سورة النمل الآية: 79

1. سورة النمل الآية: 79

2. Unusual Headache Syndromes ByDr.AhmedBorai Hassanlecturer of Neurology Sohag university

3. Introduction • Unusual headache syndromes are not as infrequent in clinical practice as was generally believed. • An attempt has been made to classify the headache disorders listed in the ICHD-II in relation to lifetime prevalence as very frequent, frequent, occasional, or rare. • Out of 199 headache types and subtypes, 154 were classified as rare .(i.e three fourths). • Some headache syndromes that were uncommon when first described, such as SUNCT, hemicrania continua, hypnic headache, and reversible cerebral vasoconstriction syndrome, are nowadays widely reported in the literature. (Luiz P,2013)

4. The aim of this presentation is to perform a literature review of the mechanisms, diagnostic criteria, and treatment of the following interesting and unusual headache disorders: • Nummular headache (NH), • The syndrome of transient headache and neurologic deficits with (CSF) lymphocytosis (HaNDL), • Exploding head syndrome (EHS) • Cold stimulus headache (CSH), • Neck tongue syndrome (NTS) • Burning mouth syndrome (BMS) • Alice in Wonderland syndrome (AWS) (Evans,2006)

5. Nummular Headache (Coin-shaped headache) • This is pain of highly variable duration, but often chronic, in a small circumscribed area of the scalp in the absence of any underlying structural lesion. ICHD-3 Diagnostic criteria: A. Continuous or intermittent head pain fulfilling criterion B B. Felt exclusively in an area of the scalp, with all of the following four characteristics: 1. sharply contoured 2. fixed in size and shape 3. Round or elliptical 4. 1–6 cm in diameter

6. This painful area may be localized in any part of the scalp, but is usually in the parietal region. Rarely, NH is bi- or multifocal, each symptomatic area retaining all the characteristics of nummular headache. • Duration is highly variable: in up to 75% of published cases, the disorder has been chronic (present for longer than 3 months) • The affected area commonly shows variable combinations of hypaesthesia, dysaesthesia, paraesthesia and or trophic changes. • Patients are usually more concerned with the possibility of having a serious disease than with the pain or with the sensory disturbances

7. Mechanism • The superficial location and consistent characteristics of the pain, local sensory symptoms, the focal increased sensitivity and the trophic changes confined to the affected area, provide evidence for an epicranial source of the pain. Treatment • NH may respond to botox injection, but the response is not universal. • Gabapentin seems to be the most used preventive treatment for NH. • (Guuerrero AL et al.,2012)

8. The Syndrome of Transient Headache and Neurologic Deficits With CSF Lymphocytosis (HaNDL) (Migrainoussyndrome with CSF pleocytosis) ICHD-3 Diagnostic criteria: • Episodes of migraine-like headache fulfilling criteria B and C; • Both of the following: • Accompanied or shortly preceded by the onset of at least one of the following transient neurological deficits lasting >4 hours: (a) hemiparaesthesia, (b) dysphasia, (c) hemiparesis; • Associated with CSF lymphocytic pleocytosis (>15 white cells/mL), with negative etiological studies.

9. Evidence of causation demonstrated by either or both of the following: • Headache and transient neurological deficits have significantly worsened in temporal relation to the CSF lymphocytosis, or led to its discovery; Also significantly improved in parallel with improvement in the CSF lymphocytosis. • Not better accounted for other diagnosis.

10. Differential Diagnosis

11. Mechanism • Transient neurological deficits were interpreted as the result of local cortical spreading depression phenomenon evidenced by neuroimaging and EEG. • It is possible that during a viral infection antibodies against neuronal or vascular antigens are produced, thus causing an aseptic leptomeningealvasculitis, which, by means of a spreading depression-like phenomenon, would trigger neurological symptomatology Treatment The use of nimodipine and magnesium might benefit the patient as a neuroprotective strategy, in a way similar to the treatment regimen used in cerebral vasospasm.Thus, nimodipine should be used for at least 3 weeks.

12. Exploding Head Syndrome • This syndrome is considered a benign sleep–wake transition disorder of unknown etiology. • The attacks typically present with sudden loud banging noises, like a "bomb explosion" or a "gun shot”. • Patients become concerned and may develop tachycardia, sweating and have difficulty breathing. • Some cases of EHS are followed by sleep paralysis.

