scholarly journals The Essential Tremors: Evolving Concepts of a Family of Diseases

2021 ◽  
Vol 12 ◽  
Author(s):  
Elan D. Louis
Keyword(s):  
Essential Tremor ◽  
Postmortem Changes ◽  
Disease Etiology ◽  
Heterogeneous Features ◽  
System Degeneration ◽  
History Of ◽  
Postmortem Studies ◽  
Essential Tremors ◽  
Gait Abnormalities ◽  
Remarkable Advance

The past 10 years has seen a remarkable advance in our understanding of the disease traditionally referred to as “essential tremor” (ET). First, the clinical phenotype of ET has been expanded from that of a bland, unidimensional, and monosymptomatic entity to one with a host of heterogeneous features. These features include a broader and more nuanced collection of tremors, non-tremor motor features (e.g., gait abnormalities) and a range of non-motor features, including cognitive, psychiatric, sleep, and other abnormalities. The natural history of these features, as well as their relationships with one another and with disease duration and severity, are better appreciated than they were previously. Studies of disease etiology have identified a number of candidate genes as well as explored several environmental determinants of disease. In addition, the decade has seen the beginnings and expansion of rigorous postmortem studies that have identified and described the postmortem changes in the brains of patients with ET. This emerging science has given rise to a new notion that the disease, in many cases, is one of cerebellar system degeneration. Across all of these studies (clinical, etiological, and pathophysiological) is the observation that there is heterogeneity across patients and that “essential tremor” is likely not a single disease but, rather, a family of diseases. The time has come to use the more appropriate terminology, “the essential tremors,” to fully describe and encapsulate what is now apparent. In this paper, the author will review the clinical, etiological, and pathophysiological findings, referred to above, and make the argument that the terminology should evolve to reflect advances in science and that “the essential tremors” is a more scientifically appropriate term.

861 Vascular dementia presenting as rapidly progressive dementia secondary to zolpidem

SLEEP ◽  
2021 ◽  
Vol 44 (Supplement_2) ◽  
pp. A335-A335
Author(s):  
Cameron Barber ◽  
Dylan Carroll ◽  
Bhavani Suryadevara
Keyword(s):  
Vascular Dementia ◽  
Microvascular Disease ◽  
Altered Mental Status ◽  
Progressive Dementia ◽  
Personality Changes ◽  
The Us ◽  
History Of ◽  
Gait Abnormalities ◽  
Scheduled Time ◽  
Chronic Use

Abstract Introduction Rapidly progressive dementia is a condition with a wide differential which remains difficult to accurately diagnose. The potential pathologies responsible include thyroid, vitamin, and electrolytes abnormalities, infectious, and malignant causes. Vascular dementia, however, typically has a slow and insidious presentation. Zolpidem (Ambien) is among the top 50 prescribed medications in the US. Report of case(s) An 84-year-old Caucasian male with a past medical history of insomnia, and sleep apnea who is noncompliant with CPAP presented after a fall associated with altered mental status. He has taken zolpidem 10 mg nightly for over six years. The patient and wife reported notable personality changes beginning six months prior, as well as four months of progressively worsening auditory and visual hallucinations. Additionally, the patient noted developing urinary incontinence, and worsening gait steadiness with recurrent falls. The patient then developed sleep-wake inversion during the three weeks prior to his fall, and an outpatient referral to neurology was subsequently sent for dementia evaluation. On the night prior to his presentation, the patient took his usual nighttime zolpidem at 22:00 and later fell and was unable to get up. Subsequent testing was negative for reversible causes of dementia and MRI Brain revealed only chronic microvascular disease. His zolpidem dose was decreased to 5 mg and scheduled earlier which resulted in the resolution of his hallucinations, gait abnormalities, and acute encephalopathy. Conclusion One month later, the patient presented to the hospital after a repeat fall secondary to taking his zolpidem at his previously scheduled time. Once more, his dosage was further decreased to 2.5 mg and scheduling earlier, resulting again, in the complete resolution of his symptoms. Zolpidem, has an increased potential for delirium in elderly patients and especially those with dementia. Chronic use of zolpidem with insidiously progressive vascular dementia led to a worsening delirium which resolved after adjustment of timing and reduction of zolpidem dosing. Support (if any):

Severe hydralazine-induced lupus presenting as systemic lupus erythematosus

Lupus ◽  
2020 ◽  
Vol 29 (5) ◽  
pp. 509-513 ◽  
Author(s):  
R L Rubin ◽  
R F Haluptzok ◽  
L M Davila
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Autoimmune Disease ◽  
Systemic Lupus ◽  
Drug Usage ◽  
Drug Induced ◽  
Disease Etiology ◽  
History Of ◽  
Anti Inflammatory ◽  
Symptoms And Signs

