This document discusses atypical parkinsonism (AP), which accounts for 15-20% of all cases of parkinsonism. It begins by classifying AP into primary, multisystem degenerations, hereditodegenerative, and secondary types. It then focuses on the major AP syndromes - Progressive Supranuclear Palsy (PSP), Multiple System Atrophy (MSA), and Corticobasal Degeneration (CBD). For PSP and MSA, it provides details on clinical presentation, diagnostic criteria, investigations, pathology, and treatment approaches. The document emphasizes the importance of differentiating AP from Parkinson's disease to allow for accurate prognosis and management of patients.

1. ATYPICAL PARKINSONISM
DR.SARATH MENON.R, MD(Med.),DNB(Med.),MNAMS
DM RESIDENT
DEPT. OF NEUROSCIENCES
AIMS,KOCHI

2. OUTLINE
 Classifications
 Red flag signs
 AP- syndromic approach
 Diagnostic criteria
 Phenotypic spectrum
 Atypical AP or mimickers
 Investigations
 Novel biomarkers
 Treatment
 Future trends

3. INTRODUCTION
 Common problem in neurology outpatient
departments
 Wide variety of sporadic / heredodegenerative
syndromes
 Aetiologies vary widely
 80-85% -IPD
 Differentiation from other syndromes
 Very important in prognostication and management

4. AKINETIC RIGID SYNDROMES -
CLASSIFICATION
 Primary (Idiopathic) Parkinsonism
 Multisystem Degenerations
 . Heredodegenerative Parkinsonism
 Secondary (Acquired, symptomatic) Parkinsonism

5.  Primary Parkinsonism Parkinson’s Disease
 - Sporadic Parkinson’s Disease
 - Hereditary

6. MULTISYSTEM DEGENERATIONS
(PARKINSONISM PLUS)
 Progressive Supranuclear Palsy
 Multiple System Atrophy (Shy-Dragger syn.) SND
(MSA P)
OPCA (MSA C)
 Corticobasal Degeneration
 Diffuse Lewy Body Disease
 Lytico-Bodig disease (Parkinsonism Dementia ALS
-Complex of Guam)
 Progressive pallidal Atrophy
 Parkinsonism dementia Complex
 Pallido pyramidal Degenerations

7. HEREDO DEGENERATIVE
 Hereditary Juvenile Dystonia Parkinsonism (AR Parkin
Mutation)
 Dopa - Responsive Dystonia
 Huntington’s Disease
 Wilson’s Disease
 Hereditary Ceruloplasmin Deficiency
 Frontotemporal dementia with parkinsonism
 Mitochondrial Cytopathies with Striatal Necrosis
 PKAN (Neurodegeneration associated with brain Iron
accumulation; Hallervorden - Spatz Disease)

8.  Spinocerebellar Degenerations (Eg: MJD)
 Gerstmann - Straussler - Scheinker Disease
 Familial Progressive Subcortical Gliosis
 Familial Basal Ganglia Calcification
 Lubag(X Linked dystonia - Parkinsonism)
 Ceroid Lipofuscinosis
 Neuroacanthocytosis
 Hereditary Haemochromatosis.
 Neuroferritinopathy
 Aceruloplasminemia

9. SECONDARY PARKINSONISM
 Infectious Post- encephalitic
 HIV; SSPE; Prion diseases
 Drugs
 Dopamine Receptor Blocking drugs, Reserpine, Tetrabenazine, Alpha
Methyl Dopa, Lithium, Flunarizine, Cinnarizine.
 Toxins
 MPTP, Carbon monoxide, Manganese, Mercury, Carbon
disulfide,Cyanide, Methanol.
 Vascular -Multi infarct; Binswangers disease.
 Trauma -Pugilistic Encephalopathy.
 Parathyroid abnormalities;
 Hypothyroidism;
 Brain Tumors
 Paraneoplastic
 NPH
 Psychogenic.

11. IDIOPATHIC PD
 UK Brain Bank Criteria
 bradykinesia and at least one of the following: rest
tremor, rigidity, or postural instability.

