scholarly journals Nasu-Hakola Syndrome: An Unusual Cause of Pathological Fractures

2012 ◽  
Vol 2012 ◽  
pp. 1-3
Author(s):  
Jaykar R. Panchmatia ◽  
Natasha Jiwa ◽  
Neil Soneji ◽  
John Paul Murphy
Keyword(s):  
Management Strategy ◽  
Neuropsychiatric Symptoms ◽  
Pathological Fractures ◽  
Symptoms And Signs

Nasu-Hakola syndrome is a hereditary cause of pathological fractures. Uniquely, patients also develop neuropsychiatric symptoms and signs. The disease is ultimately fatal. We propose a management strategy for pathological fractures in sufferers based on the stage of the disease.

Dementia management strategies associated with neuropsychiatric symptoms of elderly people with Alzheimer’s disease

Dementia ◽  
2018 ◽  
Vol 18 (7-8) ◽  
pp. 2747-2759 ◽  
Author(s):  
Lais Lopes Delfino ◽  
Ricardo Shoiti Komatsu ◽  
Caroline Komatsu ◽  
Anita Liberalesso Neri ◽  
Meire Cachioni
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Elderly People ◽  
Management Strategy ◽  
Neuropsychiatric Symptoms ◽  
Demographic Data ◽  
Communication Strategies ◽  
Management Strategies ◽  
The Elderly ◽  
Active Management

This study aims to investigate the association between management and communication strategies and the presence of neuropsychiatric symptoms presented by elderly people with Alzheimer’s disease. One hundred and thirty-four family caregivers answered a questionnaire with socio-demographic data and questions regarding the care context, the Small Communication Strategies Scale, the Dementia Management Strategy Scale, and the Neuropsychiatric Inventory. Caregivers used the criticism management strategy more when the elderly presented hallucination, agitation, depression, anxiety, irritability, nighttime behavior, and appetite abnormalities. The encouragement strategy was more significantly used only in the presence of euphoria/elation. The caregivers who used the most active management strategy were those who cared for the elderly with delirium, hallucination, agitation, depression, anxiety, irritability, and appetite and eating abnormalities. The use of communication strategies did not differ between groups with or without neuropsychiatric symptoms. It is concluded that criticism management and active management strategies are strongly associated with neuropsychiatric symptoms. The results of this study may be useful for planning treatment interventions that aim to modify the use of management strategies used by caregivers.

Republished: Retained needle after cement injection during vertebral augmentation and its management strategy

2016 ◽  
Vol 8 (12) ◽  
pp. e51-e51 ◽  
Author(s):  
Angelika Kosse ◽  
Jonathan Pishoi Nakhla ◽  
Reza Yassari ◽  
Apolonia Elisabeth Abramowicz ◽  
Allan Brook
Keyword(s):  
Multiple Myeloma ◽  
Pain Relief ◽  
Operating Room ◽  
Management Strategy ◽  
Cement Augmentation ◽  
Vertebral Augmentation ◽  
Aged Patient ◽  
Pathological Fractures ◽  
Operating Room Table ◽  
The Right

A middle aged patient with multiple myeloma resulting in numerous pathological fractures underwent an L2, L3, and L5 vertebral cement augmentation for pain relief. After injection, the trocar at L2, the final level, could not be withdrawn despite several attempts of needle rotation, a second needle inserted to distract on, and rocking the needle on the pedicle. After a neurosurgical consultation, the patient was transferred to the operating room for open removal. As the needle protruded approximately 3 inches from the patient's back, the patient could not be positioned supine, and was anesthetized and intubated in the right semi-prone position prior to being placed prone on the operating room table. The needle was surgically exposed, cut off at the pedicular bone edge, and its free component was removed.

scholarly journals Polyostotic Fibrous Dysplasia in a Child with Bone Fractures and Neuropsychiatric Symptoms, Case Report

2019 ◽  
Vol 3 (1) ◽  
pp. 1-5
Author(s):  
Hala A Al Shaikh
Keyword(s):  
Genetic Analysis ◽  
Fibrous Dysplasia ◽  
Clinical Course ◽  
Bone Fractures ◽  
Neuropsychiatric Symptoms ◽  
Polyostotic Fibrous Dysplasia ◽  
Pathological Fractures ◽  
Mccune Albright Syndrome ◽  
Gnas Gene ◽  
Albright Syndrome

