תסמונות נוירולוגיות שכיחות

1. תסמונות נוירולוגיות שכיחות דר' מרימס דורון המרכז הרפואי שהם

2. כאב ראש • סחרחרת/ חוסר יציבות • רעד ותנועות לא רצוניות אחרות.

3. Migraine Tension headache Cluster headache Trigeminal Neuralgia Secondary headache

5. Migraine Co-morbidities • Epilepsy • Stroke - • Depression • anxiety disorders • Irritable bowel syndrome; colitis • mitral valve prolapse • Allergies (children) • MRI - an increased prevalence of white matter lesions

6. Migraine – classification • Migraine without aura • Migraine with aura • Migraine with typical aura • Typical aura without headache • Familial hemiplegic migraine • Sporadic hemiplegic migraine • Basilar-type migraine • Childhood periodic syndromes • Retinal migraine • Complications of migraine • Probable migraine

7. Migraine - epidemiology • 1/2 of all migraine sufferers - never consulted a physician • prevalence - M=6% ; F=15%-18% • F>M 3:1 • decline after age 50

8. Hormones and Migraine • Up to 70% of women with migraine report attacks at the time of menses • Menstrual migraine triggered by fall in estrogen and progesterone • 60 - 70% of migraineurs improve during pregnancy

9. Migraine treatment • Changing lifestyle • eating on time • avoiding foods that trigger migraine • good sleeping habits • Medications for acute attack • Prophylaxis treatment

10. Triptans • treatment of choice • efficacy of the triptans is judged by headache relief within 2 hours • Vasoconstrictors should not be used in patients with • Coronary artery disease • Angina • Uncontrolled hypertension • Peripheral vascular insufficiency • History of stroke • Which triptan to choose? • Effectiveness • Recurrence of headache

11. Antiepileptic drugs • Antidepressants • NSAIDs • -blockers • Calcium channel blockers

12. Dull, steady pain Causes a tight feeling in the scalp or neck Depression Stress Sleep deprivation Hunger Caffeine withdrawal Tension-type Headache

13. Trigeminalneuralgia • Location of pain : V2 and V3 • V1 involved in less than 5% of cases • Quality of pain: needles • Excruciating pain lasting for a few seconds • Followed by unpleasant aching pain

14. Trigeminalneuralgia • Pain is not continual • Triggering factors: chewing, smiling, yawning (motor), hot and cold fluids, brushing teeth, • Diurnal variation - attacks at night rare

15. TGN Natural History • Increasing frequency of attacks • Recurrent attacks more disabling • Most patients over 60 years except in multiple sclerosis

16. Antiepileptics Carbamazepine Gabapentin Phenytoin Valproate Topamax Baclofen Surgery for refractory cases Surgical neurovascular decompression Trigeminal Neuralgia - Treatment

17. Intensely severe pain Unilateral Periorbital 15 to 180 minutes Nausea and vomiting uncommon No aura Male predominance Autonomic hyperactivity Lacrimation Nasal congestion Ptosis Cluster - headache

18. Abortive Oxygen 5-HT receptor agonists Preventative Calcium channel blockers Lithium Steroids Valproate Antihistamines Cluster - Treatment

19. Temporal Arteritis • Moderate to severe, unilateral pain • Patients over 65 • ESR elevated • Biopsy for definitive diagnosis • Treat with steroids • Untreated complicated by vision loss

20. A 36-year-old woman with a long history of catamenial migraines had had a headache almost every day during the previous year. The background headache was mild but became severe and incapacitating at least twice a week, interfering with work and sleep. She took six to eight tablets containing a combination of aspirin, acetaminophen, and caffeine per day, with minimal relief. She had no fever, weight loss, diplopia, or tinnitus. Her headaches were not exacerbated by a Valsalva maneuver or positional change. Her physical examination was normal. How should she be evaluated and treated? NEJM Volume 354:158-165 January 12 2006

21. Chronic Daily Headache • Bilateral, frontal or occipital • Non-throbbing • Moderately severe • Due to overuse of analgesics • Transformation of migraine

22. CDH - Treatment • Patient understanding • Remove causative medication • Antidepressants • Treatment of withdrawal

23. headache-" Red flags" • sudden onset • onset after 50 • increased frequency or severity • headache with concomitant systemic illness • focal neurologic signs or symptoms • papilledema • Recent head trauma

