the debut of neuropsychiatric symptoms. Tacrolimus was discontinued on suspicion of tacrolimus-induced neuropsychiatric symptoms. The patient recovered within 48 hours of discontinuation. She was switch to prednisone treatment, and there has been no reemergence of neuropsychiatric symptoms since.
PubMed: 35342316
DOI: 10.1177/11795476221087053 -
Frontiers in Human Neuroscience 2022This paper presents a case in whom a differential diagnosis of akinetic mutism with a disorder of consciousness was made using diffusion tensor tractography (DTT). A...
This paper presents a case in whom a differential diagnosis of akinetic mutism with a disorder of consciousness was made using diffusion tensor tractography (DTT). A 69-year-old female patient was diagnosed with subarachnoid hemorrhage, intraventricular hemorrhage, and intracerebral hemorrhage produced by the subarachnoid hemorrhage. She exhibited impaired consciousness with a Coma Recovery Scale-Revised score of 13 until 1 month after onset. Her impaired consciousness recovered slowly to a normal state according to the Coma Recovery Scale-Revised (23 points: full score) at 7 weeks after onset. On the other hand, she exhibited the typical clinical features of akinetic mutism (no spontaneous movement [akinesia] or speech [mutism]). On the DTT performed at 1-month, the upper, and lower dorsal ascending reticular activating systems, which are related to a disorder of consciousness, showed an almost normal state. In contrast, the prefronto-caudate and prefronto-thalamic tracts, which are related to akinetic mutism, showed severe injuries. These DTT results suggested that the patient's main clinical features were not a disorder of consciousness but akinetic mutism. Therefore, DTT for the ascending reticular activating system, and the prefronto-caudate and prefronto-thalamic tracts could provide additional evidence for a differential diagnosis of DOC and AM at the early stages of stroke.
PubMed: 35280207
DOI: 10.3389/fnhum.2022.778347 -
Therapeutic Advances in Neurological... 2022Ustekinumab, a monoclonal antibody against interleukin (IL)-12 and IL-23 approved for the treatment of Crohn's disease, has shown to be an effective therapy with a...
Ustekinumab, a monoclonal antibody against interleukin (IL)-12 and IL-23 approved for the treatment of Crohn's disease, has shown to be an effective therapy with a favourable safety profile. Clinical trials and real-world studies have reported very few neurological adverse events, including posterior reversible encephalopathy syndrome, idiopathic intracranial hypertension and headache. We describe the case of a 48-year-old man with Crohn's disease who initiated treatment with ustekinumab on top of ongoing treatment with methotrexate 25 mg/week who presented with an acute-onset encephalopathy that rapidly evolved to severe tetraparesis and akinetic mutism, associated with extensive leukoencephalopathy and restricted diffusion on brain magnetic resonance imaging (MRI), 1 month after the second dose of ustekinumab. Comprehensive in-patient diagnostic testing ruled out vascular, demyelinating, metabolic, tumoral and infectious etiologies. Brain biopsy showed patchy infiltrates of foamy histiocytes with perivascular distribution, associated with edema, diffuse astrocytic gliosis and focal perivascular axonal destruction without demyelination, and ustekinumab-induced neurotoxicity was suspected. After drug discontinuation, the patient presented a complete clinical recovery despite the persistence of leukoencephalopathy. In conclusion, in an era in which biological therapies are continually evolving and expanding, knowledge about the potential neurotoxicity of these new therapies and their management becomes crucial. Although ustekinumab-induced encephalopathy is uncommon, the recognition of this potentially serious side effect is important because prompt withdrawal is associated with a favourable outcome. Whether methotrexate played an additional contributing role is currently unknown, but it is a factor that should be considered.
PubMed: 35237349
DOI: 10.1177/17562864221079682 -
Medical Science Monitor : International... Feb 2022Akinetic mutism (AM) is characterized by the complete absence of spontaneous behavior (akinesia) and speech (mutism) with the preservation of executive functions for... (Review)
Review
A Review of Studies on the Role of Diffusion Tensor Magnetic Resonance Imaging Tractography in the Evaluation of the Fronto-Subcortical Circuit in Patients with Akinetic Mutism.
