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2.1
Subacute Cerebellar Degeneration
Cerebellar Degeneration Subacute

Presentation

The interest of this case is the unusual presentation and the targeted therapeutic approach that has been used. [ncbi.nlm.nih.gov]

We present this case to highlight the fact that hypothyroidism can present as acute cerebellar ataxia. Case Report A 45-year-old male presented to our hospital with history of unsteadiness of gait since 2 weeks. [jcdr.net]

This case highlights the challenges in the evaluation of a patient who presents with a cerebellar syndrome. [karger.com]

Entire Body System

  • Falling

    She further endorsed dizziness upon standing, dysphagia, falls, and increasing difficulty working her retail job. History included hypothyroidism well controlled with levothyroxine after thyroid ablation for thyroiditis. [amjmed.org]

    The gait was wide-based and there was a tendency to fall to left side. Tandem walking was impaired. His fundus was normal. His power and reflexes were normal. Sensory system was normal. There was no evidence of autonomic dysfunction. [jcdr.net]

    Truncal ataxia Patients can't sit or stand unsupported and tend to fall backwards. It is caused by a midline cerebellar lesion, or may be a feature of post-chickenpox cerebellar syndrome. [patient.info]

    She also noticed difficulty in walking in the form of imbalance and tendency to fall more towards the left. She doesn’t give any history of loss of consciousness, convulsion, headache, visual complaints and bulbar symptoms. [pubs.sciepub.com]

    Other clinical signs include early falling and fine myoclonic jerks of the fingers /limb and can be elicited by tapping the limb. The cerebellar syndrome is usually subtle with gait and limb ataxia, nystagmus and dysarthria. [neuroweb.us]

Neurologic

  • Ataxia

    We report an autopsied patient who initially presented signs of cerebellar ataxia. After seven months, small cell carcinoma of the lung was revealed. [ncbi.nlm.nih.gov]

    ataxia Cerebellar ataxia (loss of muscle coordination) Cerebellar ataxia due to toxin Cerebellar ataxia, early-onset Cerebellar ataxia, late-onset Drug-induced cerebellar ataxia Early onset cerebellar ataxia Early onset cerebellar ataxia with essential [icd9data.com]

    Wildemann: ‘Medusa head ataxia’: the expanding spectrum of Purkinje cell antibodies in autoimmune cerebellar ataxia. [en.wikipedia.org]

    In cerebellar ataxia, the most often used specific scales for the severity of cerebellar ataxia in previous studies were the scale for the assessment and rating of ataxia (SARA) [ 5, 10, 16, 56 – 59 ] and international cooperative ataxia rating scale [intechopen.com]

    The DNER-reacting control serum was from a patient suffering from Hodgkin Lymphoma but not ataxia, and was later confirmed to be anti-Tr positive. [cellbiology.science.uu.nl]

  • Cerebellar Ataxia

    We report an autopsied patient who initially presented signs of cerebellar ataxia. After seven months, small cell carcinoma of the lung was revealed. [ncbi.nlm.nih.gov]

    ataxia Cerebellar ataxia (loss of muscle coordination) Cerebellar ataxia due to toxin Cerebellar ataxia, early-onset Cerebellar ataxia, late-onset Drug-induced cerebellar ataxia Early onset cerebellar ataxia Early onset cerebellar ataxia with essential [icd9data.com]

    […] of physical therapy for cerebellar ataxia. [intechopen.com]

    Cerebellar Ataxia – The First Symptom of HIV Infection [abstract]. Mov Disord. 2017; 32 (suppl 2). http://www.mdsabstracts.org/abstract/cerebellar-ataxia-the-first-symptom-of-hiv-infection/. [mdsabstracts.org]

    This article specifically focuses on acquired causes of cerebellar ataxia. A separate complementary article discusses common hereditary causes of cerebellar ataxia. [els.net]

  • Dysarthria

    Inherited periodic ataxia, dysarthria, nystagmus and vertigo. [patient.info]

    Subsequently, the patient showed moderate improvement in ataxic dysarthria, slow saccades and hemispherical cerebellar signs with marginal improvement in truncal ataxia. [annalsofian.org]

    Laboratory Report Clinical Significance Anti-CV2, anti-Hu, anti-Ma, anti-Ta, anti-Ri, anti-Yo, anti-Zic4 antibodies, and anti-GAD65 are found in patients with acute or subacute symptoms of cerebellar degeneration including gait ataxia, nystagmus and/or dysarthria [questdiagnostics.com]

