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Dolore in corso di Sindrome di
Ehlers Danlos – Variante ipermobile
A Truini
Department of Neurology and Psychiatry, Sapienza University, Rome

Disclosure: No conflict of interest to declare
Anatomia e Fisiologia del
Sistema Nocicettivo
Five types of pain

•
•
•
•
•

Nociceptive
Neuropathic
Mixed
Psychogenic
Idiopathic/Putative/Dysfucntional
Five types of pain

•
•
•
•
•

Nociceptive
Neuropathic
Mixed
Psychogenic
Idiopathic/Putative
Nociceptive pain
Five types of pain

•
•
•
•
•

Nociceptive
Neuropathic
Mixed
Psychogenic
Idiopathic/Putative/Dysfucntional
Neuropathic pain

Neuropathic pain is pain arising as a direct consequence of a lesion or
disease affecting the somatosensory system
Treede et al., Neurology 2008
Clinical diagnosis
Peroneal
mononeuropathy

L5- radiculopathy

Saphenous
mononeuropathy

L3- radiculopathy

Brachial plexopathy

Polyneuropathy

Myelopathy

Amiotrophy

Herpes Zoster

Musculoskeletal pain
Five types of pain

•
•
•
•
•

Nociceptive
Neuropathic
Mixed
Psychogenic
Idiopathic/Putative/Dysfunctional
Che dolore hanno le persone con ED?
Può essere un dolore neuropatico?
Our preliminary findings are apparently in contrast with common sense, that is, in
EDS, pain is directly linked to primary joint damage and, consequently, is mainly
nociceptive in origin. In fact, our results suggest that pain is frequently neuropathic.
This implies that the pathophysiology of pain in EDS may be more complex than
expected, and that musculoskeletal involvement cannot explain the entire spectrum
of pain. Accordingly, it is likely that pain symptoms in EDS are the result of different
pain-triggering mechanisms.
Camerota et al., J Pain Symptom Manag 2010
Indagini strumentali
Quantitative Sensory Testing
(Aδ- and
C fibres mediated)

C-fibres
Aδ-fibres

Aβ-fibres

NCS (Aβ-fibre mediated)

Laser evoked potentials (Aδ-fibre mediated)
Questionari
DN4 Questionnaire

Fibromyalgia Rapid Screening Tool (FiRST)

Does my pain have one or more of the following
characteristics?

Items

1. Burning

Y

N

2. Painful cold

Y

N

3. Electric shocks

Y

N

Is the pain associated with one or more of the following
symptoms in the same area?
Y

N

5. Pins and needles

Y

N

6. Numbness

Y

N

7. Itching

Y

N

8. Hypoesthesia to touch

Y

N

9. Hypoesthesia to prick

Y

N

Is the pain located in an area where the physical
examination reveals one or more of the following?

In the painful area, can the pain caused or increased by
Y

My pain is accompanied by a continuous and very
unpleasant general fatigue
My pain feels like burns, electric shocks or cramps

4. Tingiling

10. Brushing

I have pain all over my body

N

My pain is accompanied by other unusual sensations
throughout my body, such as pins and needles, tingling
or numbness
My pain is accompanied by other health problems such
as digestive problems, urinary problems, headaches or
restless legs
My pain has a significant impact on my life, particularly
on my sleep and my ability to concentrate, making me
feel slower generally

Y/N
Risultati
In tutti i soggetti con sindrome di Ehlers Danlos studiati DN4, visita clinica neurologica, studio di
conduzione nervosa, dei potenziali evocati laser e delle soglie percettive termodolorifiche sono
risultati normali

Distribuzione topografica dolore

Fibromyalgia Rapid Screening Tool (FiRST)
Items

Y/N
(%)

I have pain all over my body

80

My pain is accompanied by a continuous and very
unpleasant general fatigue

90

My pain feels like burns, electric shocks or cramps

90

My pain is accompanied by other unusual sensations
throughout my body, such as pins and needles, tingling
or numbness

90

My pain is accompanied by other health problems such
as digestive problems, urinary problems, headaches or
restless legs

90

My pain has a significant impact on my life, particularly
on my sleep and my ability to concentrate, making me
feel slower generally

90
Discussione
Discussione
Infiammazione cronica

Persistente attivazione del sistema dolorifico

Sviluppo della sensitizzazione centrale
Functional connectivity of PAG in fibromyalgia
Controls

