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Cognitive Impairment with or without Cerebellar Ataxia


Presentation

  • 71, ALLELE2 23) Signs suggestive of PD diagnosis Asymmetric onset present absent Bradykinesis present absent Activation tremor present absent Resting Tremor present absent Postural Instability present absent Rigidity present absent Gait difficulties[catalog.coriell.org]
  • Occasionally patients will present with late-onset forms of this disease.[neuroweb.us]
  • Patients typically present with a wide-based gait, truncal instability, and dysarthria.[healio.com]
  • Autonomic dysfunction, vasomotor symptoms, and REM sleep disorder may also be present.[amjmed.org]
  • We investigated whether or not gluten sensitivity is involved in Japanese patients with idiopathic cerebellar ataxia with extracerebellar presentation.[jstage.jst.go.jp]
Disability
  • Affiliated tissues include brain , and related phenotypes are emotional lability and intellectual disability[malacards.org]
  • Cognitive disability aesthetics : visual culture, disability representations, and the (in)visibility of cognitive difference Fraser, Benjamin. Toronto ; Buffalo : University of Toronto Press, [2018] NLM ID: 101736291 [Book] Select item 101714310 19.[ncbi.nlm.nih.gov]
  • 1075 African Americans Epidemiology Prevention and Treatment Issues 1093 Hispanic Americans 1103 Asian Americans and Pacific Islanders 1113 Alcohol Use Among American Indians and Alaskan Natives 1119 Gays Lesbians and Bisexuals 1129 The Homeless 1141 Disability[books.google.com]
  • In the same work, authors demonstrated that fluency deficits appear to be correlated with greater neurological disability, disease duration and age [ 36 ].[omicsonline.org]
  • However, PAF is considered a milder, less disabling condition than MSA12. PAF can also be distinguished from MSA in that it will not be accompanied by Parkinsonian or cerebellar symptoms.[multiplesystematrophy.org]
Fatigue
  • Efficacy and safety of modafinil (Provigil) for the treatment of fatigue in multiple sclerosis: a two centre phase 2 study. J Neurol Neurosurg Psychiatry 2002:72:l79-l83. ‏[books.google.com]
  • Increased fatigue - Patients with ataxia due to cerebellar atrophy often experience unexpected fatigue when performing normal activities.[hopkinsmedicine.org]
  • Mildly elevated serum ALT and fatigue, as in our patient, are common initial symptoms of the transition to clinical stage disease.[tidsskriftet.no]
  • The clinical assessment also included the Fatigue Severity scale (FSS) [ 26 ].[omicsonline.org]
Physician
  • Prevention and Treatment Issues 1093 Hispanic Americans 1103 Asian Americans and Pacific Islanders 1113 Alcohol Use Among American Indians and Alaskan Natives 1119 Gays Lesbians and Bisexuals 1129 The Homeless 1141 Disability and Rehabilitation Issues 1169 Physicians[books.google.com]
  • ., any condition can be approved for medical marijuana as long as a DC-licensed physician recommends the treatment.[medicalmarijuanainc.com]
  • 27th, 2013 at 11:00 am and is filed under Physicians-In-Training .[blogs.nejm.org]
  • Patients with ataxia should remain in close contact with a primary physician, neurologist, psychiatrist or psychologist, and with physical, speech and swallowing, and occupational therapists so that new difficulties can be recognized and treated early[hopkinsmedicine.org]
Pain
  • Medical Complications of Drug Use 840 Acute and Chronic Pain 863 Substance Use Disorders in Individuals with CoOccurring Psychiatric Disorders 904 Epidemiology and Emerging Public Health Perspectives 913 HIVRelated Medical Complications and Treatment[books.google.com]
