Bilateral fracture of the shoulders following epileptic seizures: a case report / Fratura bilateral de ombros após convulsões epilépticas: relato de caso

Convulsive seizures caused by hyponatremia occur when this condition is severe and develops quickly, resulting in a brain's adaptive inability to contain brain swelling. Seizures are rarely the cause of shoulder fractures. This is a case report of bilateral humerus fracture following a single epileptic seizure caused by drug hyponatremia, an unconventional event in medical practice. A 69-year-old woman was admitted to the emergency room after a single tonic-clonic seizure with spontaneously ceased sphincter relaxation, showing Glasgow 6. No falls or restraint were reported by observers. When alert, the patient reported pain and difficulty moving both arms. During examination, the movement was li- mited to the right and left. Anteroposterior radiographs revealed bilateral fracture at the neck of humerus. To complement inves- tigation for further lesions, a computed tomography confirmed bilateral fracture-dislocation with impaction of the humeral head with the glenoid. Atraumatic bilateral fracture-dislocation of the humerus after epileptic seizure is a very rare event. It is believed that some of these diagnoses have been neglected due to the difficulty of characterizing the patient's pain in a postictal state. The importance of a detailed physical examination shall be emphasized in risk groups such as the polymedicated elderly.

Convulsive seizures caused by hyponatremia occur when this condition is severe and develops quickly, resulting in a brain's adaptive inability to contain brain swelling. Seizures are rarely the cause of shoulder fractures. This is a case report of bilateral humerus fracture following a single epileptic seizure caused by drug hyponatremia, an unconventional event in medical practice. A 69-year-old woman was admitted to the emergency room after a single tonic-clonic seizure with spontaneously ceased sphincter relaxation, showing Glasgow 6. No falls or restraint were reported by observers. When alert, the patient reported pain and difficulty moving both arms. During examination, the movement was li- mited to the right and left. Anteroposterior radiographs revealed bilateral fracture at the neck of humerus. To complement inves- tigation for further lesions, a computed tomography confirmed bilateral fracture-dislocation with impaction of the humeral head with the glenoid. Atraumatic bilateral fracture-dislocation of the humerus after epileptic seizure is a very rare event. It is believed that some of these diagnoses have been neglected due to the difficulty of characterizing the patient's pain in a postictal state. The importance of a detailed physical examination shall be emphasized in risk groups such as the polymedicated elderly.

Pure topographical disorientation in novel environments without anterograde amnesia: a case study.

Topographical disorientation (TD) in novel environments is considered to be a part of anterograde amnesia. A 56-year-old woman presented with pure TD only in novel environments following limbic encephalitis. She could not remember directions inside the hospital on weekly outpatient visits; however, her verbal and visual anterograde memories were normal. In the test of learning photographs of scenes, faces, and objects, only her scores for landscapes were worse than those in healthy controls. These findings suggested that her TD specific to landscapes and directions in novel environments was caused by category-specific memory impairment related to bilateral hippocampal and parahippocampal dysfunction.

Isolated seizures are a common early feature of paraneoplastic anti-GABAB receptor encephalitis.

OBJECTIVE: To report the clinical features and long-term outcome of 22 newly diagnosed paraneoplastic patients with GABAB receptor antibodies (GABABR-Abs). METHODS: Retrospective clinical study of CSF-confirmed cases of GABABR-Abs encephalitis. RESULTS: We identified 22 patients (4 female) with GABABR-Abs, with a median age of 64 years (range 55-85). All were paraneoplastic: 20 small-cell lung cancer, one malignant thymoma, and one uncharacterized lung mass. The most frequent first symptom was the isolated recurrent seizures without cognitive inter-ictal impairment in 17 patients (77%). In the other, three presented the first behavioral disorders and two presented de novo status epilepticus (SE). After a median delay of 10 days (range 1-30), the recurrent seizures' phase was followed by an encephalitic phase characterized by confusion in 100% of cases and SE in 81% (n = 17), with 53% (n = 9) non-convulsive SE. Dysautonomic episodes were frequent (36%, n = 8, bradycardia and central apnea) and killed three patients. CSF study was abnormal in 95% of the cases (n = 21). At the encephalitic phase, MRI showed a temporal FLAIR hypersignal in 73% (n = 16) of the cases. First-line immunotherapy was initiated after a median delay of 26 days (range 6-65) from disease onset, and a partial response was observed in 10 out of 20 patients (50%). There was no complete response. Two years after onset, a massive anterograde amnesia affected all still alive patients. Nine patients died from cancer progression (median survival: 1.2 years). CONCLUSION: Paraneoplastic GABABR-Abs encephalitis is characterized by a stereotype presentation with an epilepsy phase before an encephalitic phase with dysautonomia. The functional prognosis is poor.