Despite its long history of untoward side effects of a systemic autoimmune disease, drug-induced lupus can be difficult to recognize because of the disconnect between chronic drug usage and onset of symptoms. In this case, the patient was treated with hydralazine for two years when symptoms were initially reported, but a diagnosis of hydralazine-induced lupus was not considered for another half year. Despite treatment with steroidal and nonsteroidal anti-inflammatory medications during this period, rheumatologic symptoms and signs continued to deteriorate, consistent with the diagnosis of systemic lupus erythematosus. Not until the patient voluntarily discontinued hydralazine did symptoms begin to improve, fully resolving over the subsequent 6–12 months largely in the absence of anti-inflammatory medication. This patient demonstrates that failure to recognize a drug-induced disease etiology can result in substantial worsening of rheumatologic symptoms over the subsequent six months, ultimately satisfying criteria for systemic lupus erythematosus. While symptoms and signs largely normalized, some laboratory abnormalities and occasional arthralgia remained two years after discontinuing hydralazine, suggesting smoldering inflammatory disease.

scholarly journals Creutzfeldt–Jakob disease-like syndrome induced by gabapentin toxicity

2010 ◽  
Vol 1 (1) ◽  
pp. 3 ◽  
Author(s):  
Vicky Chau ◽  
Sadhana Prasad ◽  
Dwight Stewart ◽  
George Heckman
Keyword(s):  
Cognitive Impairment ◽  
Drug Toxicity ◽  
Clinical Manifestations ◽  
Drug Induced ◽  
Eeg Abnormalities ◽  
Negative Myoclonus ◽  
History Of ◽  
Jakob Disease ◽  
Neurological Effects ◽  
Gait Abnormalities

Patients with Creutzfeldt–Jakob disease (CJD) may exhibit characteristic abnormalities on the electroencephalogram (EEG). However, these abnormalities have been associated with a number of cases of drug toxicity. We report a case of CJD-like syndrome associated with gabapentin. A 78-year-old man was hospitalized for recurrent falls. Three months prior to admission, gabapentin was prescribed to treat symptoms of trigeminal neuralgia. The patient subsequently presented with a two-month history of worsening gait abnormalities, negative myoclonus, and cognitive impairment. The EEG showed diffuse background slowing with larger amplitude delta discharges, which at times appeared triphasic, raising the possibility of CJD. The gait abnormalities and myoclonus resolved and the EEG normalized after the gabapentin was discontinued. Several cases of drug-induced CJD-like syndrome have been reported, mainly presenting with cognitive impairment, myoclonus, Parkinsonism, and EEG abnormalities. This patient may have been predisposed to adverse neurological effects from gabapentin owing to age, concurrent renal insufficiency, and cardiac disease. We concluded that it is imperative to include drug toxicity in the differential diagnosis of patients presenting with clinical manifestations and EEG findings suggestive of CJD, particularly in the setting of advanced age and comorbidities.

scholarly journals First-line ibrutinib for Bing-Neel syndrome

2018 ◽  
pp. bcr-2018-226102 ◽  
Author(s):  
Aaron Tallant ◽  
Daniel Selig ◽  
Sam O Wanko ◽  
Joseph Roswarski
Keyword(s):  
Kinase Inhibitor ◽  
Spinal Column ◽  
Lower Extremity Weakness ◽  
Cerebrospinal Fluid Flow ◽  
Leptomeningeal Enhancement ◽  
Unsteady Gait ◽  
History Of ◽  
Spinal Mri ◽  
Gait Abnormalities

The authors present a case of an elderly man with a history of Waldenstrom macroglobulinaemia in remission who presented with progressively worsening gait abnormalities and falls for several months. His examination was notable for bilateral lower extremity weakness and an unsteady gait. Brain and spinal MRI showed focal leptomeningeal enhancement in the brain and spinal column. Lumbar puncture was performed and cerebrospinal fluid flow cytometry demonstrated a monoclonal CD5/CD10-negative, CD20-positive B-cell lymphocyte population consistent with a diagnosis of Bing-Neel syndrome. He was started on ibrutinib, an oral Bruton’s tyrosine kinase inhibitor, and had marked improvement in his weakness and gait. Repeat imaging 2 months after starting ibrutinib showed improvement in his leptomeningeal enhancement. During subsequent follow-up, he continued to tolerate ibrutinib and had a sustained clinical response.

scholarly journals Essential Tremor

2015 ◽  
Vol 22 (2) ◽  
pp. 108-118 ◽  
Author(s):  
Elan D. Louis
Keyword(s):  
Essential Tremor ◽  
Dendritic Spines ◽  
Morphological Changes ◽  
Neurological Diseases ◽  
The United States ◽  
The Past ◽  
Degenerative Disorders ◽  
Postmortem Studies ◽  
New Formulation ◽  
Cell Interface

