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Cerebellar Atrophy


Presentation

  • Late cortical cerebellar atrophy (LCCA) is a neurodegenerative disease which presents with slowly progressive cerebellar ataxia as a prominent symptom and is characterized neuropathologically by a limited main lesion to the cerebellar cortex and inferior[ncbi.nlm.nih.gov]
Weight Loss
  • We report a case of cerebral atrophy with marked cerebellar atrophy in a 20-year-old woman with anorexia nervosa admitted to our hospital for severe weight loss.[ncbi.nlm.nih.gov]
Cyanosis
  • This very rare blood disorder results in mild cyanosis, profound mental and motor impairment, and movement disorders in infancy and childhood.[ncbi.nlm.nih.gov]
Normal Hearing
  • The clinical features common to the 16 males included profound mental retardation (100%), mutism despite apparently normal hearing (100%), grand mal epilepsy (87.5%), and limited life expectancy (68.8%).[ncbi.nlm.nih.gov]
Cognitive Disorder
  • Author information 1 Clinical Genetics Department, Human Genetics and Genome Research Division, National Research Center, Cairo, 12311, Egypt. 2 INSERM UMR 1163, Laboratory of Molecular and Pathophysiological Bases of Cognitive Disorders, Paris, France[ncbi.nlm.nih.gov]
Psychomotor Regression
  • Infantile neuroaxonal dystrophy (INAD) is a rare neurodegenerative disorder characterized by infantile onset and rapid progression of psychomotor regression and hypotonia evolving into spasticity.[ncbi.nlm.nih.gov]
Postural Instability
  • RESULTS: Twenty-seven patients showed larger postural instability in all experimental conditions. Postural sway increased with nystagmus in the eyes closed condition but not with the eyes open.[ncbi.nlm.nih.gov]

Workup

MRI Scan showing Cerebellar Atrophy
  • Brain MRI scan showed cerebellar atrophy. This case demonstrates a rare association of distal myopathy and cerebellar atrophy.[ncbi.nlm.nih.gov]

Treatment

  • CONCLUSION: The transplantation of in vitro cultured neural stem cell is a feasible and effective treatment for inherited CA, but the long term effectiveness need to be taken in consideration.[ncbi.nlm.nih.gov]
  • Treatment Treatment Options: No treatment has been reported.[disorders.eyes.arizona.edu]

Prognosis

  • Cerebellar Atrophy Prognosis Atrophy of any tissue means loss of cells. In brain tissue, atrophy describes a loss of neurons and the connections between them.[topclassactions.com]
  • Please tell me the prognosis of this disease. anon293481 Post 4 My husband is a traumatic brain injury patient. His injury was brought about by a vehicular accident two years ago. He is only 55 years old.[wisegeek.com]
  • Prognosis The long-term outlook for people with cerebellar degeneration varies depending on its underlying cause. x Prognosis The long-term outlook for people with cerebellar degeneration varies depending on its underlying cause.[ninds.nih.gov]

Etiology

  • Etiology The exact etiology of MSC-c is unknown while the presence of cytoplasmic aggregates of α-synuclein, primarily in the oligodendroglia, in combination with predominant neurodegeneration of the olivopontocerebellar structures are pathological hallmark[orpha.net]
  • Etiology Diffuse atrophy can result from a variety of causes: normal aging alcoholic cerebellar degeneration drugs, e.g. phenytoin cerebellar degeneration chronic temporal lobe epilepsy (independently from phenytoin use) 7 cerebellitis multisystem atrophy[radiopaedia.org]
  • Except for JCV infection, no other etiology was found to explain cerebellar disorders.[jcm.asm.org]
  • Mutations of the aminoacyl-tRNA-synthetases SARS and WARS2 are implicated in the etiology of autosomal recessive intellectual disability. Hum Mutat. 2017 Jun;38(6):621-636.[disorders.eyes.arizona.edu]

Epidemiology

  • Keywords Adult Amnesia/diagnosis/epidemiology Atrophy Brain/anatomy & histology Cerebellar Diseases/epidemiology/pathology Cerebellum/pathology Epilepsy, Temporal Lobe diagnosis/pathology Female Functional Laterality Humans Incidence Magnetic Resonance[analyzedirect.com]
  • Summary Epidemiology MSA-c is observed predominantly in patients from Asia.[orpha.net]
  • […] features Characterized clinically by ataxia and gait disturbances in the setting of chronic alcoholism Pathologic features include cerebellar atrophy affecting the anterior / superior vermis with a loss of Purkinje cells and corresponding Bergmann gliosis Epidemiology[pathologyoutlines.com]
  • "Gluten ataxia in perspective: epidemiology, genetic susceptibility and clinical characteristics." Brain 126(Pt 3): 685-91. Pittock SJ and others. Paraneoplastic antibodies coexist and predict cancer, not neurological syndrome.[dizziness-and-balance.com]
Sex distribution
Age distribution

Pathophysiology

  • Author information 1 Clinical Genetics Department, Human Genetics and Genome Research Division, National Research Center, Cairo, 12311, Egypt. 2 INSERM UMR 1163, Laboratory of Molecular and Pathophysiological Bases of Cognitive Disorders, Paris, France[ncbi.nlm.nih.gov]
  • […] features include cerebellar atrophy affecting the anterior / superior vermis with a loss of Purkinje cells and corresponding Bergmann gliosis Epidemiology Occurs in approximately 10% of alcoholic patients Sites Anterior / superior cerebellar vermis Pathophysiology[pathologyoutlines.com]
  • 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]
  • […] and body sway during the 2-week titration period as well as the 8-week reassessment. [31] Insulin-Like Growth Factor Insulin-like growth factor-1 (IGF-1) acts as a neuromodulator in the CNS. [32] Disturbances in CNS signaling pathways may produce the pathophysiological[movementdisorders.org]

Prevention

  • Early diagnosis of NBD helps to initiate appropriate treatment, thereby modulating the course of the disease and preventing complications.[ncbi.nlm.nih.gov]
  • The aim is to treat the symptoms and prevent complications.[medlineplus.gov]
  • It helps prevent seizures that affect the entire brain (tonic-clonic seizures) as well as seizures that affect only one half or part of the brain (partial seizures). It is often used to prevent seizures during and after brain surgeries.[topclassactions.com]
  • In an animal study, gabapentin was shown to have long-lasting effects that might prevent ataxia symptoms from worsening over time 3 . Gabapentin is considered highly safe and most patients experience few or no adverse effects 4 .[multiplesystematrophy.org]

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