A Case of Bariatric Surgery-related Wernicke-Korsakoff Syndrome with Persisting Anterograde Amnesia.

OBJECTIVE: To describe the theoretical and clinical implications of the neuropsychological evaluation of a case of bariatric surgery-related Wernicke-Korsakoff syndrome. METHOD: The patient was a 37-year old, female, bilingual, bachelor's degree educated, Mexican American public relations consultant without preexisting psychiatric, neurological, or substance abuse history. Recovery from laparoscopic sleeve gastrectomy surgery for morbid obesity was complicated by intraabdominal abscess, multibacterial infection, and prolonged nausea and vomiting. About 15 weeks post-surgery she was diagnosed with Wernicke's encephalopathy. She had a positive response to thiamine supplement but was left with persisting self-reported memory problems that were confirmed by family members. Multiple neuroimaging studies were all normal. RESULTS: A neuropsychological evaluation at 14 months post-surgery revealed anterograde amnesia for verbal and visual-perceptual material. There was no clear period of temporally graded retrograde amnesia. Scores on tests of visual-perceptual, language, fine motor, and executive functions were unimpaired. She had awareness of her neurocognitive impairment, but did not exhibit emotional distress. Follow-up neuropsychological evaluation at 17 months showed a similar neurocognitive profile with increased emotional distress. CONCLUSIONS: Her preserved executive functioning is theoretically important as it supports arguments that such impairment in alcohol use-related Korsakoff syndrome derives from the toxic effects of the prolonged misuse of alcohol and not vitamin deficiency. From a clinical perspective, neuropsychological evaluation of thiamine treated, bariatric surgery-related, Wernicke's encephalopathy cases is indicated if there is suspicion of residual memory impairment.

Human herpes virus-6 encephalitis causing severe anterograde amnesia associated with rituximab, azathioprine and prednisolone combination therapy for dermatomyositis.

Human herpes virus-6 (HHV-6) reactivation is a well-recognised complication following haematological stem cell transplantation, but it is novel in the context of combination immunomodulatory therapy for autoimmune disease. We report a case of severe anterograde amnesia caused by HHV-6 encephalitis in a young female patient on rituximab, azathioprine and prednisolone for dermatomyositis (DM). The use of targeted biologic treatments for systemic autoimmune connective tissue diseases (CTDs) is increasing, particularly when refractory to conventional management. The anti-CD20 B cell depleting monoclonal antibody, rituximab is now increasingly used, often in combination with conventional immunomodulatory treatments, in certain autoimmune neurological conditions and systemic CTDs including DM. Physicians should be aware of the possibility of HHV-6 in those who develop encephalitis while CD20 B cell deplete, especially in the presence of additional immunomodulatory therapies. Prompt diagnosis and treatment of HHV-6 encephalitis with evidence-based anti-viral therapy may help reduce the extent of irreversible morbidity such as amnesia.

Autobiographical age awareness disturbance syndrome in autoimmune limbic encephalitis: two case reports.

BACKGROUND: Autobiographical memory is a form of episodic memory characterized by a sense of time and consciousness that enables an individual to subjectively re-experience his or her past. As part of this mental re-enactment, the past is recognized relative to the present. Dysfunction of this memory system may lead to confusion regarding the present perception of time. CASE PRESENTATION: Two Japanese women (42 and 55 years old) temporarily believed they were living in their past during a course of autoimmune limbic encephalitis. Their autobiographical memories and behaviour reflected their self-estimated age, and they could not recall memories experienced beyond that age. More surprisingly, their subjective age estimations and autobiographical memories were transiently corrected when they were made aware of their true age. Disorientation, anterograde amnesia, and retrograde amnesia were common additional symptoms. Neuroimaging suggested disturbances in medial temporal and orbitofrontal brain regions in both cases. CONCLUSIONS: This syndrome is characterized by three elements: 1) failure to subjectively recognize the present; 2) inability to suppress irrelevant past memories; and 3) transient restitution of awareness of the present through realization of the individual's true age. We defined this syndrome as 'autobiographical age awareness disturbance', and focused our investigation on the role of age self-awareness. If recall of relevant and suppression of irrelevant past memories are both necessary to subjectively recognize the present relative to the past, dysfunction of medial temporal and orbitofrontal brain regions is predicted to lead to abnormal subjective placement in time. However, the subjective experience of age tends to be an important informational component for retrieving remote autobiographical memories. This suggests that correct age awareness is essential for the proper recognition of the remote past in relation to the present. This is the first report to focus on the relationship between subjective temporal orientation and age self-awareness. While the role of age awareness in this process is still unclear, investigating autobiographical age awareness disturbance as a part of subjective temporal awareness dysfunction can be useful in understanding the processes underlying human time recognition.