Fibrous dysplasia of the bone in children is a rare, genetic, heterogeneous disorder, associated with extra - skeletal manifestations in the McCune Albright Syndrome . It results from a post - zygotic activating mutation in the (GNAS) gene coding for (Gs alfa) protein. This leads to replacement of the lamellar bone with under - mineralized fibrous tissue, presenting clinically with limping, pain, and pathological fractures . Objective: To demonstrate the clinical course, challenges and treatment of a child with polyostotic fibrous dysplasia presenting with limping, extra skeletal manifestations, neuropsychiatric symptoms and bone fractures. Method : The clinical information and lab results were obtained directly from the mother and the computerized medical records at the treating hospitals. Genetic analysis was done at Washington University School of Medicine, USA. Informed publication consent was obtained from the parents. R esults : The child presented at the age of 3years with limping and was found to have polyostotic fibrous dysplasia and café au lait spot. Later on, he developed neurological symptoms. He had bone fractures in the proximal femur, requiring surgical intervent ion. Bone pain and frequency of fractures improved after Zoledronate injections. Blood genetic analysis of the GNAS - gene was negative. Conclusion : Polyostotic fibrous dysplasia is a rare cause of limping and recurrent pathological fractures in children. Me dical treatment with Biphosphanate injections can positively affect the clinical course of the disease. A negative blood genetic analysis of the GNAS gene does not exclude the McCune Albright Syndrome, and indicates analysis from the affected tissue. Neuro psychiatric symptoms may manifest with fibrous dysplasia without any endocrine involvement.

scholarly journals Amyloidosis presenting as multiple vertebral fractures

2014 ◽  
Vol 33 (03) ◽  
pp. 240-243 ◽  
Author(s):  
Ricardo Vieira Botelho ◽  
Matheus Fernandes de Oliveira ◽  
José Marcus Rotta
Keyword(s):  
Bone Tumour ◽  
Percutaneous Biopsy ◽  
Bone Lesions ◽  
Pathological Fractures ◽  
History Of ◽  
Plasma Cell Disorder ◽  
Rheumatic Conditions ◽  
Multiple Vertebral Fractures ◽  
Symptoms And Signs ◽  
Cell Disorder

AbstractAmyloidosis is a plasma cell disorder characterized by the overproduction and tissue deposition of a monoclonal IG light chain or fragments. Musculoskeletal and soft tissue manifestations are arthropathy, myopathy, bone lesions, and lymphadenopathy. It can also present with symptoms and signs that mimic a variety of rheumatic conditions, such as scleroderma, rheumatoid arthritis, Sjögren's syndrome polymyalgia rheumatica, a myeloma or a bone tumour. We describe the case of a 64-year-old white woman with an acute history of fall from own height in whose investigation revealed fractures in T8, T9, T11 and L1. In order to determine the nature of lesion, the patient was submitted to percutaneous biopsy guided by fluoroscopy of T11, without any surgical complications. Pathological findings were compatible with deposition of amyloid protein and amyloidosis. Amyloidosis must be considered in differential diagnosis of pathological fractures together with other osteopenic and lytic conditions, such as bone metastasis, metabolic and infectious diseases.

Questions and Answers

2000 ◽  
Vol 5 (2) ◽  
pp. 3-3
Author(s):  
Christopher R. Brigham ◽  
James B. Talmage
Keyword(s):  
Nervous System ◽  
Peripheral Nervous System ◽  
Spinal Nerve ◽  
Sensory Loss ◽  
Residual Symptoms ◽  
Additional Information ◽  
Questions And Answers ◽  
Motor Nerves ◽  
Symptoms And Signs ◽  
Limited Motion

Abstract Lesions of the peripheral nervous system (PNS), whether due to injury or illness, commonly result in residual symptoms and signs and, hence, permanent impairment. The AMA Guides to the Evaluation of Permanent Impairment (AMA Guides) describes procedures for rating upper extremity neural deficits in Chapter 3, The Musculoskeletal System, section 3.1k; Chapter 4, The Nervous System, section 4.4 provides additional information and an example. The AMA Guides also divides PNS deficits into sensory and motor and includes pain within the former. The impairment estimates take into account typical manifestations such as limited motion, atrophy, and reflex, trophic, and vasomotor deficits. Lesions of the peripheral nervous system may result in diminished sensation (anesthesia or hypesthesia), abnormal sensation (dysesthesia or paresthesia), or increased sensation (hyperesthesia). Lesions of motor nerves can result in weakness or paralysis of the muscles innervated. Spinal nerve deficits are identified by sensory loss or pain in the dermatome or weakness in the myotome supplied. The steps in estimating brachial plexus impairment are similar to those for spinal and peripheral nerves. Evaluators should take care not to rate the same impairment twice, eg, rating weakness resulting from a peripheral nerve injury and the joss of joint motion due to that weakness.

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