Akinetic mutism (AM) is characterized by the complete absence of spontaneous behavior (akinesia) and speech (mutism) with the preservation of executive functions for movements and speaking. Elucidation of the pathophysiological mechanisms or neural correlates for AM is clinically important because patients can recover from AM after medication and neuromodulation. The fronto-subcortical circuit is a critically important neural structure in the pathophysiology of AM. Using diffusion tensor tractography, a few neural tracts in the fronto-subcortical circuit can be reconstructed. This mini-review article evaluated 6 DTT-based studies on the fronto-subcortical circuit injury in patients with AM. According to these results, the neural tracts among the fronto-subcortical circuit, which are related to AM, were as follows (in decreasing order of importance): 1) the prefronto-caudate tract, 2) the prefronto-thalamic tract, and 3) the cingulum. In particular, the medial prefrontal cortex is an important brain area related to recovery from AM. However, only 6 studies on this topic have been published, and most were case reports. In addition, these studies analyzed only a few neural tracts in the fronto-subcortical circuit. Because AM is a rare disorder, studies involving a large number of subjects might be impossible. Nevertheless, an analysis of various neural tracts in the fronto-subcortical circuit is necessary. For this, reconstruction of the other neural tracts in the fronto-subcortical circuit should be performed first. This review aims to present the findings from recent studies on the role of DTT in evaluation of fronto-subcortical circuit injury in patients with AK.
Topics: Akinetic Mutism; Diffusion Magnetic Resonance Imaging; Diffusion Tensor Imaging; Humans; Neural Pathways; Prefrontal Cortex
PubMed: 35181647
DOI: 10.12659/MSM.936251 -
Frontiers in Neurology 2021Catatonia is a psychomotor syndrome common to several medical and neuropsychiatric disorders. Here, we report on the case of a 95-year-old woman who underwent a radical...
Catatonia is a psychomotor syndrome common to several medical and neuropsychiatric disorders. Here, we report on the case of a 95-year-old woman who underwent a radical change in personality characterized by sexual disinhibition, and physical and verbal aggressiveness. Over several months, she developed verbal stereotypies, gait deterioration, and double incontinence. She eventually developed mutism and an active opposition to all attempts to be fed or cared for. Benzodiazepines, olanzapine and electroconvulsive therapy were of no benefit. Magnetic resonance imaging revealed asymmetric (more severe on the right) frontotemporal, parietal, and upper brainstem atrophy. She died from sepsis without recovering from stupor seven years after the onset of symptoms. We believe that the initial behavioral disinhibition was related to the frontotemporal injury, whereas catatonic stupor reflected the progression of the degenerative process to the parietal cortices. Our case adds to the small number of cases of catatonia as a symptom of degenerative dementia. It also supports the idea that damage to the parietal cortex gives rise to pathological avoidance of which catatonic stupor represents an extreme form.
PubMed: 35115996
DOI: 10.3389/fneur.2021.798264 -
Cureus Dec 2021Delayed leukoencephalopathy in the aftermath of toxic exposure and cerebral hypoxia-ischemia is known as "delayed post-hypoxic leukoencephalopathy" (DPHL) but the name...
Delayed leukoencephalopathy in the aftermath of toxic exposure and cerebral hypoxia-ischemia is known as "delayed post-hypoxic leukoencephalopathy" (DPHL) but the name "delayed toxic-hypoxic leukoencephalopathy" (DTHL) may be more accurate if toxic and hypoxic mechanisms are both involved in the pathogenesis of delayed leukoencephalopathy. DTHL is characterized by initial recovery from toxic exposure and cerebral hypoxia-ischemia, clinical stability over a few weeks, and subsequent neurological deterioration with the sudden emergence of diffuse white matter disease. A 46-year-old man suffered respiratory failure and hypotension as a result of opioid overdose. Brain MRI showed watershed infarcts and EEG showed diffuse theta-delta slowing consistent with global cerebral hypoperfusion. He recovered fully and was discharged with intact cognitive function. Three weeks later, he presented with abulia and psychomotor retardation. MRI revealed extensive white matter hyperintensity and EEG showed diffuse polymorphic delta activity. DTHL was diagnosed based on classic MRI features, history of opioid overdose and hypoxic brain injury, and negative test results for etiology of white matter disease. He developed akinetic mutism prompting administration of methylprednisolone 1000-mg IV q24h for five days. He also received amantadine 100-mg PO q12h. His cognition, motivation, and psychomotor function slowly improved and returned to baseline about two months after the overdose. Clinic reassessment two and a half months after the overdose revealed normal cognitive function, slight residual MRI hyperintensity, and mild EEG slowing anteriorly. Toxic-metabolic myelinopathy causing diffuse demyelination in the deep white matter is a perfect explanation for the patient's neurological symptoms, MRI changes, EEG findings, and time course of recovery.
PubMed: 35004070
DOI: 10.7759/cureus.20271 -
Clinical Neurology and Neurosurgery Feb 2022We report 3 cases of Global rostral midbrain syndrome (GRMS) and Corpus Callosum (CC) infarction, in the context of hydrocephalus followed by shunt dysfunction and slit...
