History

Fact Explanation
Ataxic gait [1][2] Cerebellar ataxia is typically described as broad-based gait where patient tends to fall on to side same as the most effected side of cerebellum
Dysarthria [3][4][8] Due to difficult in articulation of the words patient tend to have a scanning speech. [3][4]
Dysmetry [1][4] Patient have poor perception of distance, thus tends to over or undershoot while trying to touch a determined point[1][4]
Intentional tremor [1] Some patients have been reported to have tremor outlasting the other symptoms of cerebellitis [1]
Skin lesions compatible with varicella infection [1] They occur in different stages; some still developing while other maybe in resolution phase. [1] Symptoms of cerebellitis may even surface without the viral rash, such cases has been reported in both healthy and immunocompetent patients [2]
Fever [4] Associated with underlying viral infection [4]
Signs of respiratory or gasterointestinal infection [6] These self limiting signs of viral infection may precede the cerebellar signs. This phenomenon is mainly seen in children and young adults [6]
Signs of lung tumor, breast tumor or Hodgkin's lymphoma [5][6] Small cell lung cancer, breast cancer and Hodgkin's lymphoma are associated with paraneoplastic syndrome that results in cerebellar signs [5][6]
References
  1. BOZZOLA ELENA, BOZZOLA MAURO, TOZZI ALBERTO, CALCATERRA VALERIA, LONGO DANIELA, KRZYSTOFIAK ANDRZEJ, VILLANI ALBERTO. Acute cerebellitis in varicella: a ten year case series and systematic review of the literature. [online] Italian Journal of Pediatrics 2014, 40:57 [viewed 29 September 2014] Available from: doi:10.1186/1824-7288-40-57 ********Please be kind enough to note that the previous citatation was one generated by the citation tool with the doi of the article; I have manually corrected it now. Thought letting you know would improve the accuracy of the citation tool.***
  2. GILDEN D, COHRS RJ, MAHALINGAM R, NAGEL MA. Neurological Disease Produced by Varicella Zoster Virus Reactivation Without Rash Curr Top Microbiol Immunol [online] 2010:243-253 [viewed 30 September 2014] Available from: doi:10.1007/82_2009_3
  3. WITHANA MILINDA, RODRIGO CHATURAKA, CHANG THASHI, KARUNANAYAKE PANDUKA, RAJAPAKSE SENAKA. Dengue fever presenting with acute cerebellitis: a case report. Array [online] BMC Research Notes 2014, 7:125 [viewed 30 September 2014] Available from: doi:10.1186/1756-0500-7-125
  4. CIARDI MARIA, GIACCHETTI GIOVANNA, FEDELE CESARE GIOVANNI, TENORIO ANTONIO, BRANDI ANTONELLA, LIBERTONE RAFFAELLA, AJASSA CAMILLA, BORGESE LEONARDO, DELIA SALVATORE. Acute Cerebellitis Caused by Herpes Simplex Virus Type 1. CLIN INFECT DIS [online] 2003 February, 36(3):e50-e54 [viewed 30 September 2014] Available from: doi:10.1086/345781
  5. DALMAU J, ROSENFELD MR. Paraneoplastic syndromes of the CNS Lancet Neurol [online] 2008 Apr, 7(4):327-340 [viewed 01 October 2014] Available from: doi:10.1016/S1474-4422(08)70060-7
  6. HöFTBERGER R, SABATER L, ORTEGA A, DALMAU J, GRAUS F. Patient With Homer-3 Antibodies and Cerebellitis JAMA Neurol [online] 2013 Apr, 70(4):506-509 [viewed 01 October 2014] Available from: doi:10.1001/jamaneurol.2013.1955
  7. TABARKI MELAIKI B, THABET F, ANJUM S B, AMIN A. Acute cerebellitis with hydrocephalus and brainstem compression. Archives of Disease in Childhood [online] 2007 January, 92(1):38-38 [viewed 05 October 2014] Available from: doi:10.1136/adc.2006.105874
  8. SFEIR MM, NAJEM CE. Cerebellitis Associated with Influenza A(H1N1)pdm09, United States, 2013 Emerg Infect Dis [online] 2014 Sep, 20(9):1578-1580 [viewed 07 October 2014] Available from: doi:10.3201/eid2009.140160

