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5. HEADACHE ATTRIBUTED TO HEAD AND/OR NECK TRAUMA 6. HEADACHE ATTRIBUTED TO CRANIAL OR CERVICAL VASCULAR DISORDER 7. HEADACHE ATTRIBUTED TO NON-VASCULAR INTRACRANIAL DISORDER 7.1. Headache attributed to high cerebrospinal fluid pressure 7.2. Headache attributed to low cerebrospinal fluid pressure7.3. Headache attributed to non-infectious inflammatory disease7.4. Headache attributed to intracranial neoplasm [C00-D48]7.5. Headache attributed to intrathecal injection [G97.8] 7.6. Headache attributed to epileptic seizure [G40.x or G41.x to specify seizure type] 7.7. Headache attributed to Chiari malformation type I (CM1) [Q07.0] 7.8. Syndrome of transient Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL) 7.9. Headache attributed to other non-vascular intracranial disorder Bibliography and references8. HEADACHE ATTRIBUTED TO A SUBSTANCE OR ITS WITHDRAWAL 9. HEADACHE ATTRIBUTED TO INFECTION 10. HEADACHE ATTRIBUTED TO DISORDER OF HOMOEOSTASIS 11. HEADACHE OR FACIAL PAIN ATTRIBUTED TO DISORDER OF CRANIUM, NECK, EYES, EARS, NOSE, SINUSES, TEETH, MOUTH OR OTHER FACIAL OR CRANIAL STRUCTURES 12. HEADACHE ATTRIBUTED TO PSYCHIATRIC DISORDER 

IHS	Diagnosis	ICD-10
7.7	Headache attributed to Chiari malformation type I (CM1) [Q07.0]	G44.82

Diagnostic criteria:

Headache characterised by at least one of the following and fulfilling criterion D:
1. precipitated by cough and/or Valsalva manoeuvre
2. protracted (hours to days) occipital and/or sub-occipital headache
3. associated with symptoms and/or signs of brainstem, cerbellar and/or cervical cord dysfunction
Cerebellar tonsillar herniation as defined by one of the following on craniocervical MRI:
1. ≥5 mm caudal descent of the cerebellar tonsils
2. ≥3 mm caudal descent of the cerebellar tonsils plus at least one of the following indicators of crowding of the subarachnoid space in the area of the craniocervical junction:
  1. compression of the CSF spaces posterior and lateral to the cerebellum
  2. reduced height of the supraocciput
  3. increased slope of the tentorium
  4. kinking of the medulla oblongata
Evidence of posterior fossa dysfunction, based on at least two of the following:
1. otoneurological symptoms and/or signs (eg, dizziness, dysequilibrium, sensations of alteration in ear pressure, hypacusia or hyperacusia, vertigo, down-beat nystagmus, oscillopsia)
2. transient visual symptoms (spark photopsias, visual blurring, diplopia or transient visual field deficits)
3. demonstration of clinical signs relevant to cervical cord, brainstem or lower cranial nerves or of ataxia or dysmetria
Headache resolves within 3 months after successful treatment of the Chiari malformation

Comments:

Headache is often descriptively similar to primary cough headache with the exception of possibly longer duration (minutes rather than seconds).

Headache is the most common symptom of Chiari malformation type I (CM1), but patients may also have localised vestibulo-ocular (74% of cases), lower cranial nerve, brainstem, cerebellar (50%) and/or spinal cord dysfunction suggestive of syringomyelia (66%). Although no specific criteria currently exist to characterise headache attributed to CM1, rigid adherence to the clinical and radiological criteria described above is recommended prior to surgical intervention. However, these criteria require validation and will inevitably be altered in future revisions of The International Classification of Headache Disorders. Prospective studies with long-term surgical outcome are needed.

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