1.1. Parkinson disease

Transcription

1 1.TREMOR

2 1.1. Parkinson disease

3 Parkinson Disease Progressive disorder: tremor, rigidity, and slowness of movements Neuronal loss of the substantia nigra Non motor features (dementia and dysautonomia), in advanced stages of the disease Orthostatic hypotension Bradykinesia Erectile dysfunction L-Dopa lack of response Evolution of the disease (years)

4 Imaging in Parkinson Disease Although neuroimaging is usually nondiagnostic in the evaluation of PD several modalities are used in its management. TC is performed to exclude specific structural abnormalities (hydrocephalus, tumor or lacunar infarcts) MR Imaging: Narrowing or disappearance of pars compacta of substantia nigra on T2 WI MR is essential in the planning of subthalamic nucleus surgery Role of transcranial ultrasound

5 Parkinson disease. Role of CT Rule out secondary causes some of them potentially reversible Drugs Wilson s disease Essential tremor Vascular parkinsonism Normal pressure hidrocephalus Trauma

6 Parkinson disease: MR Imaging Parkinson disease. Role of MR imaging * * Axial T2 WI at midbrain level shows narrowing of the substantia nigra, pars compacta (arrow). Note the red nucleus hypointensity (*) Axial T2 WI shows hypointensity within the lentiform nuclei and mild cerebral atrophy T1 3D TFE. Coronal MPR showing bilateral gliosis due to subthalamic nucleus stimulation

7 Parkinson disease. Role of Transcranial ultrasound TCUS: Substancia nigra hiperechogenicity Hiperechogenicity of the substancia nigra: Parkinson s disease 10% healthy subjects Essential tremor Lewy dementia Corticobasal degeneration. Non hiperechogenicity of the substantia nigra: 80% healthy controls, Multiple system atrophy Progressive supranuclear palsy

8 1.2. Atypical Parkinsonian Syndromes EPIDEMIO LOGY Progressive supranuclear palsy Multiple system atrophy Corticobasal degeneration P.D. P.S.P. M.S.A. Others

9 Importance of correct diagnosis Prognosis: the prognosis for APS is worse than PD Therapeutic: APS patients respond poorly to L-Dopa Secondary effects of PD therapy: dyskinesias Atypical parkinsonism patients should not be managed by surgical means Investigation: patients with APS should not be enrolled in PD studies Is the patient in the right bowl?

10 1.2.1.Progressive supranuclear palsy

11 Progressive supranuclear palsy Also known as Steele Richardson Olszewski syndrome Vertical supranuclear palsy with downward gaze abnormalities Postural instability with unexplained falls Pseudobulbar palsy with associated dysarthria and dysphagia Frontal lobe-like dementia syndrome Course relentlessly progressive (death six years after onset)

12 Progressive supranuclear palsy Atrophy of midbrain, concave profile of the midbrain forming the floor of the third ventricle: hummingbird or penguin sign Axial images show abnormal concavity of the lateral margins of midbrain tegmentum: morning glory sign Midsagital images: Midbrain axial diameter < 14mm midbrain area <70mm2 (50% normal) midbrain to pons ratio <0,15 Thinning of the superior colliculus

13 Progressive supranuclear palsy * Midbrain tegmetum atrophy: hummingbird or penguin sign

14 Progressive supranuclear palsy Mild PSP Healthy subject Morning glory flower for comparison Advanced PSP Abnormal concavity of the lateral margins of the midbrain tegmentum: mornign glory sign

15 Progressive supranuclear palsy Significantly smaller ratio of midbrain-pons area <0,15 ó <0,20

16 Progressive supranuclear palsy Thinning of the superior colliculus Healthy subject PSP

17 1.2.2.Multiple system atrophy

18 Multiple system atrophy Progressive, idiopathic, neurodegenerative process Various degrees of parkisonism, autonomic failure, cerebellar dysfunction, and pyramidal signs Glial cytoplasmic inclusions (alpha sinuclein) Insufficent or transient response to L- Dopa Orthostatic hypotension Erectile dysfunction Bradykinesia Lack of response to L-Dopa 5 10 Evolution of the disease (years)

19 Multiple system atrophy. Subtypes PARKINSONIAN MSA-P (80%): Asymmetrical tremor, bradykinesia, rigidity and postural instability Former striatronigral degeneration CEREBELLAR MSA-C (20%): Gait and limb ataxia, ataxic dysarthria and sustained gaze evoked nystagmus Former olivoponto cerebellar atrophy MSA-P 80% MSA- C 20% Autonomic findings AUTONOMIC DISFUNCTION : Urinary dysfunction, orthostatic hypotension, erectile dysfunction, urinary incontinence Former Shy Drager Syndrome

20 MSA-P: MSA-parkinsonian - Hypointensity of the putamen in T2 weighted images that releccts iron accumulation due to striatonigral degeneration. (The hypointensity is equal to or more marked than normally seen in the pallidum, where iron accumulates during life) - A thin lateral rim of hyperintensity in the putamen relates to secondary gliosis

21 MSA-parkinsonian AMS-p. Axial T2 WI. Prominent hypointensity in the putamen and pallidus. Note also the peripheral hyperintese rim due to reactive gliosis.