Examination

Fact Explanation
Nystagmus [1][2] Elicited by asking the patient to look to one side; downbeat nystagmus is particularly seen in cerebellar lesion where the fast phase beat is in a downward direction. It increases when patient looks to sideward, downward and on lying prone. Paraneoplastic cerebellar disorders are associated with opsoclonus where there will be a rapid, irregular movements in all directions. [3]
Scanning speech [1] Occurs due to difficulty in articulation. [1]
Dysmetry and intentional tremor [1][2] Elicited by finger-nose test [4] in the upper limb and heel-shin test in lower limb. Ask the patient to touch his/ her nose tip and touch the examiner's finger or ask patient to place his heel on the other knee and draw it along the shin upto ankle. Patient with cerebellar lesions will over or undershoot the target and will have coarse tremors while approaching it.
Dysdiadokokinesia [2] Elicited by asking the patient to place one hand over the other and flip the hand on top in quick alternating movements; patients with cerebellar lesion can't maintain a normal rhythm.
Ataxic gait [1][2] Patients will have broad based ataxic gait [1]
Rebound phenomenon [5] Positive [5]
References
  1. BOZZOLA ELENA, BOZZOLA MAURO, TOZZI ALBERTO, CALCATERRA VALERIA, LONGO DANIELA, KRZYSTOFIAK ANDRZEJ, VILLANI ALBERTO. Acute cerebellitis in varicella: a ten year case series and systematic review of the literature. Array [online] Italian Journal of Pediatrics 2014, 40:57 [viewed 29 September 2014] Available from: doi:10.1186/1824-7288-40-57
  2. WITHANA MILINDA, RODRIGO CHATURAKA, CHANG THASHI, KARUNANAYAKE PANDUKA, RAJAPAKSE SENAKA. Dengue fever presenting with acute cerebellitis: a case report. Array [online] BMC Research Notes 2014, 7:125 [viewed 30 September 2014] Available from: doi:10.1186/1756-0500-7-125
  3. STRUPP M, HüFNER K, SANDMANN R, ZWERGAL A, DIETERICH M, JAHN K, BRANDT T. Central Oculomotor Disturbances and Nystagmus: A Window Into the Brainstem and Cerebellum Dtsch Arztebl Int [online] 2011 Mar, 108(12):197-204 [viewed 01 October 2014] Available from: doi:10.3238/arztebl.2011.0197
  4. AMJAD Nida, HAQUE Anwarul, AHMED Khalid. Acute Cerebellitis with Hydrocephalus [online] Journal of the College of Physicians and Surgeons Pakistan 2014, Vol. 24 (Special Supplement 2): S127-S128 [Viewed on 07 November 2014] Available from: http://www.jcpsp.pk/archive/2014/SS_May2014/23.pdf
  5. PAUL R, SINGHANIA P, HASHMI M, BANDYOPADHYAY R, BANERJEE AK. Post chicken pox neurological sequelae: Three distinct presentations J Neurosci Rural Pract [online] 2010, 1(2):92-96 [viewed 07 October 2014] Available from: doi:10.4103/0976-3147.71718