12. RED FLAGS
 Symmetric bradykinesia and rigidity
 Absence of tremor
 Prominent myoclonus
 Limb apraxia
 Alien limb phenomena
 Impaired down gaze
 Facial dystonia
 Early loss of postural reflexes
 L- dopa induced facial dyskinesias
 Ataxia
 Stridor
 Early dysphagia
 Spasticity
 Early dementia or hallucinations
 Early and prominent dysautonomia

14. MAJOR SYNDROMES
 PSP
 MSA
 CBGD
 DLBD

15. PATHOPHYSIOLOGY
 Accurate diagnosis is necessary to understand
pathogenesis
 Two proteins mainly
 Alpha synuclein
- Pre synaptic protein
- Aggregates in cell body & neurons – lewy body &
lewy neuritis- PD & DLBD
- glial cytoplasmic inclusion in MSA

18. TAUPATHY
 4 repeat tau accumalates
 NFT & in glia
 Psp- astrocytic tufts
 CBD- astrocytic plaques

21. PROGRESSIVE SUPRANUCLEAR PALSY
 Steele et al—1964
 5% of parkinsonian pts
 Prevalence—4.9 / 100,000
 Incidence 1.7 (50-59yrs) to 14.7(80-89yrs)
 Commonly misdiagnosed as PD
 Insidious onset
 No pathologic proven cases have begun before the
age of 40.

22.  Tauopathy-4-repeat t, 4R tauopathy).
 always sporadic, few familial cases-
- MAPT (microtubuli associated protein t) gene mtn.
 Mitochondrial dysfunction & oxidative stress -
pathophysiology of PSP

23. Postural Instability & EP Features :
 Falls—backward
 Poor postural reflexes
 Rigidity –axial
 Dysarthria—spastic,
 Hypophonic
 ataxic
 Frontal release signs
 Pseudobulbar palsy
 L-DOPA UNRESPONSIVENESS

24.  Reduced blink rate and closure of eyelids due to eyelid
dystonia or levator inhibition ( apraxia of eyelid
opening)
 square wave-jerks
 on doll’s eye maneuver, there is improved range, as
vestibulo-ocular reflex is preserved
 Subcortical-type dementia
 Typical facies- “surprised look”

27. NINDS–SPSP DIAGNOSTIC CRITERIA FOR PSP
 Possible PSP
Mandatory inclusion criteria:
 gradually progressive disorder
 onset age 40 or later
 Either vertical supranuclear palsy or both slowing of
vertical saccades
 postural instability with falls within a year of disease onset
 no evidence of other diseases that could explain the
foregoing features, as indicated by exclusion criteria

28. Mandatory exclusion criteria:
 recent history of encephalitis
 alien limb syndrome
 cortical sensory deficits
 focal frontal or temporoparietal atrophy
 hallucinations or delusions unrelated to
dopaminergic therapy
 cortical dementia of Alzheimer type
 prominent, early cerebellar symptoms
 unexplained dysautonomia

29. Supportive features:
 symmetrical akinesia or rigidity
 proximal more than distal
 abnormal neck posture
 especially retrocollis
 poor or absent response of parkinsonism to levodopa
 early dysphagia and dysarthria
 early onset of cognitive impairment including two or
more of: apathy, impairment in abstract thought,
decreased verbal fluency, utilisation or imitation
behaviour, or frontal release signs

30. Probable PSP
Mandatory inclusion criteria
 gradually progressive disorder
 onset age 40 or later
 vertical supranuclear palsy
 prominent postural instability with falls within a year
of disease onset
 no evidence of other diseases that could explain
the foregoing features, as indicated by exclusion
criteria

31. Definite PSP
Mandatory inclusion criteria:
 clinically probable or possible PSP and
histopathological evidence of typical PSP

32. PHENOTYPIC SPECTRUM OF PROGRESSIVE
SUPRANUCLEAR PALSY
 typical PSP phenotype- Richardson syndrome
 PSP-p = PD at least at the initial stages, with asymmetric
parkinsonism, rest-tremor, better levodopa response, longer
mean survival
 pure akinesia with gait freezing (PSP-PAGF)
 corticobasal syndrome (PSP-CBS),
 frontotemporal dementia (PSP-FTD), progressive nonfluent
aphasia
 no clinical sign to predict PSP pathologic abnormality
accurately in the non-RS phenotypes