  • Efficacy of Inhaled Cannabis on Painful Diabetic Neuropathy. Journal of Pain, 16(7), 616-27. Retrieved from . Woodhams, S.G., Sagar, D.R., Burston, J.J., and Chapman, V. (2015). The role of the endocannabinoid system in pain.[medicalmarijuanainc.com]
  • With the decrease in muscle spasms and general muscle pain, patients are able to get through their required physical therapy without experiencing painful side effects.[mmjrecs.com]
  • What gait disturbance is seen with pain? 15-2. Patients limp in order to protect an injured area. 15-3.[dartmouth.edu]
  • Fever, fullness and pain in the ear, and mastoiditis may serve as clues to the diagnosis.[healio.com]
Cerebral Palsy
  • Children and youth with complex cerebral palsy : care and management Glader, Laurie J; Stevenson, Richard D. London : Mac Keith Press, 2019. NLM ID: 101746343 [Book] Select item 101741450 6.[ncbi.nlm.nih.gov]
  • Cerebellum - cross-sectional view Cerebral palsy Cerebral palsy is a condition that affects movement, and is a result of brain injury either before, during, or shortly after birth. If the injury damages the cerebellum, cerebral ataxia can occur.[kenhub.com]
  • Cerebral palsy. There are various other uncommon neurological or metabolic diseases which may involve the cerebellum. Idiopathic cerebellar ataxia - a diagnosis of exclusion.[patient.info]
  • The changing panorama of cerebral palsy in Sweden. IX. Prevalence and origin in the birth-year period 1995–1998. Acta Paediatr 2005 ; 94 : 287 –94 Berciano J, Boesch S, Perez-Ramos JM, et al.[ajnr.org]
  • These include cerebral palsy (often associated with brain injury at birth due to lack of oxygen or blood supply), hydrocephalus (increased pressure caused by blockage or buildup of cerebrospinal fluid), brain tumors, and other injuries to the brain.[bcm.edu]
Hearing Impairment
  • The auditory brain and age-related hearing impairment Eggermont, Jos J. London, United Kingdom ; San Diego, CA : Elsevier/Academic Press, [2019] NLM ID: 101745395 [Book] Select item 101746343 5.[ncbi.nlm.nih.gov]
Emotional Lability
  • Affiliated tissues include brain , and related phenotypes are emotional lability and intellectual disability[malacards.org]
Nystagmus
  • Features include bilateral esophoria, strabismatic amblyopia, unsustained gaze evoked nystagmus on horizontal gaze, ataxic gait, dysmetria in the upper limbs and dysarthria, with normal strength, tone, and reflexes.[malacards.org]
  • Inherited periodic ataxia, dysarthria, nystagmus and vertigo.[patient.info]
  • Vertical Nystagmus may occur.[brainaacn.org]
  • Presence of nystagmus should be noted with relevant details. Nystagmus may arise from pathology in the vestibular system (“peripheral” type) or the central nervous system (“central”).[healio.com]
  • Patients have ataxia along with spasticity, speech problems, nystagmus, weakness of lower extremities, and sensory problems.[bcm.edu]
Dysmetria
  • Features include bilateral esophoria, strabismatic amblyopia, unsustained gaze evoked nystagmus on horizontal gaze, ataxic gait, dysmetria in the upper limbs and dysarthria, with normal strength, tone, and reflexes.[malacards.org]
  • The cerebellar cognitive affective syndrome: Clinical correlations of the dysmetria of thought hypothesis. International Review of Psychiatry, 13, 313-322. a b c d e f g h Schmahmann, J. D. (2010).[en.wikipedia.org]
  • The clinical findings of cerebellar dysfunction (dysarthria, bilateral dysmetria, dysdiadochokinesia, intention tremor, ataxic gait) improved markedly.[em-consulte.com]