Neuropsychiatric Manifestations of Colloid Cysts: a review of the literature.

Colloid cysts account for approximately 2% of primary brain tumours and the majority of cases are identified in the fourth and fifth decade. They are small, gelatinous neoplasms lined by a single layer of mucin-secreting columnar epithelium that are thought to arise from errors in folding of the primitive neuroepithelium. They develop in the rostral aspect of the third ventricle in the foramen of Monro in 99% of cases and despite their benign histology carry a poor prognosis, with a mortality greater than 10% in symptomatic cases. The location of colloid cysts within the ventricular system results in obstruction of the foramen of Monro as the cyst grows, disrupting the circulation of cerebrospinal fluid (CSF) and causing hydrocephalus. This is the mechanism behind the most common presenting symptoms of postural headache, nausea and vomiting - a clinical picture synonymous with hydrocephalus and intracranial pathology. In addition to these classical neurological symptoms, there is a high prevalence of psychiatric symptoms in the patient population, with symptoms ranging from anterograde amnesia to gustatory hallucination. These symptoms can occur with or without the presence of hydrocephalus, and are thought to be secondary to compression of connecting pathways between the mesocortices and subcortical limbic regions. These symptoms have been shown to be comparative in frequency to the classical symptoms, yet are rarely the reason for referral to a neurological or neurosurgical service for investigation.

The Role of Baseline Cognitive Function in the Neurocognitive Effects of Electroconvulsive Therapy in Depressed Elderly Patients.

OBJECTIVE: Electroconvulsive therapy (ECT) is an effective biological treatment option for severely depressed elderly patients; however, it can cause cognitive side effects, including anterograde and retrograde amnesia. Elderly patients with "cognitive impairment no dementia" (CIND) are reported as being more vulnerable to the cognitive side effects of ECT compared with patients with "no cognitive impairment" (NCI). The few studies that have reached this conclusion can be criticized for using insensitive outcome measures. METHOD: The present study investigated cognitive side effects using standard neuropsychological tests before and after twice-weekly ECT. Patients were assessed at baseline (T1) and within one week after a course of ECT (consisting of a mean of 10 treatments) (T2), and were followed up for three months after T2 (T3). The sample included 54 patients with NCI (n = 36) or CIND (n = 18). For a control group, we recruited 17 healthy elderly persons. Tests of anterograde memory, information-processing speed, executive function, and retrograde memory were administered. We computed reliable change indices using simple regression methods. RESULTS: Short-term side effects were detected at T2 in a large minority of patients, with no significant differences between NCI and CIND patients. Considerable improvement in global cognitive function from T1 to T3 was observed in 44% of the CIND patients. At the group level, information-processing speed improved significantly in CIND vs. NCI patients. CONCLUSIONS: CIND patients were not more vulnerable to amnesia than were NCI patients. Long-term cognitive side effects of ECT were not detected.

Hypoxia/reoxygenation impairs memory formation via adenosine-dependent activation of caspase 1.

After hypoxia, a critical adverse outcome is the inability to create new memories. How anterograde amnesia develops or resolves remains elusive, but a link to brain-based IL-1 is suggested due to the vital role of IL-1 in both learning and brain injury. We examined memory formation in mice exposed to acute hypoxia. After reoxygenation, memory recall recovered faster than memory formation, impacting novel object recognition and cued fear conditioning but not spatially cued Y-maze performance. The ability of mice to form new memories after hypoxia/reoxygenation was accelerated in IL-1 receptor 1 knockout (IL-1R1 KO) mice, in mice receiving IL-1 receptor antagonist (IL-1RA), and in mice given the caspase 1 inhibitor Ac-YVAD-CMK. Mechanistically, hypoxia/reoxygenation more than doubled caspase 1 activity in the brain, which was localized to the amygdala compared to the hippocampus. This reoxygenation-dependent activation of caspase 1 was prevented by broad-spectrum adenosine receptor (AR) antagonism with caffeine and by targeted A1/A2A AR antagonism with 8-cyclopentyl-1,3-dipropylxanthine plus 3,7-dimethyl-1-propargylxanthine. Additionally, perfusion of adenosine activated caspase 1 in the brain, while caffeine blocked this action by adenosine. Finally, resolution of anterograde amnesia was improved by both caffeine and by targeted A1/A2A AR antagonism. These findings indicate that amygdala-based anterograde amnesia after hypoxia/reoxygenation is sustained by IL-1ß generated through adenosine-dependent activation of caspase 1 after reoxygenation.

