Progressive Ataxia and Palatal Tremor

Four New Cases of Progressive Ataxia and Palatal Tremor (PAPT) and a Literature Review

“…With the lack of other seminal findings on extensive aetiological work-up, the relatively isolated clinical PAPT syndrome, the disease course, and the cerebral imaging in this patient would be consistent with case reports of patients with presumed idiopathic PAPT [6,[9][10][11][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39].…”

Four New Cases of Progressive Ataxia and Palatal Tremor (PAPT) and a Literature Review

“…With the lack of other seminal findings on extensive aetiological work-up, the relatively isolated clinical PAPT syndrome, the disease course, and the cerebral imaging in this patient would be consistent with case reports of patients with presumed idiopathic PAPT [6,[9][10][11][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39].…”

Frequency of Bilateral Hypertrophic Olivary Degeneration in a Large Retrospective Cohort

“…HOD has previously been described as primarily a unilateral process . Increased prevalence of bilateral HOD in this series may be due to a referral bias at a tertiary referral center or a population otherwise skewed toward patients with PAPT which is typically a bilateral process . Included in referral bias is passage of additional time for signs, symptoms, and imaging findings to progress into a more coherent picture.…”

“…Structurally there may be brainstem and cerebellar atrophy as well as hypertrophy of the ION. Familial forms of PAPT usually have normal ION and this is thought to be a functional, rather than structural, abnormality resulting in PT …”

Delayed Occurrence of Hypertrophic Olivary Degeneration after Therapy of Posterior Fossa Tumors: A Single Institution Retrospective Analysis

“…It is noteworthy that if a lesion could be identified, HOD was due to a variety of etiologies in this study, such as trauma/surgery (32%), vascular malformation (21%), and infarction (16%). In rare cases, HOD can occur without injury to the DROP and can be accompanied by progressive ataxia and palatal tremor, so-called PAPT-syndrome, which is idiopathic and typically a bilateral process [10,11,[19][20][21]. The development of HOD after focal radiotherapy such as Gamma-Knife with injury to the DROP was described in a case series by Yun et al for four patients and in a single case report of radiosurgery for pontine metastasis, but did not occur in our series [22,23].…”

Four New Cases of Progressive Ataxia and Palatal Tremor (PAPT) and a Literature Review

“…With the lack of other seminal findings on extensive aetiological work-up, the relatively isolated clinical PAPT syndrome, the disease course, and the cerebral imaging in this patient would be consistent with case reports of patients with presumed idiopathic PAPT [6,[9][10][11][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39].…”

Frequency of Bilateral Hypertrophic Olivary Degeneration in a Large Retrospective Cohort

“…HOD has previously been described as primarily a unilateral process . Increased prevalence of bilateral HOD in this series may be due to a referral bias at a tertiary referral center or a population otherwise skewed toward patients with PAPT which is typically a bilateral process . Included in referral bias is passage of additional time for signs, symptoms, and imaging findings to progress into a more coherent picture.…”

“…Structurally there may be brainstem and cerebellar atrophy as well as hypertrophy of the ION. Familial forms of PAPT usually have normal ION and this is thought to be a functional, rather than structural, abnormality resulting in PT …”

Delayed Occurrence of Hypertrophic Olivary Degeneration after Therapy of Posterior Fossa Tumors: A Single Institution Retrospective Analysis

“…It is noteworthy that if a lesion could be identified, HOD was due to a variety of etiologies in this study, such as trauma/surgery (32%), vascular malformation (21%), and infarction (16%). In rare cases, HOD can occur without injury to the DROP and can be accompanied by progressive ataxia and palatal tremor, so-called PAPT-syndrome, which is idiopathic and typically a bilateral process [10,11,[19][20][21]. The development of HOD after focal radiotherapy such as Gamma-Knife with injury to the DROP was described in a case series by Yun et al for four patients and in a single case report of radiosurgery for pontine metastasis, but did not occur in our series [22,23].…”

Four New Cases of Progressive Ataxia and Palatal Tremor (PAPT) and a Literature Review

“…With the lack of other seminal findings on extensive aetiological work-up, the relatively isolated clinical PAPT syndrome, the disease course, and the cerebral imaging in this patient would be consistent with case reports of patients with presumed idiopathic PAPT [6,[9][10][11][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39].…”

Frequency of Bilateral Hypertrophic Olivary Degeneration in a Large Retrospective Cohort

“…HOD has previously been described as primarily a unilateral process . Increased prevalence of bilateral HOD in this series may be due to a referral bias at a tertiary referral center or a population otherwise skewed toward patients with PAPT which is typically a bilateral process . Included in referral bias is passage of additional time for signs, symptoms, and imaging findings to progress into a more coherent picture.…”

“…Structurally there may be brainstem and cerebellar atrophy as well as hypertrophy of the ION. Familial forms of PAPT usually have normal ION and this is thought to be a functional, rather than structural, abnormality resulting in PT …”

Delayed Occurrence of Hypertrophic Olivary Degeneration after Therapy of Posterior Fossa Tumors: A Single Institution Retrospective Analysis

“…It is noteworthy that if a lesion could be identified, HOD was due to a variety of etiologies in this study, such as trauma/surgery (32%), vascular malformation (21%), and infarction (16%). In rare cases, HOD can occur without injury to the DROP and can be accompanied by progressive ataxia and palatal tremor, so-called PAPT-syndrome, which is idiopathic and typically a bilateral process [10,11,[19][20][21]. The development of HOD after focal radiotherapy such as Gamma-Knife with injury to the DROP was described in a case series by Yun et al for four patients and in a single case report of radiosurgery for pontine metastasis, but did not occur in our series [22,23].…”