Franziska Hoche, MD


Dr. Franziska Hoche (M.D.), a postdoctoral young investigator and highly trained pediatric neurology resident who has joined us in 2012 having received cerebellar research scholarships of the German Neuropediatric Society and the Dr. Senckenberg’ Society Frankfurt.

Dr. Hoche received her M.D. at the Goethe – University in Frankfurt, Germany, where she finished her doctoral thesis on neurodegeneration of auditory brainstem nuclei and fibre tracts in Spinocerebellar Ataxia Type 2,  Type 3 and Type 7 (SCA2, SCA3, SCA7) at the lab of Professor Udo Rüb and Professor Horst Werner Korf at the Dr. Senckenberg’ Institute of Chronomedicine.

Her research focuses on the understanding of influences and contributors to motor and cognitive dysfunctions in cerebellar disorders of childhood and adulthood. She uses both tools of classic clinical neurology, developmental psychology, neuropsychology and cognitive neuroscience as well as cutting-edge neuroimaging techniques in order to search for interactions, concepts, maps and analogies of how cognition and behavior are affected in children and adults with cerebellar diseases.

Dr. Hoche has a special interest in the neurodegenerative and cognitive pattern of a hereditary cerebellar diseases of childhood and she is one of few international physician scientist experts in the neurocognitive profile of Ataxia-telangiectasia (AT).

Her research projects in the Schmahmann Lab include evaluation of cognitive and behavioral changes and the cerebellar cognitive affective syndrome (CCAS) in adult ataxic patients and pediatric patients with Ataxia-telangiectasia (AT), as well as the development of a pediatric ataxia rating scale.

Dr. Hoche received awards of the Neuroscientific Society in Frankfurt (2011) for her work on cerebellar pathology in SCAs and the American Neurological Association (ANA) (2014) for her work on the role of the cerebellum in social cognition.

Dr. Hoche has received research grants from the National Ataxia Foundation (2013) for the development of a pediatric ataxia rating scale and the A-T Children’s Project (ATCP) (2014) for investigating the cognitive profile of patients with AT.

Her recent publication on defining the cerebellar phenotype in AT has been chosen article of the month by the Editor of the Journal of Pediatric Neurology (2014).



  1. Growth retardation and growth hormone deficiency in patients with Ataxia telangiectasia. (2014)
  2. Cognitive Phenotype in Ataxia-Telangiectasia. (2014)
  3. Novel N-terminal truncating CLCN1 mutation in severe Becker disease (2014)
  4. Involvement of the cholinergic basal forebrain nuclei in spinocerebellar ataxia type 2 (SCA2) (2013)
  5. Degeneration of the cerebellum in Huntington’s disease (HD): possible relevance for the clinical picture and potential gateway to pathological mechanisms of the disease process. (2013)
  6. Neurodegeneration in ataxia telangiectasia: what is new? What is evident?  (2012)
  7. Rare brain biopsy findings in a first ADEM-like event of pediatric MS: histopathologic, neuroradiologic and clinical features (2011)
  8. Spinocerebellar ataxia type 2 (SCA2): identification of early brain degeneration in one monozygous twin in the initial disease stage (2011)
  9. Extracerebellar MRI-lesions in ataxia telangiectasia go along with deficiency of the GH/IGF-1 axis, markedly reduced body weight, high ataxia scores and advanced age (2010)
  10. Involvement of the auditory brainstem system in spinocerebellar ataxia type 2 (SCA2), type 3 (SCA3) and type 7 (SCA7) (2008)

A full list of Dr. Hoche’s publications can be found here.

Share this with someone!