  • […] the anterior medullary velum] Additional Cerebellar Disorders Dysdiadochokinesia (aka adiadochokinesia) Abnormalities of rapid alternating movements, such as tapping one hand with the palm and dorsum of the other hand Eye Movement Abnormalities Ocular Dysmetria[brainaacn.org]
  • It seems likely that the predictive and preparative function of the cerebellum can lead to two types of dysmetria; one in motor functions and one in cognitive functions.[jnnp.bmj.com]
Cognitive Deficit
  • Conclusion: Patients with NPCA have significant cognitive deficits with an asymmetric profile and better verbal than nonverbal performance.[neurology.org]
  • Up to one third of affected children have symptoms and signs that resolve slowly over a period of many months, and some have permanent clinical syndromes that affect motor control and behavioral, social, and cognitive deficits.[blogs.nejm.org]
  • The presence of the MCP sign in patients with FXTAS is correlated with more severe cognitive deficits and a longer history of symptoms 2 . Currently, there are no disease-modifying treatments for FXTAS.[radiopaedia.org]
  • Subtle cognitive deficits after cerebellar infarcts. European Journal of Neurology, 13, 161-170. a b Yildiz, O., Kabatas, S., Yilmaz, C., Altinors, N., & Agaoglu, B. (2010).[en.wikipedia.org]
  • Another explanation for the cognitive deficits in cerebellar disease is based on a disruption in cerebrocerebellar connections.[e-jmd.org]
Gait Ataxia
  • Ask the patient to walk heel-to-toe (to elicit any gait ataxia). If there are unilateral signs - check V, VII, and VIII (cerebellopontine angle pathology).[patient.info]
  • ataxia minor parkinsonism moderate to severe short-term memory deficiency executive function deficit neuropathy Radiological features major MRI white matter lesions in middle cerebellar peduncle MRI white matter lesions in splenium of the corpus callosum[radiopaedia.org]
  • These patients present with gait ataxia usually with headache and papilledema. Progressive hydrocephalus can also present with subacute-onset ataxia.[jpgmonline.com]
  • Friedreich's ataxia (FRDA) , the most frequent inherited ataxia, is an autosomal recessive disease which begins usually before age 20 with gait ataxia, proprioceptive and superficial sensory loss, weakness and atrophy of the extremities, and spasticity[neuropathology-web.org]
  • The common clinical problems in SCA are gait ataxia, eye movement abnormalities (nystagmus or jerkiness and double vision), and dysarthria (speech difficulty).[bcm.edu]
Mild Cognitive Impairment
  • Neurodegenerative Diseases and Mild Cognitive Impairment. Johns Hopkins Diabetes Guide. 2018. . Accessed May 13, 2019. Pletnikova, A., Marano, C., & Peters, M. E. (2018). Neurodegenerative Diseases and Mild Cognitive Impairment.[hopkinsguides.com]
  • The patients had variable cerebellar ataxia and mild cognitive impairment without quadrupedal gait. The brain MRI showed variable cerebellar volume loss and ill-defined peritrigonal white matter abnormalities.[kclpure.kcl.ac.uk]
  • MCI: mild cognitive impairment.[e-jmd.org]
  • Affected subjects exhibit a moderate gait and limb ataxia variably associated with akinesia, tremor, rigidity, hyporeflexia, and mild cognitive impairment.[springerlink.com]
  • In order to assess the value of neurophysiology as an AD biomarker, wholehead magnetoencephalographic (MEG) resting state recordings were obtained from 35 AD patients, 23 mild cognitive impairment (MCI) patients, and 24 healthy controls.[practicalneurology.com]
Sexual Dysfunction
  • Cognitive behavioral therapy for sexual dysfunction Metz, Michael E; Epstein, Norman, 1947-; McCarthy, Barry W, 1943-. New York : Routledge, 2018. NLM ID: 101706115 [Book] Select item 101736291 18.[ncbi.nlm.nih.gov]