    After resection, his paralysis and dysarthria were resolved to the extent of being able to walk and speak fluently. Brain MP-RAGE showed no findings suggestive of metastasis or atrophy. [dear-medic.jp]

    Radical surgery did not significantly ameliorate her neurological impairment, although the dysarthria improved slightly. Conclusion. [scinapse.io]

  • Nystagmus

    Inherited periodic ataxia, dysarthria, nystagmus and vertigo. [patient.info]

    We report the case of a 76-year-old woman with breast carcinoma and anti-Ri antibody-mediated PCD along with ataxia, opsoclonus, torsional nystagmus, and eyelid myokymia, who improved after tumor resection and immunotherapy. [neurologyindia.com]

    Her examination revealed a right horizontal beating nystagmus and a normal sensory, motor and gait examination. Review of her MRI Brain revealed mild diffuse cerebellar degeneration but otherwise normal. [neurology.org]

    Slurred speech and vertical nystagmus gradually appeared. [journals.lww.com]

    Range(s) See Laboratory Report Clinical Significance Anti-CV2, anti-Hu, anti-Ma, anti-Ta, anti-Ri, anti-Yo, anti-Zic4 antibodies, and anti-GAD65 are found in patients with acute or subacute symptoms of cerebellar degeneration including gait ataxia, nystagmus [questdiagnostics.com]

  • Tremor

    Tremor Cerebellar lesions can produce unilateral or bilateral intention tremor, or a truncal tremor. Nausea and vomiting Cerebellar lesions can produce nausea and/or vomiting. [patient.info]

    The remaining approximately 2/3 of sporadic ataxia patients develop a form of multiple system atrophy (MSA), characterized by ataxia associated with a combination of dementia, ophthalmoplegia, bulbar findings, tremor, bradykinesia or rigidity, autonomic [ataxia.uchicago.edu]

    […] ataxia Cerebellar ataxia (loss of muscle coordination) Cerebellar ataxia due to toxin Cerebellar ataxia, early-onset Cerebellar ataxia, late-onset Drug-induced cerebellar ataxia Early onset cerebellar ataxia Early onset cerebellar ataxia with essential tremor [icd9data.com]

    Prominent ataxic dysarthria, dysmetria of upper limbs more than lower limb, dysdiadochokinesia and intentional tremors were observed. No cranial neuropathy except for mild sensorineural hearing loss was identified. [annalsofian.org]

    Intention tremor, parkinsonism, and generalized brain atrophy in male carriers of fragile X. Neurology 2001 ; 57 : 127 –30 ↵ Verkerk AJ, Pieretti M, Sutcliffe JS, et al. [ajnr.org]

Workup

Subsequent workup by primary care physician revealed thiamine deficiency and normal TSH, B12 and folate. She started thiamine supplementation. [neurology.org]

The image below illustrates the workup of paraneoplastic cerebellar degeneration. The workup of paraneoplastic cerebellar degeneration. [emedicine.medscape.com]

PCD presents with an acute/subacute severe pancerebellar syndrome and should lead to prompt and extensive diagnostic workup. Early detection and management of the underlying malignancy is imperative to modify the prognosis of the condition. 1. [annalsofian.org]

MR

  • MRI Shows Cerebellar Atrophy

    Brain MRI showed cerebellar atrophy. He was admitted to our hospital for anal bleeding and was diagnosed with colon cancer. An associated diagnosis of PCD was made. [dear-medic.jp]

    Serology HIV test was positive and a brain MRI showed cerebellar atrophy. CSF exam showed hyperproteinorrachia of 51.9 mg. Viral HIV load was 24880 and CD4 count of 65. [mdsabstracts.org]

    Brain MRI may show predominantly cerebellar atrophy and signal changes on T2 in the putamina. [neuroweb.us]

Treatment

A trial using this new type of treatment should be performed in patients who have this therapeutically refractory clinical condition. [ncbi.nlm.nih.gov]

Early treatment (within first three months of symptoms) with immunotherapy may improve long term outlook. There are rare reports of people who have improved without treatment. [rarediseases.info.nih.gov]

Coverage includes major updates on genetics of diseases, new diagnostic techniques, and the newest treatment options. This second volume covers tumors, the phacomatoses, and vascular disease. [books.google.com]

Prognosis

No correlation of the prognosis was seen with the type or stage of Hodgkin's lymphoma. [annalsofian.org]

Prognosis of patients with cancer presenting with PNS is better compared to those without PNS due to detection of cancer at early stage although the prognosis may be bad due to severe neurological morbidity [ 4 ]. [omicsonline.org]