We report 3 cases of Global rostral midbrain syndrome (GRMS) and Corpus Callosum (CC) infarction, in the context of hydrocephalus followed by shunt dysfunction and slit ventricles. Prior shunt implantation had been indicated for adult-onset hydrocephalus secondary to aqueductal stenosis of varying causes. All three patients had been stable for months before developing repeated shunt dysfunctions, ultimately progressing to parkinsonism, Parinaud syndrome, akinetic mutism, pyramidal signs, cognitive impairment, CC infarction and slit ventricles, in the context of CSF overdrainage. Parkinsonism-related symptoms responded to dopa in all cases, but Parinaud syndrome and cognitive impairment persisted. Although GRMS has been described in the context of a transtentorial pressure gradient after shunt blockage, in these three cases with similar clinical presentation, reverse transtentorial pressure gradient and slit ventricles due to shunt overdrainage was the likely cause. The authors discuss the role of CC infarction and provide a detailed analysis after gathering previously described data, to unify information under a recognizable clinical entity and better understand the underlying pathophysiology, treatment options and outcome.
Topics: Adult; Cerebrospinal Fluid Shunts; Corpus Callosum; Humans; Hydrocephalus; Infarction; Mesencephalon; Ventriculoperitoneal Shunt; Ventriculostomy
PubMed: 34973650
DOI: 10.1016/j.clineuro.2021.107098 -
Prion Dec 2022An 84-year-old woman who had been diagnosed as having dementia with Lewy body (DLB) upon initial examination exhibited cognitive impairments and person delusional...
An 84-year-old woman who had been diagnosed as having dementia with Lewy body (DLB) upon initial examination exhibited cognitive impairments and person delusional misidentification (DMS): she transiently claimed that her spouse was a stranger. She was re-examined at the age of 89 years; her frequency of speech and activities of daily living had both decreased, leading to verbal communication difficulties complicated by sensory aphasia, and brain diffusion-weighted (DW) magnetic resonance imaging (MRI) showed cortical hyperintensities in some areas of both hemispheres. About 4 months later, the DW high-intensity areas were observed to have expanded into diffuse cortical areas. While the clinical features of Creutzfeldt Jakob disease (CJD) (myoclonus; ataxia; parkinsonism; rapidly progressive cognitive impairments; periodic sharp discharges on electroencephalograms) were not observed, a genetic analysis of the prion protein () gene, which was performed because of a family history of dementia, revealed a V180I mutation (heterozygosis: valine/isoleucine) suggesting genetic CJD (g-CJD). Her activity progressively decreased, reaching akinetic mutism about 11 months after the re-examination. Finally, she suffered from severe bedsores and died from aspiration pneumonia at the age of 90 years. The present report describes the first case of person DMS as an initial neuropsychiatric symptom for V180I g-CJD; the typical long-term clinical symptoms of CJD were not observed in this patient. The inclusion of person DMS as an initial clinical symptom and the presence of expansive cortical hyperintensity areas may be useful for clinicians attempting to diagnosis V180I g-CJD in patients with elusive symptoms.
Topics: Activities of Daily Living; Aged, 80 and over; Creutzfeldt-Jakob Syndrome; Female; Humans; Mutation; Prion Proteins; Prions
PubMed: 34965177
DOI: 10.1080/19336896.2021.2017701 -
Neurologia (Barcelona, Spain) Oct 2022
PubMed: 34785833
DOI: 10.1016/j.nrl.2021.09.007 -
Case Reports in Psychiatry 2021. Catatonia is caused by a variety of psychiatric and organic conditions. The onset, clinical profile, and response to treatment may vary depending on the underlying...
. Catatonia is caused by a variety of psychiatric and organic conditions. The onset, clinical profile, and response to treatment may vary depending on the underlying cause. Catatonia is more likely to be associated with neurotic and psychotic disorders, but some psychiatric symptoms are key components in the clinical presentation of other medical conditions. . We report the case of a woman who started showing paroxysmal recurrent episodes since the age of 57 years, characterized by surrounding disconnection, disorientation, and muscle spasm (myoclonus), followed by a postictal state. In the following months, the symptoms evolved to akinetic mutism, catatonia, and rapidly progressive vision and audition loss. She underwent a battery of tests, most of them inconclusive, until a neoplastic meningoencephalitis was diagnosed after more than two years of symptoms. Numerous medical conditions can mimic psychiatric disorders. This uncommon presentation may lead to a late diagnosis and treatment initiation, increasing significantly morbidity and mortality. A differential diagnosis with infectious, autoimmune, and neoplastic etiologies should always be carried out.
PubMed: 34745679
DOI: 10.1155/2021/5936673