Migraine

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• Migraine
• Tension headache
• Cluster headache
• Trigeminal Neuralgia

• Secondary headache

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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

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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

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Migraine - epidemiology

• 1/2 of all migraine sufferers - never consulted a
  physician
• prevalence - M6 F15-18
• FgtM 31
• decline after age 50

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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

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Migraine treatment

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

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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

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• Antiepileptic drugs
• Antidepressants
• NSAIDs
• ?-blockers
• Calcium channel blockers

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Tension-type Headache

• Dull, steady pain
• Causes a tight feeling in the scalp or neck
• Depression

• Stress
• Sleep deprivation
• Hunger
• Caffeine withdrawal

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Trigeminal neuralgia

• 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

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Trigeminal neuralgia

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

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TGN Natural History

• Increasing frequency of attacks
• Recurrent attacks more disabling
• Most patients over 60 years except in multiple
  sclerosis

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Trigeminal Neuralgia - Treatment

• Antiepileptics
• Carbamazepine
• Gabapentin
• Phenytoin
• Valproate
• Topamax
• Baclofen

• Surgery for refractory cases
• Surgical neurovascular decompression

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Cluster - headache

• Intensely severe pain
• Unilateral
• Periorbital
• 15 to 180 minutes
• Nausea and vomiting uncommon
• No aura

• Male predominance
• Autonomic hyperactivity
• Lacrimation
• Nasal congestion
• Ptosis

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Cluster - Treatment

• Abortive
• Oxygen
• 5-HT receptor agonists

• Preventative
• Calcium channel blockers
• Lithium
• Steroids
• Valproate
• Antihistamines

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Temporal Arteritis

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

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• 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 354158-165 January 12 2006
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Chronic Daily Headache

• Bilateral, frontal or occipital
• Non-throbbing
• Moderately severe
• Due to overuse of analgesics
• Transformation of migraine

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CDH - Treatment

• Patient understanding
• Remove causative medication
• Antidepressants
• Treatment of withdrawal

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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

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• 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

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I am dizzy

• Vertigo
• Impending faint
• Disequilibrium
• ill - defined

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vertigo

• Peripheral
• Vestibelar neurinitis
• Menieres syndrome
• Central
• Vertebrobasilar ischemia
• Posterior fossa tumors
• Multiple sclerosis

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Impending faint - syncope

• Orthostatic hypotension
• Drugs
• Autonomic insufficiency
• Cardiac
• Vasovagal
• Arrhythmias
• obstructive

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disequelibrium

• Cerebellar disfunction
• Extrapyramidal disease
• Sensory deficits

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Brainstem vascular disease

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

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other

• Anxiety
• depression

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cerebellum

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

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cerebellum

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

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Clinical manifastations

• Symptoms ipsilateral to the damage
• Incoordination
• Intention tremor
• Equilibrium and gait
• Diminished muscle tone

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incoordination

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

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tremor

• Intention tremor
• Wing beating tremor
• Titubation head tremor

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Speech disorders

• Slurring dysarthria
• Scanning dysarthria

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Acute cerebellar ataxia

• Intoxication
• Alcohol, lithium, phenytoin
• Associated with nystagmus / dysarthria/
  confussion
• viral
• Postinfectious

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Acute cerebellar ataxia

• Infarct
• Hemorrhage
• Abscess
• Truma

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subacute

• Brain tumors
• Alcohol nutritional
• Paraneoplastic
• Creutzfeldt jackob disease

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Alcoholic cerebellar degeneration

• Wide base gait
• Legs affected more than arms
• Evolution weeks / months
• Nutritional deficit (thiamine)

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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

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chronic

• Spinocerebellar atrophy (SCA)
• Friedrich ataxia
• Machado joseph disease

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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

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Machado-Joseph (SCA 3)

• people of Portuguese ancestry
• autosomal dominant disorder
• Clinical Ataxia, Extrapyramidal signs Bulging
  eyes

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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

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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

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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)

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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

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Celiac Disease (Gluten-sensitive enteropathy)

• Ataxia
• Peripheral neuropathy
• Myopathies

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Celiac Disease (Gluten-sensitive enteropathy)

• Laboratory
• Antibody testing
• Tissue diagnosis Intestinal biopsy is gold
  standard
• Treatment
• Gluten free diet
• Dietary correction of deficiencies

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Ménières syndrome

• pressure, discomfort, fullness in the ear
• fluctuating hearing loss
• fluctuating tinnitus
• episodic vertigo.