Essential tremor (ET) is one of the most common neurological diseases, with an estimated 7 million affected individuals in the United States. Postmortem studies in the past few years have resulted in new knowledge as well as a new formulation of disease pathophysiology. This new formulation centers on the notion that ET might be a disease of the cerebellum and, more specifically, the Purkinje cell (PC) population. Indeed, several investigators have proposed that ET may be a “Purkinjopathy.” Supporting this formulation are data from controlled postmortem studies demonstrating (1) a range of morphological changes in the PC axon, (2) abnormalities in the position and orientation of PC bodies, (3) reduction in the number of PCs in some studies, (4) morphological changes in and pruning of the PC dendritic arbor with loss of dendritic spines, and (5) alterations in both the PC-basket cell interface and the PC-climbing fiber interface in ET cases. This new formulation has engendered some controversy and raised additional questions. Whether the constellation of changes observed in ET differs from that seen in other degenerative disorders of the cerebellum remains to be determined, although initial studies suggest the likely presence of a distinct profile of changes in ET.

Defining Parkinsonism in the Canadian Study of Health and Aging

2001 ◽  
Vol 13 (S1) ◽  
pp. 107-113 ◽  
Author(s):  
Susan L. Mitchell ◽  
Kenneth Rockwood
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Side Effects ◽  
Operational Definition ◽  
Extrapyramidal Side Effects ◽  
Canadian Study ◽  
Resting Tremor ◽  
History Of ◽  
Gait Abnormalities ◽  
Definition Of

This study sought an operational definition of parkinsonism in elderly people (n = 2,914) who underwent a clinical examination in the Canadian Study of Health and Aging (CSHA). Parkinsonism was defined as having two of the following features: (1) bradykinesia of face or limbs, (2) resting tremor, (3) rigidity, and (4) abnormality of gait and posture. The association of parkinsonism with other parkinsonian-related features (prior diagnosis of Parkinson's disease, use of drugs with extrapyramidal side effects, and use of antiparkinsonian medications) and variables not expected to be related to parkinsonism (stroke and Hachinski score > 5) was determined. Parkinsonism was identified in 337 people (11.6%). It was significantly more likely with other parkinsonian-related characteristics, and was not associated with a history of stroke, but was slightly higher among those subjects with a Hachinski score > 5. Posture and gait abnormalities were significantly associated with other parkinsonian-related variables, but were also more common among subjects with stroke-related features. When the gait and posture disturbance category was excluded as a parkinsonian sign, the narrower definition was more specific but less sensitive in detecting cases with a clinical diagnosis of Parkinson's disease. Despite limitations, the approach presented in this article is a valid method to operationalize parkinsonism from the dataset.

PARTING (WITH) THE DEAD: BODY PARTIBILITY AS EVIDENCE OF COMMONER ANCESTOR VENERATION

2012 ◽  
Vol 23 (1) ◽  
pp. 115-130 ◽  
Author(s):  
Pamela L. Geller
Keyword(s):  
Social Class ◽  
Life Histories ◽  
Postmortem Changes ◽  
Body Parts ◽  
Ancestor Veneration ◽  
Social Settings ◽  
Three Rivers Region ◽  
The Reformation ◽  
History Of ◽  
Three Rivers

AbstractAs a complement to life histories authored by many researchers of Maya bones, this study narrates death histories. The latter entails detection of perimortem and postmortem changes to decedents' bodies, followed by translation of these changes' encoded meanings. Biographical analysis of body parts and the buildings in which they are situated facilitates such an endeavor. Past investigations of partibility have focused on protracted processing of noble and royal bodies as a means to reconstitute decedents' identities. Commoners' burials, however, have received far less attention. Consequently, it is difficult to determine if partible practices differ according to or transcend social class. To address this lacuna, a multiscalar frame is applied to a burial sample comprised of decedents from varied social settings in the Three Rivers region, northwestern Belize. Identification of widely shared practices related to the becoming and venerating of ancestors offers a springboard for examining particulars within patterns. Scaling down, commoner burials unearthed at the minor center RB-11 are summarized and special attention is paid to the death history of Individual 71. This decedent's intentionally fragmented body reflects general thinking about ancestors as partible and dividual persons. Yet, certain attributes of Individual 71's burial are unique to the sample as a whole, which demonstrates how social class, circumstance, and individual life history are also instrumental in the reformation of ancestorhood.

scholarly journals Mantle Cell Lymphoma of the Gastrointestinal Tract (Lymphomatous Polyposis)