[Memory improvement in patients with temporal lobe epilepsy at one-year postoperative]. / Amélioration mnésique postopératoire un an après traitement chirurgical de l'épilepsie du lobe temporal.

INTRODUCTION: Several studies have assessed the impact of surgery on both anterograde and remote memory in patients with temporal lobe epilepsy (TLE). The majority of results have shown an extensive memory deficit in patients after temporal resection (TL). However, few protocols have used a prospective longitudinal design. Moreover, the postoperative delays were variable from one study to the next, instead of regular monitoring to identify the potential effect of time elapsed after surgery on memory performance. In addition, some studies have not used strict inclusion criteria to establish homogeneous patient groups. Finally, the impact of surgery on memory has been often assessed by comparing memory skills between epileptic patients and healthy controls. Our aim was to examine the impact of TL on memory in patients with TLE, recruited according to clear-cut clinical criteria. Moreover, we focused on memory performance progression per se in epileptic patients pre- and postoperatively, rather than on memory performance analysis expressed as "deficient" or "normal". METHODS: We assessed 30 patients with unilateral TLE (17 right TLE and 13 left TLE) on four anterograde memory tests and six remote memory tasks. Patients completed all tests preoperatively, and 5 and 12 months after TL. RESULTS: Five months after surgery, performance was equivalent to the preoperative scores for both groups in anterograde memory tasks and remote memory tests. One year after TL, patients with right TLE showed enhanced performance in the verbal anterograde memory tests and in retrieving recent autobiographical memories. Results for left TL showed improved scores only in a recognition memory test of faces. CONCLUSIONS: In the present study, surgical patients were "double winners" gaining seizure freedom and potential of memory stability or recovery. The gain was observed only 12 months after surgery and following temporal resection lateralisation. Our data showed postoperative memory improvement in patients with temporal lobe epilepsy presenting with specific clinical characteristics.

SenseCam as a rehabilitation tool in a child with anterograde amnesia.

We present the case of a 13-year-old boy, CJ, with profound episodic memory difficulties following the diagnosis of a metastatic intracranial germ cell tumour and subsequent treatment with radiotherapy and chemotherapy. At the core of this study is the first application of SenseCam to a child with severe memory impairment. CJ was taken for a walk while he was wearing SenseCam. This included visiting four different locations. We manipulated the number of locations he could review on SenseCam "films" and then tested recognition memory (forced choice) for both reviewed and non-reviewed locations. We also collected his justifications for the choices he made. Our results indicate that repeated viewings of SenseCam images support the formation of personal semantic memories. Overall our results suggest that the use of SenseCam in memory rehabilitation extends beyond supporting episodic memory and recollection, and supports the feasibility of its use with children who have marked memory difficulties.

Apical ballooning resulting from limbic encephalitis.

BACKGROUND: Neurogenic stunned myocardium is an increasingly recognized cause of left ventricular apical ballooning, or takotsubo cardiomyopathy. We report the first case of neurogenic stunned myocardium as a result of limbic encephalitis. METHODS: This 73-year-old woman with anterograde and retrograde amnesia was investigated using electrocardiography, magnetic resonance imaging, and left ventricular angiography. RESULTS: Electrocardiography showed deep T-wave inversions in multiple leads, magnetic resonance imaging demonstrated increased signal on fluid-attenuated inversion recovery images symmetrically within the medial temporal lobes consistent with limbic encephalitis. Left ventricular angiography showed apical ballooning. CONCLUSIONS: Because the insula has extensive interconnections with limbic structures, limbic encephalitis could lead to alterations in the sympathetic regulation of the insular cortex resulting in neurogenic stunned myocardium.

Understanding hippocampal sclerosis in the elderly: epidemiology, characterization, and diagnostic issues.

Hippocampal sclerosis (HS) is a pathologic term used to describe severe loss of neurons and reactive gliosis without cystic cavitation in the CA1 sector of the hippocampus. In late life, HS is associated with hippocampal atrophy, severe amnesia, and slowly progressive dementia without clinical seizure activity. HS is difficult to distinguish clinically from Alzheimer's disease and is often diagnosed postmortem. In autopsy series, HS may be found without significant other pathology (2%-4% of cases), but it occurs frequently in combination with other vascular and neurodegenerative disorders (12%-20% of cases). HS is found bilaterally in 50% of cases and unilaterally in 50% of cases, with similar predilection for the right versus left hemisphere. The pathogenesis of HS is unknown and may be multifactorial in origin, possibly due to anoxic/ischemic injury or TDP-43-related neurodegeneration. Little is known about the prevention and treatment of late-life HS, although circumstantial evidence suggests the importance of identifying and treating vascular risk factors.