13. Mechanism • The basis of this syndrome may be a delay in the reduction of activity in selected areas of the brainstem reticular formation as the patient passes from wakefulness to sleep. • At the onset of sleep, the neuronal activity in the brainstem reticular formation subsides. This, in turn, switches off the motor, sensory, visual, and auditory parts of the cerebral hemispheres; if there is a delay in selected areas of the reticular formation in switching off, then a paroxysm of neuronal activity is manifested by the alarming experiences, such as a loud noise, a flash of light, or a myoclonus. (Evans,2001)

14. Cold stimulus headache (Ice-cream headache; brain-freeze headache) • Headache attributed to ingestion or inhalation of cold stimulus is a common form of headache recognized by the IHS as one triggered by exposure to cold or the ingestion of icy food . • Although it is of frequent occurrence ,headache induced by a cold stimulus is still little studied.

15. ICHD-3 Diagnostic criteria • Throbbing or nonthrobbing pain involving frontal or temporal areas. • Cold stimulus to the palate or pharynx, due to ingestion of icy food or drink and cold air inhalation. • Headache arising within 5 minutes after exposure to the cold stimulus. • Headache disappears within 10 minutes after removal of the cold stimulus. • The headache may, exceptionally, last up to 30 minutes

16. Burning mouth syndrome Intraoral burning sensation for which no medical or dental cause can be found. ICHD-3 Diagnostic criteria: • Oral pain fulfilling criteria B and C; • Recurring daily for >2 hours/day for >3 months; • Pain has both of the following characteristics: (1) burning quality; (2) felt superficially in the oral mucosa. • Oral mucosa is of normal appearance and clinical examination including sensory testing is normal. (Marcelo M et al.,2015)

17. Types • idiopathic BMS : Subjective xerostomia (ie, dry mouth sensation without hyposalivation) • Secondary BMS: may present true xerostomia (complaining of having a "cotton mouth"), due to a lack of saliva production, as in Sjögren's syndrome.

18. Alice in Wonderland Syndrome • AWS may be classified as a migraine variant. • The neurological manifestations of the AWS are in the form of short (seconds to few minutes) episodes of hallucination or illusion, affecting mainly individuals with a history of throbbing headache, particularly migraine with aura. • Derealization,depersonalization , perceptual symptoms (illusory changes in the size, distance, or position of stationary objects) are characteristic. • Children and young adults appear to be more susceptible to this syndrome. (Marcelo M et al.,2015)

19. AWS was also reported to be caused by epilepsy seizure, acute viral infection (Epstein-Barr virus, H1N1 influenza virus), encephalitis, Lyme ,psychoactive substance abuse as cannabis or Cough syrup (dihydrocodeinephosphate) • Guidelines to treat these patients are lacking. Three children with recurrent episodes were treated with flunarizine. • A patient with abdominal migraine and AWS presented a good prophylactic response after using valproate. • Interestingly, topiramate, which used as prophylactic treatment in migraineurs, can also induce AWS attacks.

20. Neck-Tongue Syndrome • In NTS patients, a sudden rotation of the neck induces a sharp pain on the side of the upper neck or occiput, followed immediately by transient ipsilateral numbness of the tongue on the same side. • The pain lasts a few seconds to 1 minute and the paresthesia "seconds" to 5 minutes. • It may present as transient symptoms, which may persist, resolve, and relapse. (Sjaastad O et al.,2006)

21. Mechanism • The proposed mechanism for the site of the pain is related to an irritation of C2and C3 roots with tongue involvement due to afferent impulses traveling from the lingual nerve via the hypoglossal nerve to the C1-C2 roots. • Secondary NTS includes congenital anomalies of cervical spine, Chiari-1 malformation, ankylosing spondylitis, degenerative spondylosis, rheumatoid arthritis, tuberculousatlantoaxialosteoarthritis and cervical acute transverse myelopathy Treatment Treatment commonly used includes cervical collars, manipulation, NSAIDs, muscle relaxants, carbamazepine, gabapentin, amitriptyline, steroids, injections of local anesthetic and nerve resection

22. Conclusion • In the present article, we have reviewed some headache entities whose pathophysiology is still poorly understood. • HaNDLis a very intriguing neurologic emergency, as yet unknown to the physician, who has great difficulty in differentiating it from a stroke. • BMS is a chronic disease with multiple causes and still no effective treatment. • Alice in Wonderland syndrome, for its part, may be interpreted as a psychiatric illness rather than a migraine variant, • The cold stimulus headache is a curious entity in which pain is caused simply by the pleasurable act of eating ice cream. • .

23. Conclusion • NH ,EHS and NTS are also characteristic in their nature. • In conclusion,theunusual headache syndromes as a distinct group of disorders are not as infrequent in clinical practice as was generally believed. • Diagnosing and treating the most common headache disorders are not so difficult. Recognizing some unusual and uncommon headaches, either primary or secondary, may be a challenge for a neurologist. • Hence, a knowledge of these so-called unusual headache syndromes is of critical importance in the day-to-day management of our patients

24. THANK YOU