33. INVESTIGATIONS
 clinical diagnosis
 MRI-
midbrain atrophy
Superior cerebellar peduncle atrophy.
“morning glory flower sign” and the “hummingbird sign” are
quite specific but show low sensitivity (50% and 68.4%,
respectively
 (DATscan) is abnormal in PD and all AP syndromes

34.  differentiate with PD, [123]meta-iodobenzylguanidine
(MIBG) and 123I-iodobenzamide (IBZM) SPECT may be
useful
 MIBG is abnormal in PD because of postganglionic
sympathetic denervation, but is typically normal in PSP.
 IBZM SPECT assessing the postsynaptic receptors is
abnormal in PSP and normal in PD
 IBZM SPECT is abnormal in all APs and therefore
cannot differentiate between PSP and other AP
 Novel diagnostic approaches and biomarkers
- csf tau protein
-neurofilament light chain

37. PATHOLOGY
 Neurofibrillary tangles are present in these areas.
 Tufted astrocytes (Gallyas-positive) -hallmark
feature of PSP that differentiates other 4R
tauopathies such as CBD (astrocytic plaques

39. RX
 no effective symptomatic or neuroprotective treatments
 A trial of levodopa (up to 1 g/d) and amantadine (up to 450 mg/d)
 Botulinum toxin injections can be used to treat levator inhibition.
 Serotonin reuptake inhibitors (SSRIs) may be used for apathy with no
clear benefit.
 A small study with Coenzyme Q10- no RCT study
 Recent large, double-blind studies with GSK-3b inhibitors (Tideglusib,
Davunetide) have failed to show any clinical effect.
 Tideglusib reduced the rate of brain atrophy in one study.
 Supportive measures such as physiotherapy, walking aids, speech
therapy and PEG

40. MULTIPLE SYSTEM ATROPHY :
 Sporadic neurodegenerative disorder clinically any
combination of parkinsonian, autonomic, cerebellar, or
pyramidal signs
 Pathologically–
cell loss, gliosis, and glial cytoplasmic inclusions in
several CNS structures.
 MSA is an a-synucleinopathy
 usually a sporadic disease; however, rarely, familial
cases - mutations in COQ2 gene

41.  Epidemiology :
 prevalence of MSA in four studies ranged from 1·9
to 4·9 cases per 100 000 people.
 similar to those of other well-known
neurodegenerative disorders such as Huntington’s
disease and motor neuron disease.
 no single environmental factor shown to increase
or to reduce risks of MSA

42. Clinical presentation :
 affects both men and women
 the sixth decade of life progresses with a mean
survival of 6–9 years.
 substantial variation of disease progression with
survival of more than 15 yrs.
main features –
 autonomic failure
 Parkinsonism
 cerebellar ataxia
 pyramidal signs in any combination.

43. TWO MAJOR MOTOR PRESENTATIONS
 distinguished clinically–
 Parkinsonian features predominate in 80% of
patients (MSA-P subtype),
 cerebellar ataxia is the main motor feature in 20%
of patients (MSA-C subtype).
 Both similar survival times.
 patients with MSA-P have a more rapid functional
deterioration than those with MSA-C

44.  MSA-P-associated parkinsonism :
 progressive akinesia and rigidity
 jerky postural tremor and tremor at rest.
 orofacial or craniocervical dystonia associated with a
characteristic quivering high-pitched dysarthria.
 Postural stability is compromised early on in the
disease course
 recurrent falls at disease onset are unusual in
contrast to psp.

45.  90% of the MSA-P pts- unresponsive to levodopa in
the long term.
 50% have levodopa-induced dyskinesia affecting
orofacial and neck muscles, without motor benefit.
 fully developed clinical picture of MSA-P evolves
within 5 years of disease onset, allowing a clinical
diagnosis during follow-up.