[Non-paraneoplastic limbic encephalitis revealed by anterograde amnesia]. / Une encéphalite limbique non paranéoplasique révélée par une amnésie antérograde.

INTRODUCTION: Limbic encephalitis is a syndrome, most commonly paraneoplastic, related to an often undiagnosed cancer of unpredictable prognosis. Neurological symptoms are progressive for a few weeks before stabilizing. CASE: We report a case of limbic encephalitis in a 56-year-old man, revealed by anterograde amnesia. The diagnosis was suggested after magnetic resonance imaging (MRI) showed bilateral hippocampal lesions, with signals that were hypointense in IR sequences and hyperintense in FLAIR. The non-neoplastic causation was suggested by an array of clinical, laboratory, imaging, and therapeutic arguments. After five years of follow-up, no neoplasia has been found. DISCUSSION: This case provides the opportunity for a comparison of the radiologic, imaging, and neurologic findings related to bilateral lesions of the hippocampus.

Memento ... life imitates art: the request for an ethics consultation.

This was a troublesome case, without complete consensus about the right thing to do. It seemed there were arguments on both sides: those who thought that the goal of comfort care without treatment was most consistent with his wishes, versus those who thought the additional testing and treatment might have produced a net benefit in spite of the burdens. Ultimately, the patient was placed in a facility with a do-not-resuscitate (DNR) order in effect and a plan for comfort care. He may live a few months or more. We all hope that whatever time he has is spent free of pain and symptoms, and that he is able to live a life of quality consistent with his wishes. The main story line is consistent with actual events; however, there have been changes in terms of gender, disease, and relationships that have been included to protect the anonymity of the patient.

Immediate peri-operative memory.

BACKGROUND: Information about anterograde and retrograde amnesias in the immediate peri-operative period is scarce. During this period, assessment of memory for real-life events is also rare. We hypothesized that there would be both anterograde and retrograde amnesias and memory for peri-operative events would be better than for verbal memory. METHODS: We studied 40 patients who underwent general anesthesia and surgery and 19 control volunteers who were matched to the patients but did not have surgery. Patients completed the state anxiety part of the Spielberger State-Trait Anxiety Inventory in the pre-operative period. They were presented with three word lists in the holding area, the operating room before induction of anesthesia and the recovery room. Memory for the words was tested the next day by recall and recognition tests. Memory for events that happened on the day of surgery was tested by administering a questionnaire. The control subjects were tested similarly but did not complete the events questionnaire. Retrograde amnesia would be demonstrated by a decline in patients' memory from the holding area to the operating room which exceeded any corresponding changes in controls; anterograde amnesia would be demonstrated by memory impairment of the patients in the recovery room, relative to controls. RESULTS: Recall and recognition of words were significantly impaired in the recovery room with a decline from 12% in the holding area to zero% in the recovery room for recall and from 43% to 7% for recognition. The decline in memory from the holding area to the operating room was not significantly greater in patients than in controls, 80% vs. 56% for recall and 27% vs. 14% for recognition. There were no significant differences for recognition of events which happened in the three rooms. CONCLUSIONS: We were unable to detect retrograde amnesia. Patients' memory for neutral stimuli in the recovery room was severely impaired. The events questionnaire proved to be insensitive.

Clinical application of functional MRI for memory using emotional enhancement: deficit and recovery with limbic encephalitis.

Although some functional MRI memory studies show reliable neural activity in the hippocampus and mesial temporal lobe (MTL), most typically report results from group studies. However, fMRI memory probes need to be robust enough to show MTL activity in individual patients to be helpful in diagnosis and treatment planning. We present the case of a patient with non-paraneoplastic limbic encephalitis who had severe anterograde amnesia with subsequent recovery to illustrate a fMRI probe of MTL activity that is easily administered to neurological patients. The task uses emotionally positive and affiliative stimuli to elicit responsivity in the amygdala-hippocampus region. In this patient, weak bilateral hippocampal activation was observed in the acute stage that increased after recovery, paralleling findings on structural MRI and neuropsychological memory assessment. This case study demonstrates that using emotional stimuli to enhance memory responsivity may be an effective way to visualize clinical changes in individual patients.