22 MSA- cerebellar MSA-C: Atrophy of the brain stem and cerebellum: on sagittal iamges atrophy of the pons and medulla with flat ventral surface of the pons Atrophy of the cerebellar vermis, middle cerebellar peduncles and hemispheres Signal changes in the transverse pontine fibers ( hot cross bun sign), middle cerebellar peduncles and cerebellum

23 MSA- cerebellar Cerebellar atrophy and high signal in middle cerebellar peduncles AMS Atrophy of the pons and medulla with flat ventral surface of the pons Cruciform shape in the pons, hot cross bun sign

24 1.2.3.Corticobasal degeneration

25 Corticobasal degeneration Progressive neurodegenerative disease Presents with congitive dysfunction, asymetrical parkinson Severe focal symmetric cortical atrophy Perrolandic (posterior frontal, parietal cortex) Relative sparing of temporal, occipital regions

26 Corticobasal degeneration A B C D Axial T2 (A), axial FFE T1(B), Coronal and sagittal MPR reconstructions. (C,D) Volumen rendering (E). Asimetric volume loss involving precentral and postcental giri, more strking in parasagital regions. E

27 1.3. Hereditary parkinsonism

28 Neurodegeneration with brain iron accumulation (PKAN-2) Former Hallervorden-Spatz Autosomal recessive, mutation in the patothene kinase 2 gene (PKAN-2) Imaging: hyperintensity within a region of hypointensity in the medial globus pallidus, eye of the tiger Eye of the tiger sign

29 Wilson disease Hepatolenticular degeneration Autosomal recessive inherited copper metabolism disorder Imaging: Hyperintense signal on T2 WI, sometimes with a central core of hypointensity in the basal ganglia Face of the giant panda sign

30 1.4. Secondary parkinsonism EPIDE MIOLO GY P.D. P.S.P. M.S.A. Others Toxins (carbon monoxide, methanol) Metabolic (chronic liver failure, Wilson's disease extrapontine myelinolysis) Infections (prion disease, HIV/AIDS) Head trauma (boxing) Structural brain lesions (hydrocephalus, chronic subdural hematoma, tumor) Small vessel disease ("vascular parkinsonism") Drugs (antipsychotic agents)

31 Carbon monoxide poisoning Most common cause of accidental poisoning in Europe and North America Imaging: Globi pallidi hyperintensity on T2 WI due to necrosis Associated abnormalities: CO induced parkinsonism Axial T2 WI shows hyperintense lesion within globi pallidi

32 Methanol poisoning Axial T2 WI shows bilateral hyperintensity of the putamen and pallidal nuclei

33 Hepatic (Manganic) encephalopaty Associated with acute liver failure, portal systemic bypass and liver cirrhosis Functional, potentially reversible due to manganese accumulation A B Sagittal T1WI MR (A) and parasagittal T1WI MR (B) show hyperintense signal within lentiform nucleus extending into midbrain and pitutitary gland

34 Hepatic (Manganic) encephalopaty Coronal T2 WI. High signal along hemispheric white matter in and around corticospinal tract. DWI. High mean diffusivity In hemispheric white matter

35 Rapidily progressing, fatal, transmissible dementig disorder caused by a prion T2 WI: hyperintensity of basal ganglia, thalamus and cerebral cortex. DWI: hyperintense changes in striatum and cerebral cortex A C Creutzfeldt Jacob disease B D Axial FLAIR : bilateral high signal intensity in the caudate nuclei, putamina and thalami (A) and in the cingulate girus (B) Axial DWI (C,D) show hyperintense signal consistent with restricted diffusion in caudate nuclei, putamina, thalami (C) and gyriform hyperintense areas in cingulate cortex. Pulvinar and hockey stick sign

36 1.5. Essential tremor

37 Essential tremor Classical essential tremor consists of a bilateral, visible, persistent, and largely symmetrical postural and/or kinetic tremor involving the hands and forearms Radiologist must know that routine CT and MR have not shown any consistent abnormalities

38 1.6. Palatal tremor

39 Palatal tremor In patients with palatal tremor the MR imaging finding of inferior olivary nucleus hypertrophy suggests symptomatic palatal tremor Bilateral olivary hipertrophy

40 1.7. Fragile X tremor ataxia syndrome (FTAX)

41 Fragile X tremor ataxia syndrome (FTAX) Genetic disorder affecting mostly men and causing tremor, ataxia, and dementia Increased T2 signal in the white matte of middle cerebellar peduncles ( the MCP sign) The middle cerebellar peduncles sign

42 2.CHOREA

43 Huntington disease Autosomal dominant Clinical triad Chorea Dementia Behavioral changes Imaging: Atrophy of caudate nucleus that leads to frontal horns enlargement Diffuse cerebral atrophy

44 Chorea-ballismus in acute non-ketotic hyperglycaemia Unilateral T1 high signal intensity, low on T2 WI and restricted DWI Reversible after treatment

45 3.HEMIFACIAL SPASM

46 Hemifacial Spasm Unilateral involuntary facial spasms Begins with orbicularis oculi spasms Tonic-clonic but becomes constant over time

47 Hemifacial Spasm Common Vascular loop syndrome, CPA-IAC Vacular loop compressing facial nerve at its root exit zone within CPA cistern Causing hemifacial spasm Hemifacial spasm offending vessel: AICA (50%), PICA (30%), VA (10%), Vein (5%) Less Common Epidermoid cyst, CPA-IAC Meningioma, CPA-IAC Aneurysm, CPA-IAC Schwannoma facial nerve, CPA-IAC Perineural tumor CN7, parotid space Hemangioma facial nerve Rare but important Multiple sclerosis Ateriovenous malformation Hemangioma IAC

48 Hemifacial spasm Axial T2 WI. Right PICA loop Axial T2 WI. Right vestibular schwannoma FLAIR and DWI. Acute (superior) and chronic (inferior) pontine infarcts

49 Can you name the signs and the diseases?

50 Hummingbird Eye of the tiger sign PKAN-2 MSA-C hot cross bun PSP Hockey stick Creuztfeldt Jakob Morning glory Giant panda sign Wilson disease