Differential Diagnoses

Fact Explanation
Acute primary infective cerebellitis [2] Occur with varicella zoster, Epstein–Barr, measles, mumps, rubella, herpes simplex and coxsackie viral infections, influenza virus [2][6] Concurrent features specific for the infections along with the cerebellar signs points more towards acute primary infective cerebellitis due to direct invasion of organism in cerebellum rather than post infective cerebellitis. [6]
Post-infective cerebellitis [2] Have been described following varicella zoster virus, coxsackie virus, Epstein–Barr virus and human immunodeficiency virus, influenza virus [2][6] Patients will have history of the above illness rather than concurrent illness as seen in acute primary infective cerebellitis [8]
Syndrome of unexplained cerebellar degeneration [3] Cases have been described in patients with HIV; suspect in known patient with HIV [3]
Autoimmune cerebellitis [5] Glutamic acid decarboxylase, metabotropic glutamate receptor type 1 (mGluR1), contactin-associated protein 2, Homer-3 antibodies are associated with autoimmune cerebellitis [5] Autoimmune disease with glutamic acid decarboxylase antibody has a slower progression than cerebellitis associated with paraneoplastic syndrome and is associated with polyendocrine dysfunction such as late-onset insulin-dependant diabetes mellitus, thyroiditis and pernicious anaemia. [4] When suspected, antibody levels allows to differenciate it from other causes. [5]
Cerebellitis following paraneoplastic syndrome [4] Small cell lung cancer, breast cancer and Hodgkin's lymphoma are examples of cancers that cause paraneoplastic syndrome with cerebellar disorder. [4]
Creutzfeldt-Jakob disease [4] This is a prion disease, symptoms may mimic cerebellar disorder [4]
Drug induced cerebellitis [7] Has been reported with isoniazid and alcohol. This should be suspected when the drug is known to be used on the patient in recent pass and can be confirmed by improvement following cessation of the drug [7]
References
  1. BOZZOLA ELENA, BOZZOLA MAURO, TOZZI ALBERTO, CALCATERRA VALERIA, LONGO DANIELA, KRZYSTOFIAK ANDRZEJ, VILLANI ALBERTO. Acute cerebellitis in varicella: a ten year case series and systematic review of the literature. [online] Italian Journal of Pediatrics 2014, 40:57 [viewed 29 September 2014] Available from: doi:10.1186/1824-7288-40-57
  2. WITHANA MILINDA, RODRIGO CHATURAKA, CHANG THASHI, KARUNANAYAKE PANDUKA, RAJAPAKSE SENAKA. Dengue fever presenting with acute cerebellitis: a case report. Array [online] BMC Research Notes 2014, 7:125 [viewed 30 September 2014] Available from: doi:10.1186/1756-0500-7-125
  3. CIARDI MARIA, GIACCHETTI GIOVANNA, FEDELE CESARE GIOVANNI, TENORIO ANTONIO, BRANDI ANTONELLA, LIBERTONE RAFFAELLA, AJASSA CAMILLA, BORGESE LEONARDO, DELIA SALVATORE. Acute Cerebellitis Caused by Herpes Simplex Virus Type 1. CLIN INFECT DIS [online] 2003 February, 36(3):e50-e54 [viewed 30 September 2014] Available from: doi:10.1086/345781
  4. DALMAU J, ROSENFELD MR. Paraneoplastic syndromes of the CNS Lancet Neurol [online] 2008 Apr, 7(4):327-340 [viewed 01 October 2014] Available from: doi:10.1016/S1474-4422(08)70060-7
  5. HöFTBERGER R, SABATER L, ORTEGA A, DALMAU J, GRAUS F. Patient With Homer-3 Antibodies and Cerebellitis JAMA Neurol [online] 2013 Apr, 70(4):506-509 [viewed 01 October 2014] Available from: doi:10.1001/jamaneurol.2013.1955
  6. SFEIR MM, NAJEM CE. Cerebellitis Associated with Influenza A(H1N1)pdm09, United States, 2013 Emerg Infect Dis [online] 2014 Sep, 20(9):1578-1580 [viewed 07 October 2014] Available from: doi:10.3201/eid2009.140160
  7. PETER P, JOHN M. Isoniazid-induced cerebellitis: a disguised presentation. Singapore Med J [online] 2014 Jan, 55(1):e17-9 [viewed 07 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24452983
  8. PAUL R, SINGHANIA P, HASHMI M, BANDYOPADHYAY R, BANERJEE AK. Post chicken pox neurological sequelae: Three distinct presentations J Neurosci Rural Pract [online] 2010, 1(2):92-96 [viewed 07 October 2014] Available from: doi:10.4103/0976-3147.71718

Investigations - for Diagnosis

Fact Explanation
Full blood count and ESR [4] Elevated white cell count with high ESR (Erythrocyte Sedimentation Rate) can be found if due to infection. [4]
Blood for dengue antibodies [3] Dengue nonstructural protein antigen 1 can be detected during early infection and IgM/ IgG antibody in late presentation. [3]
Onconeural antibodies [5] These are markers of paraneoplastic syndrome and performed when suspected. [5]
Autoantibodies [5] Several autoantibodies have been described in primary autoimmune cerebellitis. Ex: Glutamic acid decarboxylase, metabotropic glutamate receptor type 1 (mGluR1), contactin-associated protein 2, Homer-3 antibodies
Lumbar puncture and CSF analysis [1] CSF full report mild lymphocytic pleocytosis is detected in viral infection such as Varicella Zoster Virus, HSV, EBV, CMV, HHV 6-8 14-3-3 protein maybe detected in cerebellitis associated with paraneoplastic syndrome but is not specific to this. [2][6] PCR for viruses such as Varicella Zoster virus and if delayed antibodies against them can be detected. [1][2]
Magnetic resonance imaging (MRI) [1][4] Hyperintense areas in bilateral cerebellar gray matter in T2-weighted sequences can be visualised but this is not patognomonic and has no prognostic value. If clinically obvious MRI is not recommended and where performed MRI is superior to CT. [1][4]
References
  1. BOZZOLA ELENA, BOZZOLA MAURO, TOZZI ALBERTO, CALCATERRA VALERIA, LONGO DANIELA, KRZYSTOFIAK ANDRZEJ, VILLANI ALBERTO. Acute cerebellitis in varicella: a ten year case series and systematic review of the literature. [online] Italian Journal of Pediatrics 2014, 40:57 [viewed 29 September 2014] Available from: doi:10.1186/1824-7288-40-57
  2. GILDEN D, COHRS RJ, MAHALINGAM R, NAGEL MA. Neurological Disease Produced by Varicella Zoster Virus Reactivation Without Rash Curr Top Microbiol Immunol [online] 2010:243-253 [viewed 30 September 2014] Available from: doi:10.1007/82_2009_3
  3. WITHANA MILINDA, RODRIGO CHATURAKA, CHANG THASHI, KARUNANAYAKE PANDUKA, RAJAPAKSE SENAKA. Dengue fever presenting with acute cerebellitis: a case report. Array [online] BMC Research Notes 2014, 7:125 [viewed 30 September 2014] Available from: doi:10.1186/1756-0500-7-125
  4. CIARDI MARIA, GIACCHETTI GIOVANNA, FEDELE CESARE GIOVANNI, TENORIO ANTONIO, BRANDI ANTONELLA, LIBERTONE RAFFAELLA, AJASSA CAMILLA, BORGESE LEONARDO, DELIA SALVATORE. Acute Cerebellitis Caused by Herpes Simplex Virus Type 1. CLIN INFECT DIS [online] 2003 February, 36(3):e50-e54 [viewed 30 September 2014] Available from: doi:10.1086/345781
  5. HöFTBERGER R, SABATER L, ORTEGA A, DALMAU J, GRAUS F. Patient With Homer-3 Antibodies and Cerebellitis JAMA Neurol [online] 2013 Apr, 70(4):506-509 [viewed 01 October 2014] Available from: doi:10.1001/jamaneurol.2013.1955
  6. DALMAU J, ROSENFELD MR. Paraneoplastic syndromes of the CNS Lancet Neurol [online] 2008 Apr, 7(4):327-340 [viewed 01 October 2014] Available from: doi:10.1016/S1474-4422(08)70060-7