Patients

Controls > Patients

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Truni

  • 1. Dolore in corso di Sindrome di Ehlers Danlos – Variante ipermobile A Truini Department of Neurology and Psychiatry, Sapienza University, Rome Disclosure: No conflict of interest to declare
  • 2. Anatomia e Fisiologia del Sistema Nocicettivo
  • 3. Five types of pain • • • • • Nociceptive Neuropathic Mixed Psychogenic Idiopathic/Putative/Dysfucntional
  • 4. Five types of pain • • • • • Nociceptive Neuropathic Mixed Psychogenic Idiopathic/Putative
  • 6. Five types of pain • • • • • Nociceptive Neuropathic Mixed Psychogenic Idiopathic/Putative/Dysfucntional
  • 7. Neuropathic pain Neuropathic pain is pain arising as a direct consequence of a lesion or disease affecting the somatosensory system Treede et al., Neurology 2008
  • 8. Clinical diagnosis Peroneal mononeuropathy L5- radiculopathy Saphenous mononeuropathy L3- radiculopathy Brachial plexopathy Polyneuropathy Myelopathy Amiotrophy Herpes Zoster Musculoskeletal pain
  • 9. Five types of pain • • • • • Nociceptive Neuropathic Mixed Psychogenic Idiopathic/Putative/Dysfunctional
  • 10. Che dolore hanno le persone con ED? Può essere un dolore neuropatico? Our preliminary findings are apparently in contrast with common sense, that is, in EDS, pain is directly linked to primary joint damage and, consequently, is mainly nociceptive in origin. In fact, our results suggest that pain is frequently neuropathic. This implies that the pathophysiology of pain in EDS may be more complex than expected, and that musculoskeletal involvement cannot explain the entire spectrum of pain. Accordingly, it is likely that pain symptoms in EDS are the result of different pain-triggering mechanisms. Camerota et al., J Pain Symptom Manag 2010
  • 11. Indagini strumentali Quantitative Sensory Testing (Aδ- and C fibres mediated) C-fibres Aδ-fibres Aβ-fibres NCS (Aβ-fibre mediated) Laser evoked potentials (Aδ-fibre mediated)
  • 12. Questionari DN4 Questionnaire Fibromyalgia Rapid Screening Tool (FiRST) Does my pain have one or more of the following characteristics? Items 1. Burning Y N 2. Painful cold Y N 3. Electric shocks Y N Is the pain associated with one or more of the following symptoms in the same area? Y N 5. Pins and needles Y N 6. Numbness Y N 7. Itching Y N 8. Hypoesthesia to touch Y N 9. Hypoesthesia to prick Y N Is the pain located in an area where the physical examination reveals one or more of the following? In the painful area, can the pain caused or increased by Y My pain is accompanied by a continuous and very unpleasant general fatigue My pain feels like burns, electric shocks or cramps 4. Tingiling 10. Brushing I have pain all over my body N My pain is accompanied by other unusual sensations throughout my body, such as pins and needles, tingling or numbness My pain is accompanied by other health problems such as digestive problems, urinary problems, headaches or restless legs My pain has a significant impact on my life, particularly on my sleep and my ability to concentrate, making me feel slower generally Y/N
  • 13. Risultati In tutti i soggetti con sindrome di Ehlers Danlos studiati DN4, visita clinica neurologica, studio di conduzione nervosa, dei potenziali evocati laser e delle soglie percettive termodolorifiche sono risultati normali Distribuzione topografica dolore Fibromyalgia Rapid Screening Tool (FiRST) Items Y/N (%) I have pain all over my body 80 My pain is accompanied by a continuous and very unpleasant general fatigue 90 My pain feels like burns, electric shocks or cramps 90 My pain is accompanied by other unusual sensations throughout my body, such as pins and needles, tingling or numbness 90 My pain is accompanied by other health problems such as digestive problems, urinary problems, headaches or restless legs 90 My pain has a significant impact on my life, particularly on my sleep and my ability to concentrate, making me feel slower generally 90
  • 15. Discussione Infiammazione cronica Persistente attivazione del sistema dolorifico Sviluppo della sensitizzazione centrale
  • 16. Functional connectivity of PAG in fibromyalgia Controls Patients Controls > Patients