46. MSA –P RED FLAGS
 Early instability
 Rapid progression
 Pisa syndrome,camptocormia,contractures
 Bulbar dysfunction
 Respiratory dysfn- stridor,insp sighs
 Emotional incontinence
 2/6 – probable MSA-P – additional criteria

47. MSA-C :
 gait ataxia
 limb kinetic ataxia
 scanning dysarthria,
 cerebellar oculomotor disturbances.
 Most patients develop additional non-cerebellar
symptoms and signs
 may be indistinguishable from other patients with
idiopathic late onset cerebellar ataxia

48.  Dysautonomia :
 urogenital and orthostatic dysfunction.
 Early erectile dysfunction is nearly universal in men
with MSA
 Female- genital insensitivity
 urinary incontinence or retention are common early
in the course or as presenting symptoms

49. CONSENSUS STATEMENT FOR THE CLINICAL
DIAGNOSIS OF MSA
 clinical domains,
 features
 criteria used in the diagnosis of MSA

50.  Autonomic and urinary dysfunction :
 Features
 1. Orthostatic hypotension
 2. Urinary incontinence or incomplete bladder emptying
Criteria
 Reduction of least 30mmHg or in diastolic blood pressure
by at least 15 mm Hg after 3 min of standing
 urinary incontinence (persistent, involuntary partial or
total bladder emptying, accompanied by erectile
dysfunction in men) or both

51.  Parkinsonism :
 A. Features
 1. Bradykinesia
 2. Rigidity
 3. Postural instability (not caused by primary visual,
vestibular, cerebellar, or proprioceptive dysfunction) 4.
Tremor (postural, resting or both)
 B. Criteria
 Bradykinesia plus at least one of features 2–4

52.  Cerebellar dysfunction :
 A. Features
 1. Gait ataxia
 2. Ataxic dysarthria
 3. Limb ataxia
 4. Sustained gaze-evoked nystagmus
 Criteria
 Gait ataxia plus at least one of features 2–4

53.  Corticospinal tract dysfunction
 A. Features
 1. Extensor plantar responses with hyper-reflexia
 Criteria
 Corticospinal tract dysfunction in MSA: no
corticospinal tract features are used in defining the
diagnosis of MSA
 prominent and severe spasticity should raise
suspicion for an alternative diagnosis

54. Consensus statement: exclusion criteria for the
diagnosis of MSA :
 History
 Symptomatic onset under 30 years of age
 Family history of a similar disorder
 Systemic disease or other identifiable causes
 Hallucinations unrelated to medication
 DSM IV criteria for dementia
 Prominent slowing of vertical saccades or vertical
supranuclear gaze palsy
 Evidence of focal cortical dysfunction such as aphasia,
alien limb syndrome, and parietal dysfunction
 Laboratory investigation- Metabolic, molecular genetic
and imaging evidence of an alternative cause of
features

55. DIAGNOSTIC CLINICAL APPROACH
Motor signs
 Parkinsonism poorly responsive to levodopa
 Cerebellar ataxia
 Pyramidal signs
 Early instability and falls Within 3 years of disease
onset
 Rapid progression (wheelchair sign) despite
dopaminergic treatment within 5 years of disease
onset

56.  Orofacial dystonia or dyskinesias
 Atypical spontaneous or levodopa induced
dystonia
 dyskinesia mainly affecting orofacial muscles,
[resembling risus sardonicus of cephalic tetanus]
 Axial dystonia -Pisa syndrome (subacute axial
dystonia with a severe tonic lateral flexion of the
trunk, head, and neck)
 early severe camptocormia
 Disproportionate antecollis -Chin on chest, neck
can only be passively and forcibly extended to its
normal position with difficulty; despite severe
chronic neck flexion, flexion elsewhere is minor.