Investigations - Followup

Fact Explanation
No long term complications are expected in acute viral cerebellitis. [1] In multiple long term follow up of patients who have had acute viral cerebellitis there has been no cases of developmental retardation, epilepsy, deafness or disorders directly caused by cerebellitis. [1]
Cerebellar symptoms may outlast the febrile illness. [2] In a reported case of dengue cerebellar symptoms outlasted fever by weeks. [2]
References
  1. BOZZOLA ELENA, BOZZOLA MAURO, TOZZI ALBERTO, CALCATERRA VALERIA, LONGO DANIELA, KRZYSTOFIAK ANDRZEJ, VILLANI ALBERTO. Acute cerebellitis in varicella: a ten year case series and systematic review of the literature. [online] Italian Journal of Pediatrics 2014, 40:57 [viewed 29 September 2014] Available from: doi:10.1186/1824-7288-40-57
  2. WITHANA MILINDA, RODRIGO CHATURAKA, CHANG THASHI, KARUNANAYAKE PANDUKA, RAJAPAKSE SENAKA. Dengue fever presenting with acute cerebellitis: a case report. Array [online] BMC Research Notes 2014, 7:125 [viewed 30 September 2014] Available from: doi:10.1186/1756-0500-7-125

Management - Specific Treatments

Fact Explanation
Antiviral therapy [1][2] 30 mg/kg/day acyclovir IV for five days is recommended for acute cerebellitis caused by Varicella Zoster Viral infection. But the use of antiviral therapy controversial due to possible autoimmune pathogenesis [1]
Steroids [1][2] 0.15-0.5 mg/Kg/day dexamethasone IV is recommended in some patients but the use of this is controversial [1]
Treatment of tumor [3] In cerebellitis caused by paraneoplastic syndrome treatment of the tumor is associated with recovery from symptoms. Treatment with steroids, plasma exchange, IV immunoglobulin or cyclophosphamide does not modify the outcome in patients in whom the tumor has been successfully treated. [3]
Stop the insulting drug [4] If drug induced cerebellitis is suspected, stopping the drug results in improvement of the symptoms. In cases due to isoniazid, pyridoxine can be used. [4]
References
  1. BOZZOLA ELENA, BOZZOLA MAURO, TOZZI ALBERTO, CALCATERRA VALERIA, LONGO DANIELA, KRZYSTOFIAK ANDRZEJ, VILLANI ALBERTO. Acute cerebellitis in varicella: a ten year case series and systematic review of the literature. [online] Italian Journal of Pediatrics 2014, 40:57 [viewed 29 September 2014] Available from: doi:10.1186/1824-7288-40-57
  2. CIARDI MARIA, GIACCHETTI GIOVANNA, FEDELE CESARE GIOVANNI, TENORIO ANTONIO, BRANDI ANTONELLA, LIBERTONE RAFFAELLA, AJASSA CAMILLA, BORGESE LEONARDO, DELIA SALVATORE. Acute Cerebellitis Caused by Herpes Simplex Virus Type 1. CLIN INFECT DIS [online] 2003 February, 36(3):e50-e54 [viewed 30 September 2014] Available from: doi:10.1086/345781
  3. DALMAU J, ROSENFELD MR. Paraneoplastic syndromes of the CNS Lancet Neurol [online] 2008 Apr, 7(4):327-340 [viewed 01 October 2014] Available from: doi:10.1016/S1474-4422(08)70060-7
  4. PETER P, JOHN M. Isoniazid-induced cerebellitis: a disguised presentation. Singapore Med J [online] 2014 Jan, 55(1):e17-9 [viewed 07 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24452983