These evaluations may demonstrate that the patient requires rehabilitation following discharge, and, depending upon the prognosis, even long-term placement in a skilled nursing facility. F. Prognosis and Patient Counseling. [clinicaladvisor.com]

The disease has a poor prognosis and often results in severe disability. Treatments have been variable and unpredictable. [ahcmedia.com]

Onset Time: Before or after neoplasm Symptoms: Pain in back or Radiating to legs Weakness: Flaccid paraplegia Sensory loss: All modalities Sphincter paralysis Reflexes: Tendon reduced; ± Extensor plantar Course Progressive: Ascending paralysis Poor prognosis [neuromuscular.wustl.edu]

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]

結節性硬化症 Tuberous sclerosis complex (TSC), unknown genetic etiology 結節性硬化症、遺伝子型不明 1 不要 有 HPS2395 HPS2396 HPS2397 HPS2398 HPS2399 HPS2400 159. [cell.brc.riken.jp]

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 multiple system [radiopaedia.org]

An etiologic association between cancer and cerebellar degeneration was first clearly postulated by Lord Russell Brain in 1951 ( Brain et al 1951 ). [medlink.com]

Conclusions : HIV infection should be considered as an etiology in clinical setting of subacute cerebellar ataxia, particularly in a young or immunocompromised patient. To cite this abstract in AMA style: L. Beltrami, J. Kristochik, M. Novaes, G. [mdsabstracts.org]

Epidemiology

Plasma exchange Ataxia with antibodies to CARP 8 10 Epidemiology: 1 patient; 77 year old female Clinical: Pure cerebellar syndrome Onset: 3 years after neoplasm Ataxia: Limb; Truncal; Gait; Dysarthria Ocular: Horizontal nystagmus Mental status: Normal [neuromuscular.wustl.edu]

Epidemiology Frequency United States In one study, paraneoplastic cerebellar degeneration was observed in 25% of paraneoplastic neurologic syndromes, occurring in 2 of every 1000 patients with cancer. [12] Mortality/Morbidity In the study cited above, [emedicine.medscape.com]

Epidemiology and estimated population burden of selected autoimmune diseases in the United States. Clin Immunol Immunopathol 1997; 84: 223-43. In article View Article PubMed [2] Golden SH, Robinson KA, Saldanha I, Anton B, Ladenson PW. [pubs.sciepub.com]

Pathophysiology

Not all gynecologic cancers present as paraneoplastic neurologic syndrome; however, in a clinical presentation consistent with a paraneoplastic neurologic syndrome, the chances of underlying malignancy are very high. " "Pathophysiology: Paraneoplastic [usmleforum.com]

Pathophysiology [ edit ] The anti-Purkinje cell antibodies originally described in PCD led to the hypothesis that the antibody might be pathogenic, much as earlier studies had demonstrated pathogenicity of anti-acetylcholine receptor antibodies in myasthenia [en.wikipedia.org]

Pathophysiology Paraneoplastic cerebellar degeneration is caused by the secondary effects of cancer and is believed to be immune mediated. [emedicine.medscape.com]

Pathophysiology of this syndrome is not entirely understood but onconeural antibodies suggest that it is an immune mediated state in which body may produce antibodies against tumor cells and these antibodies cross react with cerebellar tissue and destroy [omicsonline.org]

A case of finger clubbing associated with nasopharyngeal carcinoma in a young girl, and review of pathophysiology. J Pak Med Assoc 2009; 59(4): 253-4. [ PubMed ] 29. Garganese MC, et al. [medical.nikkeibp.co.jp]

Prevention

Since 2005, he also acts as director of the Department of Clinical Medicine and Preventive Medicine at the Danube University. His main scientific interests focus on cerebrovascular disorders including early treatment and rehabilitation. [books.google.de]

Página 310 - A randomized trial comparing ticlopidine hydrochloride with aspirin for the prevention of stroke in high-risk patients. ‎ [books.google.es]

At the same time, underlying complications were prevented actively, and the peripheral nerves were protected. [journals.lww.com]

All these changes can be prevented (and in their early stages, reversed) by vitamin B12 administration. Updated: May, 2017 [neuropathology-web.org]

Treatment [ edit ] Tumor removal is still the therapeutic mainstay with very early treatment being essential to prevent irreversible neuronal loss. Immunosuppressive or immunomodulatory treatments are often ineffective. [en.wikipedia.org]

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