Welcome to the WCN 2021 Interactive Program

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Displaying One Session

Free Communication
Session Time
11:30 - 13:00
Room
Free Communication B
Chair(s)
  • Max J. Hilz (Germany)
Free Communication

CARDIOVASCULAR AUTONOMIC DYSFUNCTION IN PATIENTS WITH POSTERIOR CIRCULATION ISCHEMIC STROKE

Session Type
Free Communication
Date
06.10.2021, Wednesday
Session Time
11:30 - 13:00
Room
Free Communication B
Lecture Time
11:30 - 11:40
Presenter
  • Ruihao Wang (Germany)

Abstract

Background and Aims:

Although it is well known that ischemic stroke may cause cardiovascular autonomic dysfunction, most studies focus on patients with anterior circulation stroke. In patients with posterior circulation ischemic stroke (PCIS), cardiovascular autonomic modulation has only been rarely studied. Therefore, we aimed to assess cardiovascular autonomic modulation in PCIS patients.

Methods:

In 57 patients with PCIS (12 occipital lobe, 14 thalamic, 11 cerebellar, 20 brainstem strokes) and 30 age- and gender-matched controls, we recorded RR-intervals (RRI), systolic, diastolic blood pressures (BPsys, BPdia), and respiration (RESP) at supine rest, within one week after stroke-onset. We calculated parameters reflecting total cardiac autonomic modulation [RRI-standard-deviation (RRI-SD), RRI-total-powers], mainly or exclusively sympathetic [RRI-low-frequency-powers (RRI-LF-powers) and BPsys-LF-powers] and parasympathetic cardiovascular modulation [Root-Mean-Square-of-Successive-RRI-Differences (RMSSD), RRI-high-frequency-powers (RRI-HF-powers)], sympathetic-parasympathetic balance (RRI-LF/HF-ratios), and baroreflex-sensitivity (BRS). Values were compared with one-way ANOVA or Kruskal-Wallis-Test, with post-hoc analyses. Significance was assumed for p<0.05.

Results:

In the patients of all PCIS- subgroups, values of RRI, RRI-SD, RMSSD, RRI-HF-powers, and BRS were significantly lower, while BPsys-LF-powers were higher than in the controls. In patients with ischemia in the occipital lobe, RRI-LF/HF-ratios were significant higher than in controls. Autonomic parameters did not differ between PCIS-subgroups.

Conclusions:

Within one week after PCIS-onset, patients have reduced parasympathetic and increased sympathetic cardiovascular modulation with diminished BRS. These findings are similar to our previous findings in patients after acute ischemic stroke in the middle cerebral artery territory (Hilz et al. 2011). The shift towards sympathetic predominance seems to be more pronounced in patients with occipital lobe lesions.

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Free Communication

SUDOMOTOR AND CARDIOVASCULAR AUTONOMIC FUNCTION IN DE NOVO PARKINSON'S DISEASE ASSESSED BY SUDOSCAN AND CARDIOVASCULAR REFLEXES

Session Type
Free Communication
Date
06.10.2021, Wednesday
Session Time
11:30 - 13:00
Room
Free Communication B
Lecture Time
11:40 - 11:50
Presenter
  • Camilla Rocchi (Italy)

Abstract

Background and Aims:

The prevalence of autonomic involvement in early stage of Parkinson ’s disease (PD) is still debated. Aim of this study is to assess the autonomic functions in de novo PD patients (dnPD) in comparison with PD patients on therapy (PDot) and healthy controls (HC).

Methods:

Twenty-eight dnPD and 24 PDot, to whom Unified Parkinson’s Rating Scale (UPDRS) was administered, and 23 HC underwent electrochemical skin conductance (ESC) measured with sudoscan, cardiovascular reflexes (head-up tilt test HUTT, Valsalva maneuver, deep breathing, hand grip, and cold face),and Scales for Outcomes in Parkinson's Disease-Autonomic Dysfunction (SCOPA-AUT) questionnaire.

Results:

The mean SCOPA-AUT total score was significantly higher in dnPD group compared with HC group (p<0.001) and significantly lower than PDot (p=0.004). No significant difference of ESC mean values were found between dnPD and HC group. DnPD had a significantly lower diastolic blood pressure (BP) response at handgrip test (p=0.005) compared with HC. Hands and feet ESC significantly negatively correlated with disease duration (p=0.014; p=0.025) and feet ESC significantly negatively correlated with UPDRS III (p=0.039). Systolic and diastolic BP responses at 3rd minute of HUTT correlated significantly negatively with disease duration (p<0.001; p=0.003) and with UPDRSIII (p=0.001; p<0.001). BP response to Valsalva maneuver negatively correlated with UPDRSIII (p=0.006).

