NEWS - YEAR 2020

JUNE 2020

27 June 2020 
ANOTHER GREAT STUDY ABOUT BEHAVIORAL PROBLEMS IN AHC PUBLISHED BY THE DUKE GROUP

In the article (Wallace K, Uchitel J, Prange L, Jasien J, Bonner M, D'Alli R, Maslow G, Mikati MA. Characterization of Severe and Extreme Behavioral Problems in Patients with Alternating Hemiplegia of Childhood. Paediatr Neurol. 2020 Jun 27. doi: 10.1016/j.pediatrneurol.2020.06.012) published by the group of the AHC Clinic at the Duke University Medical Center, directed by Professor Mohamad Mikati, they analyzed 39 consecutive AHC patients for occurrence of behavioral problems.

Findings

  • Six (15%) patients had extreme problems defined as those present in major risk of injury to themselves or to others
  • Ten (26%) had severe problems which presented minor risk of injury to themselves or to others
  • Five (13%) had moderate problems defined as requiring intervention but presenting no risks of injury
  • Two (5%)  had mild problems defined as not requiring interventions
  • Sixteen (41%) had no behavior problems

Triggers noted in many patients 

  • peer-provocation
  • low frustration tolerance
  • limits set by others  
  • sleep disruption
  • Some medications

Reversible psychotic symptoms occurred in 2 of the 39 patients

  • In one, the trigger was trihexyphenidyl
  • In the above patient infection was a trigger on another occasion
  • In another patient, the trigger was the medication sertraline.

Management

  • Diagnosis and therapy of coexisting neuropsychiatric diagnoses like anxiety, depression or attention deficit disorder
  • Involvement of a multidisciplinary team including mental-health professionals neurology and developmental Pediatrics
  • Medications targeting the patient’s specific problem and coexisting neuropsychiatric diagnosis and that often alpha-adrenergic agonists and SSRIs.

Conclusions

  • Forty one percent of AHC patients often experience severe/extreme behavioral problems
  • On rare occasions, AHC patient may have medication-triggered psychotic symptoms.
  • These correlate with the known AHC brain pathophysiology.
  • Awareness of these behavioral problems should allow anticipatory guidance and better management of such problems when they occur in some AHC patients

You can read the article at this link

   Article about Behavioural Problems in Alternating Hemiplegia of Childhood    

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NEWS - YEAR 2019

NOVEMBER 2019

30 November 2019 
DATA COLLECTION STARTED FOR THE IAHCRC INTERNATIONAL STUDY OBSERV-AHC

Data collection for the OBSERV-AHC Study (a Study of the IAHCRC Consortium) started last September 1st, in Spain, France, UK and USA, supported by their national patient associations. In Italy, they are still waiting for the approval of their Ethics Committee, but they too are ready to start as soon as they obtain it.
Data will be collected and entered in the IAHCRC-CLOUD Platform by the data-entry clinicians during the neurological visits to the patients organized in collaboration with their supporting patient associations.
The caregivers of the enrolled patients are offered the possibility to record their AHC episodes directly in the IAHCRC-CLOUD Platform, through its Mobile App, so that also their data can be included in the Study.

   Read Prof. Mikati's Article on the Neurology Website    ObservAhcDataCollectionItalyMar2019  
 

Prof. Mohamad Mikati presenting the OBSERV-AHC Study
at the International Symposium on AHC
organized by the Spanish Patient Association AESHA
and the Reference Center for AHC at the San Juan de Deu Hospital
in Barcelona, 9 February 2019

 

  Prsentation of the OBSERV-AHC Study
at the Family Meeting of the Italian Patient Association
in Milan, March 2019

 
 
  ObservAhcDataCollectionSpainSep2019   ObservAhcDataCollectionFranceSep2019  
  Family Meeting for the start of the data collection
for the OBSERV-AHC Study, organized by
the Spanish Patient Association in Barcelona, September 2019
 
  Family Meeting for the start of the data collection
for the OBSERV-AHC Study, organized by
the French Patient Association in Lyon, October 2019
 
 
  ObservAhcMeetingReykjavikOct2019    IahcrcCloudEventCalendarMobileBrowser02  
  Working meeting organized on the occasion of the
8th Symposium on ATP1A3 in Disease (Reykjavik, Iceland, 3 October 2019),
about the start of the data collection for the OBSERV-AHC Study.
Standing, Prof. Mohamad Mikati, Scientific Coordinator of the Study;
seated, the data-entry clinicians of the Centers participating in the Study,
in France, Spain, Italy, UK and the United States.
  The Mobile App through which a caretaker
can record the patient's events in the IAHCRC-CLOUD Platform

 

 
 

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NEWS - YEARS 2014 to 2018

SEPTEMBER 2018

17 September 2018 
PRESS CONFERENCE FOR THE PRESENTATION OF THE PROJECT IAHCRC-CLOUD PLATFORM
a service for the international research on AHC and the ATP1A3 diseases

Tuesday October 30 2018, 3.30pm
I.E.ME.S.T. Institute www.iemest.eu
via Michele Miraglia, 20 – Palermo, Italy

Palermo, 17 September 2018
The President of the Euro-Mediterranean Institute of Science and Technology (I.E.ME.S.T.) Bartolomeo Sammartino communicates that on Tuesday October 30 2018, at 3.30pm, the press conference will take place at the headquarters of the Institute (via Michele Miraglia, 20 – IT Palermo), for the presentation of the Project “IAHCRC-CLOUD Platform”, aimed at establishing an international platform for the data collection and sharing serving the collaborative studies of the IAHCRC Consortium.
The technical and ethical aspects will be presented, as well as the innovative features of the IAHCRC-CLOUD Platform, making it a Second Generation Rare Disease Registry, implementing the principles and methods of patient engagement and platform cooperativism.
The current studies of the IAHCRC Consortium using the Platform will also be presented; among them, the longitudinal Study OBSERV-AHC, coordinated by the Division of Pediatric Neurology - AHC and Related Disorders Program of the Duke University in Durham, NC USA. In addition, some anticipations will be given on future developments already foreseen for the Platform, to make it an open, scalable and adaptable tool for any other rare disease.
Please confirm your participation via email to This email address is being protected from spambots. You need JavaScript enabled to view it. by Friday October 26 2018.

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IAHCRC - International Consortium for the Research on Alternating Hemiplegia of Childhood and other ATP1A3 related diseases