1996 ◽  
Vol 10 (3) ◽  
pp. 144-148
Author(s):  
Hugh James Freeman
Keyword(s):  
Gastrointestinal Tract ◽  
Mantle Cell Lymphoma ◽  
Cell Lymphoma ◽  
Mantle Cell ◽  
History Of ◽  
Postmortem Studies ◽  
Gastrointestinal Lymphomas ◽  
Aggressive Clinical Course ◽  
Widespread Dissemination ◽  
Entire Gastrointestinal Tract

A74-year-old male with a history of a tonsillar lymphoma developed diarrhea. Investigations led to detection of extensive intestinal lymphomatous polyposis (mantle cell lymphoma). After an aggressive clinical course with associated nodal and peripheral blood involvement, death followed within three months. Postmortem studies revealed widespread dissemination within the entire gastrointestinal tract, including the esophagus, stomach, and small and large intestines. Although this type of lymphoma is rare and accounts for only about 1% to 8% of all forms of primary B cell gastrointestinal lymphomas in North America, separation from other subtypes has become more important because of reported responses of mucosa-associated lymphoid tissue-lymphomas to antibiotics aimed atHelicobacter pylorieradication.

scholarly journals A Case of Psychosis Due to Acute Hypocalcemia From Hypoparathyroidism

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A172-A173
Author(s):  
Joseph Theressa Nehu Parimi ◽  
John Chen Liu ◽  
Rajani Gundluru ◽  
Sowjanya Naha ◽  
Timur Gusov ◽  
...  
Keyword(s):  
Psychiatric Symptoms ◽  
Case Reports ◽  
Severe Depression ◽  
Altered Mental Status ◽  
Auditory Hallucinations ◽  
Psychotic Symptoms ◽  
Organic Psychosis ◽  
Neuropsychiatric Manifestations ◽  
History Of ◽  
Gait Abnormalities

Abstract A 61-year-old female with past medical history of depression, hypoparathyroidism (hypoPtH), and hypothyroidism had disappeared from her home and was found wandering a few hours away with persecutory delusions, visual and auditory hallucinations. Serum calcium (Ca) was 6.3 mg/dL (range 8.6–10.2), albumin 3.7 g/dL (range 3.5–5.2) and ionized Ca 0.89 mmol/L (range 1.12–1.30). She was admitted and treated with Ca and calcitriol. Work-up for altered mental status was negative except for hypocalcemia (hypoCa) and scattered bilateral basal ganglia calcifications (BGC) with cortical and subcortical frontal lobe calcifications on CT. Psychiatry diagnosed delirium due to hypoCa. Acute psychosis resolved once Ca levels improved. Diagnosis of idiopathic hypoPtH was in 1997. Her regimen included Ca citrate 1500mg daily and 10 mcg of Forteo twice daily. She had skipped her medications for at least 2 days prior to presentation. Her medical records revealed that she was seen for severe depression, progressive gait abnormalities, slowed movements, and imbalance, in 2015. CT scan and MRI brain showed BGC. Her son gave a history of multiple admissions for psychosis, violence, delusions with agitation, and wandering at times when the patient was hypoCa, which was diagnosed as schizophrenia. Neuropsychiatric disturbances are commonly associated with hypercalcemia. Review of literature found a few case reports of psychosis and hypoPtH 1,2 BGC is common in hypoPtH. Psychotic symptoms due to BGC include auditory hallucinations, delusions of influence, paranoid states, and complex perceptual distortions.3,5 HypoCa is associated with cognitive impairment. Neurological manifestations tend to improve with Ca correction, but psychiatric symptoms do not improve substantially.4,5 Further studies are needed in hypoPtH with BGC to appropriately diagnose organic psychosis. This is important in management of the vicious cycle of psychiatric illness leading to noncompliance resulting in psychosis. Prevention of BGC will play a key role. References: 1. Finan M, Axelband J. This is your brain on calcium: psychosis as the presentation of isolated hypoparathyroidism. Am J Emerg Med. 2014;32:945.e1-4. 2. Ang AW, Ko SM, Tan CH. Calcium, magnesium, and psychotic symptoms in a girl with idiopathic hypoparathyroidism. Psychosom Med. 1995;57:299–302. 3. Burns K, Brodaty H. Fahr’s disease and psychosis. In: Sachdev PS, Keshavan MS, editors. Secondary schizophrenia. Cambridge: Cambridge University; 2010. p. 358–66. 4. Maiti A, Chatterjee S. Neuropsychiatric manifestations and their outcomes in chronic hypocalcaemia. J Indian Med Assoc. 2013;111:174–7. 5. Amara A, Novais C, Coelho M, Silva A, Curral R, Brandao I, Torres A. Organic psychosis due to hypoparathyroidism in an older adult: a case report. Braz. J. Psychiatry; 2016; 38(4)

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