58.  Jerky tremor
 Irregular myoclonic postural or action tremor of the hands
or fingers
 Dysarthria-
- Atypical quivering, irregular and severely hypophonic or
slurring high pitched dysarthria,
- tends to develop earlier and be more severe than in PD
 notable dysphagia

59. Non-motor signs
 Severe dysautonomia
 Abnormal respiration
 Nocturnal (harsh or strained, high pitched inspiratory
sounds) or diurnal inspiratory stridor,
 involuntary deep inspiratory sighs and gasps, sleep
apnoea
 snoring increased from premorbid level
 newly arisen REM sleep behaviour disorder
 Emotional incontinence

60. “RED FLAGS’’
 RBD and autonomic failure- pre motor stage
 Early falls and postural instability can be seen- look for
eye signs & fronto-subcortical dysfunction for PSP
 L-dopa induced oro facial dystonia- MSA
 Raynaud phenomenon is a common in MSA
 Freezing of gait may be prominent, early, and severe,
causing diagnostic difficulties -PSP -PAGF phenotype
 dementia, it is considered a non supporting feature for
the diagnosis of MSA
 frank, prominent, early dementia should lead the clinician
to other diagnoses.

61. Investigations
 Autonomic function tests
 Cardiovascular function test-within in 2-3 ys
 Bladder function tests
 standard urine analysis will exclude infection.
 The residual volume –usg,cystometry ,UDS

62. IMAGING
 MRI
- Hot cross burn sign- mcp/pons- MSA-c
- Putaminal rim- MSA-p
- DAT scan
- abnormal in all MSA, PSP, and PD
- MIBG scintigraphy
- abnormal in PD, normal in MSA
 IBZM SPECT is normal in PD,abnormal in MSA (but
also in PSP and CBD)

64. NOVEL BIOMARKERS
 Mollenhauer and colleagues-
CSF mean a-synuclein levels , not total t, or Ab42 levels
differentiated PD and MSA from neurologic controls (70%
sensitivity, 53% specificity)
 a-synuclein and phosphorylated t/total t could differentiate
PD from MSA -sensitivity of 90% & specificity of 71%
 Flt3 ligand, a cytokine
PD and MSA with a sensitivity of 99% and a specificity of
95%.

65. RX
 Symptomatic
 PD- L-dopa/ dopa agonists- cranio cervical dystonia
postural hypotension
Amantidine- gait disturbances
 Orthostatic hypotension-
high salt, fludrocortisone,midodrine
 Urinary dysfunction
- UDS- characterise nature
-Neurogenic bladder- Oxybutinin or Tolderotidne
 Erectile dysfunction-
- sildenafil
-intracavernosal inj. Or penile implants

66.  Emotional incontinence
- SSRI/TCA
RCT – Rasagiline and rifampicin- no effects
Promising studies
- IVIG
- Intrarterial/IV autologous stem cells
- Future
- Alpha synuclein targeting antibodies

67. CORTICOBASAL DEGENERATION :
sixth to eighth decades of life,
onset of symptoms at mean age 63 years (SD 7·7)
. sporadic disease
4–6% of parkinsonism,

68. Clinical presentations
 Five initial presentations
 The most common presentation (55%) -“useless
arm” (ie, a rigid, dystonic, akinetic, or apraxic arm),
 gait disorder (27%)
 prominent sensory symptoms
 isolated speech disturbance
 behavioural disturbance

69.  Clinical features
Motor
Limb clumsiness (asymmetric)
Bradykinesia/Akinesia (asymmetric)
Rigidity (asymmetric)
Tremor (action/postural)
Myoclonus
Limb dystonia (asymmetric)
Blepharospasm
Choreoathetoid movements
Speech abnormalities
Gait disorder

70. Higher cortical functions
 Apraxia (limb more common than orofacial, eyelid-
opening)
 Dementia
 Alien-limb phenomenon
 Aphasia
 Frontal-lobe-release signs
 Cortical sensory abnormalities
 Depression
 apathy
 Anxiety Irritability
 Disinhibition
 Delusions
 Obsessive compulsive disorder

71. DIAGNOSTIC CRITERIA FOR CORTICOBASAL
DEGENERATION
Inclusion criteria (one of A or B)
A) Rigidity (easily detectable without reinforcement) and
one cortical sign:
 apraxia (more than simple use of limb as object;
absence of cognitive or motor deficit sufficient to explain
disturbance)
 cortical sensory loss (preserved primary sensation,
asymmetric)
 alien-limb phenomenon (more than simple levitation)
B) Asymmetric rigidity, dystonia (focal in limb; present at
rest at onset),
 focal reflex myoclonus (spreads beyond stimulated digits