Conclusions:

Although dnPD patients complain of thermoregulatory symptoms, we found no alteration in the sudomotor function investigated with sudoscan. Furthermore, a deficit of the sympathetic vasoconstrictive response to the isometric exercise was detected, suggesting an early involvement of the autonomic cardiovascular components in dnPD.

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Free Communication

ASSESSMENT OF CARDIOVASCULAR AUTONOMIC FUNCTION IN HEREDITARY TRANSTHYRETIN AMYLOIDOSIS

Session Type
Free Communication
Date
06.10.2021, Wednesday
Session Time
11:30 - 13:00
Room
Free Communication B
Lecture Time
11:50 - 12:00
Presenter
  • Pietro Guaraldi (Italy)

Abstract

Background and Aims:

Autonomic dysfunction is a key feature of hereditary transthyretin amyloidosis (hATTR). In addition to causing prominent and disabling heterogenic manifestation (orthostatic hypotension, sweating disturbances, erectile and voiding dysfunction, etc.), it carries important prognostic value. The objective of this study was to assess in detail cardiovascular autonomic function throughout a battery of standardized autonomic tests in patients with genetically proven hATTR and healthy controls (HC).

Methods:

Systolic and diastolic blood pressure (SBP, DBP), heart rate (HR) and breathing were continuously monitored at supine rest and during head-up tilt test (HUTT), Valsalva manoeuvre (VM), deep breathing (DB), cold face (IE) test and isometric handgrip (HG) in 19 hATTR and 19 HC.

Results:

During HUTT, hATTR presented significantly lower SBP and DBP values compared to HC, and 6 hATTR patients fulfilled the criteria for orthostatic hypotension. Overshoot and Valsalva ratio were significantly lower in hATTR and pathological in all symptomatic patients. During DB, hATTR presented significantly lower respiratory sinus arrhythmia compared to HC and all symptomatic patients displayed pathological results. SBP, DBP and HR responses to HG were significantly reduced in hATTR compared to HC.

Conclusions:

both cardiovagal and cardiovascular adrenergic function are affected in hATTR, however, cardiovagal dysfunction was more diffused and pronounced.

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Free Communication

DECREASED CARDIAC AUTONOMIC ACTIVITY AND INCREASED SYMPATHETIC VASCULAR CONTROL IN ADVANCED STAGES OF AMYOTROPHIC LATERAL SCLEROSIS

Session Type
Free Communication
Date
06.10.2021, Wednesday
Session Time
11:30 - 13:00
Room
Free Communication B
Lecture Time
12:00 - 12:10
Presenter
  • Zehui Li (China)

Abstract

Background and Aims:

To explore the changes in cardiovascular autonomic function in patients with amyotrophic lateral sclerosis (ALS) during disease progression.

Methods:

Heart rate and systolic and diastolic blood pressure (SBP, DBP) were continuously recorded 24 hours and compound autonomic symptom score (COMPASS31) was performed for 26 ALS patients and 26 controls. Heart rate variability parameters including SDNN, SDANN, RMSSD, and pNN50 were calculated. Disease severity was evaluated by ALS Functional Rating Scale-Revised (ALSFRS-R) score and King's clinical staging system (early-ALS-Group: King's stage 1 [1 patient] and stage 2 [12 patient]; late-ALS-Group: King's stage 3 [9 patients] and stage 4 [4 patients]).

Results:

No differences were found in age, gender ratio, heart rate, SBP, RMSSD, pNN50 and COMPASS31 score among the three groups. In the late-ALS-Group, SDNN (105.00±21.68ms) and SDANN (91.77±21.26ms) were lower than the early-ALS-Group (SDNN: 134.31±26.11ms, p=0.002; SDANN: 116.46±29.38ms, p=0.014) and controls (SDNN: 130.15±22.46ms, p=0.002; SDANN: 116.46±23.94ms, p=0.005), while in the late-ALS-Group diastolic blood pressure in both day time (day-DBP, 77.2±5.8mmHg) and night time (night-DBP, 76.6±5.7mmHg) were higher than the early-ALS-Group (day-DBP: 71.3±5.1mmHg, p=0.041; night-DBP: 72.8±4.9mmHg, p=0.037) and controls (day-DBP: 70.6±7.2mmHg, p=0.022; night-DBP: 71.9±7.5mmHg, p=0.036). ALSFRS-R score was positively correlated with SDNN (ρ=0.633, p=0.008) and SDANN (ρ=0.610, p=0.012) in ALS patients.