Workup

Brain Edema
  • History of often postprandial headaches, recurrent episodes of ataxia, vomiting associated with psychiatric symptoms, seizures and unexplained somnolence or coma with brain edema. The alterations in mental state may last up to days.[neuroweb.us]

Treatment

  • Medical Complications of Drug Use 840 Acute and Chronic Pain 863 Substance Use Disorders in Individuals with CoOccurring Psychiatric Disorders 904 Epidemiology and Emerging Public Health Perspectives 913 HIVRelated Medical Complications and Treatment[books.google.com]
  • Internet gaming disorder : theory, assessment, treatment, and prevention King, Daniel L; Delfabbro, Paul H (Paul Howard). San Diego, California : Academic Press, [2019] NLM ID: 101735124 [Book] Select item 101739947 8.[ncbi.nlm.nih.gov]
  • ., any condition can be approved for medical marijuana as long as a DC-licensed physician recommends the treatment.[medicalmarijuanainc.com]
  • The symptoms are similar to those of other conditions, making it difficult to diagnose and get ahead on the preventative treatment.[mmjrecs.com]

Prognosis

  • […] premutation carriers with FXTAS axial and radial diffusivity values in the middle cerebellar peduncle and cerebral peduncle have been shown to be increased in asymptomatic premutation carriers, without alteration in fractional anisotropy 5 Treatment and prognosis[radiopaedia.org]
  • Prognosis Spinocerebellar ataxias due to repeat expansion mutations usually become sick in middle age. In addition to ataxia, other neurological findings are often present depending on the variant of SCA.[verywell.com]
  • Management and prognosis This depends upon the underlying cause.[patient.info]
  • The prognosis is usually bad with survival rates less than a few years after onset.[neuroweb.us]

Etiology

  • Code First Code First Help Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10data.com]
  • The remainder of the review discusses etiological considerations that vary depending on the age group under consideration.[healio.com]
  • Initially, we found no family history, no exposure history, and nothing else suggestive of an alternative etiology.[amjmed.org]
  • Cause [ edit ] The causes of CCAS lead to variations in symptoms, but a common core of symptoms can be seen regardless of etiology.[en.wikipedia.org]

Epidemiology

  • Medical Complications of Drug Use 840 Acute and Chronic Pain 863 Substance Use Disorders in Individuals with CoOccurring Psychiatric Disorders 904 Epidemiology and Emerging Public Health Perspectives 913 HIVRelated Medical Complications and Treatment[books.google.com]
  • Gluten ataxia in perspective: epidemiology, genetic susceptibility and clinical characteristics. Brain. 2003;126:685-91. Hou J-G, Jankovic J. Movement disorders in Friedreich's ataxia. J Neurol Sci. 2003;206:59-64. Jankovic J, Tolosa E, eds.[bcm.edu]
  • Molecular epidemiology and clinical spectrum of hereditary spastic paraplegia in the Japanese population based on comprehensive mutational analyses. J Hum Genet 2014; 59 : 163–172.[nature.com]
  • A population-based epidemiological study in Japan showed no reports of SCA10 cases [ 12 ].[journals.plos.org]
Sex distribution
Age distribution

Pathophysiology

  • The neuropathology, pathophysiology and genetics of multiple system atrophy. Neuropathol Appl Neurobiol. 2012;38:4-24. PubMed Koeppen AH, Mazurkievicz JE. Friedreich Ataxia: Neuropathology Revised. J Neuropathol Exp Neurol 2013;72(2):78-90.[neuropathology-web.org]
  • Instead they may be both long lasting and potentially damaging. 12 Stress, sleep disruption, exercise and inflammation have all been linked with regulating hippocampal neurogenesis and implicated in the pathophysiology of mood disorders. 13 But can gluten[thepaleodiet.com]
  • Most cases follow severe injuries; 20 however, a few children have been noted to have cerebellar concussion following relatively minor head trauma. 21 The pathophysiology is believed to be damage to the connections between the cerebellum and the cortex[healio.com]
  • In precedent works [ 27 - 29, 31, 32 ], we demonstrated the involvement of the thalamus in the pathophysiological mechanisms of RR-MS as evidenced in other works [ 33 - 35 ].[omicsonline.org]

Prevention

  • […] and Treatment of Addiction Among African Americans 1250 The Public Health Approach to the Prevention of Substance Abuse 1255 Medical Education in Substance Abuse The Acquisition of Knowledge Attitudes and Skills 1269 Education and Training of Clinical[books.google.com]
  • Health promotion and disease prevention in clinical practice Coviello, Jessica Shank. Third edition. Philadelphia : Wolters Kluwer, [2020] NLM ID: 101738883 [Book] Select item 101739755 3.[ncbi.nlm.nih.gov]
  • Other clues to abnormal extrapyramidal function include abnormal movements at rest and slowed eye movements (some conditions prevent voluntary vertical eye movements).[dartmouth.edu]
  • The symptoms are similar to those of other conditions, making it difficult to diagnose and get ahead on the preventative treatment.[mmjrecs.com]
  • A major cannabinoid found in cannabis, tetrahydrocannabinol (THC), has been shown to help in the treatment of Parkinson’s disease by preventing free radical damage and encouraging the formation of new mitochondria (Zeissler, et al., 2013).[medicalmarijuanainc.com]

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