24. hypertension(basal ganglia, subcortical white matter, cerebellum, thalamus and pons) • hemorrhagic transformation of infarction • hemorrhage from metastatic disease (thyroid, renal cell carcinoma, melanoma, and lung cancer) • In younger patients, vascular malformations • amyloid angiopathy

25. I am dizzy • Vertigo • Impending faint • Disequilibrium • ill - defined

26. vertigo • Peripheral • Vestibelar neurinitis • Meniere’s syndrome • Central • Vertebrobasilar ischemia • Posterior fossa tumors • Multiple sclerosis

27. Impending faint - syncope • Orthostatic hypotension • Drugs • Autonomic insufficiency • Cardiac • Vasovagal • Arrhythmias • obstructive

28. disequelibrium • Cerebellar disfunction • Extrapyramidal disease • Sensory deficits

29. Brainstem vascular disease • The most common symptom of brainstem vascular disease • Look for other brainstem symptoms • Drug induced Parkinsonism d/t prochlorperazine

30. other • Anxiety • depression

31. cerebellum • Muscle tone • Coordination of movements • Eapeciialy skilled coordinated • Control of posture and gait

32. cerebellum • Flocculonodular lobe • Vestibulocerebellum / equilibrium • Anterior lobe • Posterior lobe • spinocerebellum / posture and muscle tone

33. Clinical manifastations • Symptoms ipsilateral to the damage • Incoordination • Intention tremor • Equilibrium and gait • Diminished muscle tone

34. incoordination • Speed of initiating movement is slowed • Dysmetria • Dysdiadochokinesis • The velocity and force are not checked in normal manner

35. tremor • Intention tremor • Wing beating tremor • Titubation – head tremor

36. Speech disorders • Slurring dysarthria • Scanning dysarthria

37. Acute cerebellar ataxia • Intoxication • Alcohol, lithium, phenytoin • Associated with nystagmus / dysarthria/ confussion • viral • Postinfectious

38. Acute cerebellar ataxia • Infarct • Hemorrhage • Abscess • Truma

39. subacute • Brain tumors • Alcohol nutritional • Paraneoplastic • Creutzfeldt jackob disease

40. Alcoholic cerebellar degeneration • Wide base gait • Legs affected more than arms • Evolution – weeks / months • Nutritional deficit (thiamine)

41. paraneoplastic • Development : days – weeks • Adenocarcinoma of ovary and lung • Antibodies to Purkinje cells – anti Yo • Small cell lung carcinoma – anti Hu • symptoms precede the detection of the tumours

42. chronic • Spinocerebellar atrophy (SCA) • Friedrich ataxia • Machado joseph disease

43. Spinocerebellar Ataxia (SCA) • Dominant SCA syndromes have many overlapping signs: Difficult to distinguish on clinical grounds • Common features to all: gait ataxia dysarthria • Features in some ataxias • Extrapyramidal symptoms • Pripheral nerve / motor neuron • Intellectual deterioration :

44. Machado-Joseph (SCA 3) • people of Portuguese ancestry • autosomal dominant disorder • Clinical: Ataxia, Extrapyramidal signs & Bulging eyes

45. Machado-Joseph (SCA 3) • Chromosome 14q32.1; Dominant • CAG trinucleotide repeats • Normal range: 12 to 40 • Disease: 51 to 86 • More CAG repeats • Earlier onset • More rapid progression • More intergenerational instability of the CAG repeat length with (anticipation) • Male parent

46. Machado-Joseph (SCA 3) • Frequency: Common dominantly inherited ataxia; 23% to 36% of SCA • Between families • Variable disorders • Most syndromes include cerebellar signs • Within families: • Tends to be consistent syndrome • Anticipation: Common • Ophthalmoparesis • Homozygotes have more severe syndrome than heterozygotes

47. Expansion of trinucleotide repetitions • Increased number of triplets Þ disease • More repetitions, more severe disease • Increase of repetition number from one generation to the next (anticipation)

48. Celiac Disease (Gluten-sensitive enteropathy) • Clinical features • Hematologic • Anemia: Iron or Folate deficiency • Hemorrhage: Vitamin K deficiency • GI • Clinical: Diarrhea (50%); Weight loss • Pathology: Enteropathy of the proximal small bowel

49. Celiac Disease (Gluten-sensitive enteropathy) • Ataxia • Peripheral neuropathy • Myopathies

50. Celiac Disease (Gluten-sensitive enteropathy) • Laboratory • Antibody testing • Tissue diagnosis: Intestinal biopsy is gold standard • Treatment • Gluten free diet • Dietary correction of deficiencies