Conclusions:

In the early stages of ALS, patients had similar cardiac autonomic activity and sympathetic vascular control compared with controls. Notably, cardiac autonomic activity decreased and sympathetic vascular control increased in advanced stages of ALS without significant autonomic symptoms.

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Free Communication

NEUROTOXICITY-RELATED TO CAR-T THERAPY: PROPOSED PROTOCOL AND PRELIMINARY DATA FROM BOLOGNA

Session Type
Free Communication
Date
06.10.2021, Wednesday
Session Time
11:30 - 13:00
Room
Free Communication B
Lecture Time
12:10 - 12:20
Presenter
  • Umberto Pensato (Italy)

Abstract

Background and Aims:

Chimeric antigen receptor (CAR) T-cells therapy is an effective treatment for haematological malignancies, yet hampered by cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS)1, a neuropsychiatric syndrome whose biological underpinnings are still unravelled2,3. We present our neurological protocol to manage ICANS and preliminary data.

Methods:

From September 2019 to March 2021, we recruited patients affected by refractory B-cells lymphoma (DLBCL) or multiple myeloma (MM) who received CAR-T therapy. Patients were comprehensively screened (neurological examination, EEG, brain MRI, electroneurography, neuropsychological tests) before infusion and prophylactic anti-epileptic treatment was started. From the day of CAR-T infusion, patients underwent serial neurological/instrumental examinations to monitor development of neurotoxicity. Treatment was based on ICANS severity and consisted in high-doses IV corticosteroids, variably associated with anti-cytokines antibodies.

Results:

Among 31 CAR T-cells infused patients (26 DLBCL; nine females, median age 57±16 years), 84% (n=26) patients developed CRS (median duration 4±2 days). None MM, but 38% (n=10) DLBCL patients developed ICANS (four severe; six preceded by CRS; median duration was 10±8 days). Earliest symptoms included headache, ideo-motor slowing and reduced speech fluency. Dysgraphia followed by a rapid evolution to a severe encephalopathy occurred in three cases. Brain MRI was unremarkable. EEG showed predominant frontal slowing in all cases. One patient developed refractory neurotoxicity with multifocal brain oedema, eventually dying at day 16, whereas other patients recovered.

Conclusions:

We report the first Italian data on ICANS, confirming its high incidence in DLBCL. Frequent clinical/instrumental assessment was essential to recognize early signs of neurotoxicity and promptly start appropriate treatment.

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Free Communication

MULTIFOCAL MOTOR NEUROPATHY: LONG-TERM TREATMENT WITH SUBCUTANEOUS IMMUNOGLOBULIN

Session Type
Free Communication
Date
06.10.2021, Wednesday
Session Time
11:30 - 13:00
Room
Free Communication B
Lecture Time
12:20 - 12:30
Presenter
  • Luca Gentile (Italy)

Abstract

Background and Aims:

Multifocal motor neuropathy (MMN) is a rare disease with a prevalence of less than 1 per 100 000 people. Intravenous immunoglobulin therapy (IVIG), performed for a long-term period, has been demonstrated able to improve the clinical picture of MMN patients, ameliorating motor symptoms and/or preventing disease progression. Treatment with subcutaneous immunoglobulin (SCIg) has been shown to be as effective as IVIG. However, previously published data showed that follow-up of MMN patients in treatment with SCIg lasted no more than 56 months.

Methods:

We report herein the results of a long-term SCIg treatment follow up (up to 96 months) in a group of 8 MMN patients (6M; 2F), previously stabilized with IVIG therapy. Clinical follow-up included the administration of Medical Research Council (MRC) sum-score, the Overall Neuropathy Limitation Scale (ONLS) and the Life Quality Index questionnaire (LQI) at baseline and then every 6 months.

Results:

Once converted to SCIg, patients’ responsiveness was quite good. Strength and motor functions remained stable or, even, improved during this long term follow-up with benefts on walking capability, resistance to physical efforts and ability in hand fine movements.

Conclusions:

These results strengthen the recommendation to use SCIg as an alternative chronic therapy in patients with MMN previously responders to IVIG courses.