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Tremor

• Tremor is a rhythmic, involuntary back-and-forth
  oscillation of part of the body
• While at rest
• While maintaining a fixed arm position or posture
  (postural tremor)
• With movement or action

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Essential tremor

• Hand tremor is the most common form
• It is typically biphasic and involves agonist and
  antagonist muscles
• in about 10 to 15 of patients, tremor is first
  noted in the dominant hand
• The frequency of the tremor is between 4 to 12 Hz

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conditions in association with essential tremor

• Parkinson's disease (6.1)
• dystonia (6.9)
• myoclonus (1.8).

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ES treatment

• beta-adrenergic blockers
• benzodizepines
• prysoline
• Topamax
• Thalamotomy

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Motor tics

• sudden, rapid, recurrent, involuntary movements,
  particularly of the head and facial area
• Simple motor tics eye blinking or facial
  twitching neck stretching, head jerking, or
  shoulder shrugging
• complex motor tics repetitive touching of others,
  jumping, smelling of objects, hand gesturing, or
  turning in a circle

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vocal tics

• sudden, involuntary, recurrent, often relatively
  loud vocalizations
• simple vocal tics
• Complex vocal tics may involve repeating certain
  phrases(echolalia coprolalia)

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Movement disorders
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• Hypokinetic movement disorders
• Parkinsons disease
• Parkinsons plus syndrome
• Hyperkinetic movement disorders
• Chorea
• Dystonia
• Myoclonus
• Hemiballismus
• Tics
• tremor

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Involuntary movements

• Chorea
• Dystonia
• Tics
• Tremor
• Myoclonus
• Hemiballismus

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Dystonia

• sustained muscle contractions, usually producing
  twisting and repetitive movements
• Focal dystonia
• Generalized dystonia
• Task specific dystonia

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Definition of Chorea

• Greek derivation meaning dancing
• Excessive spontaneous, random, abrupt,
  brief,movements
• Distal predominance
• Flow abruptly from one body part to another

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Motor tics

• sudden, rapid, recurrent, involuntary movements,
  particularly of the head and facial area
• Simple motor tics eye blinking or facial
  twitching neck stretching, head jerking, or
  shoulder shrugging
• complex motor tics repetitive touching of others,
  jumping, smelling of objects, hand gesturing, or
  turning in a circle

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vocal tics

• sudden, involuntary, recurrent, often relatively
  loud vocalizations
• simple vocal tics
• Complex vocal tics may involve repeating certain
  phrases

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Tremor

• Tremor is a rhythmic, involuntary back-and-forth
  oscillation of part of the body
• While at rest
• While maintaining a fixed arm position or posture
  (postural tremor)
• With movement or action

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Essential tremor

• Prevalence 0.4 to 5.6. (FM)
• Family history is positively identified in
  approximately 60 of patients, inheritance -
  autosomal dominant.
• Age of onset has two peaks, early in life and in
  older age group.
• Drinking alcohol often temporarily reduces the
  tremor.
• Patients with essential tremor may have
• Parkinsonism
• Cerebellar dysfunction

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Essential tremor Cognition

• 232 patients with ET and 696 matched controls age
  65 years or older
• cases performed less well on
• test of global cognitive performance MMSE
• frontal executive function - Trail Making Test
• Forgetfulness was reported in 117 (50.4)
  patients with ET vs 300 (43.1) controls (p _
  0.05)

Benito-Leon J et al. Neurology. 200666(1)69-74.
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Essential tremor- Dementia

• 31 (11.4) of 273 ET cases had dementia vs 204
  (6.0) of 3,382 controls
• ET cases with tremor onset after age 65 were 70
  more likely to be demented than controls
• ET cases with tremor onset before age 65 and
  controls were equally likely to be demented

Benito-Leon J, Louis ED, Bermejo-Pareja F
Neurological Disorders in Central Spain Study
Group. Elderly-onset essential tremor is
associated with dementia. Neurology. 2006 May
2366(10)1500-5.
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Personality and Essential tremor

• 55 patients 61 control
• TPQ questionnaire
• Harm avoidance
• Novelty seeking
• Reward dependence
• Significant difference in (p0.005) harm
  avoidance pessimistic, fearful, shy, anxious,
  easily fatigued

Chatterjee A, Jurewicz EC, Applegate LM, Louis
ED. Personality in essential tremor further
evidence of non-motor manifestations of the
disease. J Neurol Neurosurg Psychiatry.
200475(7)958-61.
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Pathology in Essential tremor10 cases

• Six cases (60) had Lewy bodies
• tremor duration 25.2 (6-65)
• Four high Lewy bodies density in the Locus
  Ceruleus

Louis ED, Vonsattel JP, Honig LS, Ross GW, Lyons
KE, Pahwa R. Neuropathologic findings in
essential tremor. Neurology. 2006 Jun
1366(11)1756-9.
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ES treatment

• beta blockers
• benzodizepines
• prysoline
• Topamax
• Thalamotomy