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Free Communication

THE DIAGNOSTIC FIELD OF THE AUTONOMIC TESTS IN DIAGNOSIS OF EARLY PARKINSONIAN AND CEREBELLAR MULTIPLE SYSTEM ATROPHY VARIANTS

Session Type
Free Communication
Date
06.10.2021, Wednesday
Session Time
11:30 - 13:00
Room
Free Communication B
Lecture Time
12:30 - 12:40
Presenter
  • Grazia Devigili (Italy)

Abstract

Background and Aims:

Distinguishing the Parkinson's variant of MSA (MSA-P) from Parkinson's disease (PD) is often difficult at the early stage. Similarly, it is difficult to differentiate the cerebellar variant of MSA (MSA-C) from other causes of postural instability and ataxia. We aimed to evaluate the diagnostic yield of cardiovascular and sudomotor autonomic tests in differentiating at early stages the variants of MSA from other diseases.

Methods:

We prospectively enrolled patients with parkinsonisms at an early stage (Hoehn & Yahr stage <2.5) and patients with postural instability and/or ataxia. All performed a battery of cardiovascular tests and the sympathetic skin responses. We analyzed sympathetic indexes during the Valsalva maneuver, and the power spectral analysis of the R-R variability. The final diagnosis of probable MSA-P, PD or MSA-C was performed by three senior investigators, after a mean of 10 months, and was supported by cerebral MRI and myocardial scintigraphy with [123I] metaiodobenzylguanidine (MIBG).

Results:

We enrolled 89 patients with the final diagnosis of MSA-P, 51 with MSA-C, and 81 with PD. Other 75 with parkinsonism received an alternative diagnosis, and 35 received a diagnosis of other late-onset cerebellar ataxic diseases. Cardiovascular reflexes indices were significantly more affected in MSA-P compared to the other groups, including MSA-C. The comparison between groups showed a different pattern of autonomic impairment able to differentiate the distinct variants also in early stages.

Conclusions:

Fully comprehensive autonomic tests are not-invasive and reliable tools for the diagnosis of variants of MSA at early stages.

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Free Communication

AN EMERGING SPECTRUM OF AUTOIMMUNE ASTROCYTHOPATHY: BEYOND ANTI-GFAP ANTIBODIES

Session Type
Free Communication
Date
06.10.2021, Wednesday
Session Time
11:30 - 13:00
Room
Free Communication B
Lecture Time
12:40 - 12:50
Presenter
  • Federica Azzolini (Italy)

Abstract

Background and Aims:

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a steroid-responsive meningo-myelo-encephalitis characterized by anti-GFAP Antibodies and specific morphological findings such as linear perivascular radial gadolinium enhancement on brain-MRI.

Methods:

Two cases of myelo-meningo-encephalitis with some common features are herein described.

Results:

Case-1:45-year-old woman, with acute urinary retention, gait ataxia, mental slowness and headache.
Case-2:45-year-old man, with acute urinary retention, cerebellar ataxia and progressive paraparesis.
The two cases shared:
- a post-infectious onset (febrile urinary tract infection);
- spine MRI with linear leptomeningeal gadolinium enhancement, brain MRI with linear perivascular radial enhancement and FLAIR parenchimal and leptomeningeal hyperintensities (figure).
- CSF: high cellularity (204 and 177 leukocytes/mcl, respectively), high proteins (123 and 177 mg/dl, respectively) and intrathecal oligoclonal IgG production.
- no other CSF/serum investigations suggesting autoimmune-inflammatory, metabolic or infectious disorders.
- response to IV corticosteroids.

Case-1 only, showed CSF and serum anti-GFAP IgG1 antibodies, revealed by home-made cell-based-assay.

anti gfap case series.jpg

Conclusions:

Two cases of myelo-meningo-encephalitis with perivascular linear enhancement have been described, although definite diagnosis of autoimmune anti-GFAP astrocytopathy could be reached in Case-1 only. Nevertheless, anti-GFAP antibodies more likely represent an epiphenomenon accompanying a predominant T-cell mediated process.

Noteworthy, in both cases new MRi findings not typical of other disimmune CNS disease, as leptomeningeal FLAIR hyperintensities in cerebral sulci, brainstem and perivascular spaces, were also observed, suggesting a novel “autoimmune astrocytopathy spectrum disease (AASD)”. Considering that seronegative patients with histological features consistent with anti-GFAP-astrocitopathy have been described, in the second case the same diagnosis could be hypothesized even in absence of anti-GFAP antibodies.

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Free Communication

LIVE Q&A

Session Type
Free Communication
Date
06.10.2021, Wednesday
Session Time
11:30 - 13:00
Room
Free Communication B
Lecture Time
12:50 - 13:00