72. Exclusion criteria
 Early dementia (will exclude some patients with
corticobasal degeneration)
 Early vertical gaze palsy
 Rest tremor
 Severe autonomic disturbances
 Sustained responsiveness to levodopa
 Lesions on imaging studies indicate another
pathological process

73. PROPOSED CRITERIA FOR THE DIAGNOSIS OF
CORTICOBASAL DEGENERATION
Core features
 Insidious onset and progressive course
 No identifiable cause (eg, tumour, infarct)
 EPS – one of the follow
-focal or asymmetric appendicular rigidity
-lacking prominent and sustained l-dopa response
-focal or asymmetric appendicular dystonia
 Cortical dysfunction - at least one of the following
: focal or asymmetric ideomotor apraxia
alien-limb phenomena
cortical sensory loss visual or sensory hemineglect
constructional apraxia
focal or asymmetric myoclonus
apraxia of speech or nonfluent aphasia

74. Supportive investigations
 Variable degrees of focal or lateralised cognitive
dysfunction,
 with relative preservation of learning and memory
on neuropsychometric testing
 Focal or asymmetric atrophy on CT or MRI imaging,
typically in perifrontal cortex
 Focal or asymmetric hypoperfusion on SPECT or
PET, typically maximal in parietofrontal cortex with
or without basal ganglia involvement

75. NEUROPATHOLOGICAL CRITERIA
Core features
 Focal cortical neuronal loss
 Substantia nigra neuronal loss
 Cortical and striatal Gallyas/tau-positive neuronal and
glial lesions, especially astrocytic plaques and threads, in
both white and grey matter
Supportive features
 Cortical atrophy, commonly with superficial spongiosis
 Ballooned neurons, in atrophic cortices
 Tau-positive oligodendroglial coiled bodies

76. PHENOTYPIC SPECTRUM OF CORTICOBASAL
DEGENERATION
 Classic CBD phenotype- CBS
 CBD- present with FTD,RS,PPA,PCA
 AD,PSP,FTD present with CBS
 Symmetrical bilateral CBS- AD pathology/RS
 Early onset PSP – CBD pathology
 For which proposed clinical criteria applied

77. INVESTIGATIONS
 Imaging
asymmetric frontal, and parietal cortical atrophy becomes
evident with dilatation of the lateral ventricle
 EEG –
-normal at first
- show asymmetric slowing that is maximum over the
hemisphere contralateral to the more affected limb
 Dopamine transporter SPECT- abnormal
- differentiate them from those with Alzheimer’s and Pick’s
diseases (in whom this scan is typically normal) early in
the course of the disease.

78.  FDG-PET
- Asymmetric reduction in fronto parietal regions

79. RX
 Anecdotal
 L-dopa trial (upto 1 gm/d)/Amantadine- PD symptoms
 Valproate, Levetiracetam- myoclonus
 Botox inj- dystonic hand
 No trials with ACEI – dementia
 Palliative rx

80. “CORTICO BASAL DEGENERATION-LOOKALIKE”
SYNDROMES
 atypical manifestations of PSP
 -progressive nonfluent aphasia FTD.
 Parkinson’s disease
 multiple-system atrophy
 Wilson’s disease
 progressive subcortical gliosis
 rigid-akinetic type Huntington’s disease
 atypical Pick’s disease
 parkinsonism– dementia–amyotrophic-lateral-sclerosis
complex
 prion related disease
 sudanophilic leukodystrophy
 neurofilament inclusion disease.

81. DEMENTIA WITH LEWY BODIES
Central feature (essential for a diagnosis of possible
or probable DLB)
 Dementia -progressive cognitive decline of sufficient
magnitude to interfere with normal social or
occupational function.
 Prominent or persistent memory impairmen- not occur
in the early stages ,usually evident with progression.
 Deficits on tests of attention, executive function, and
visuospatial ability may be especially prominent.

82. CORE FEATURES (TWO CORE FEATURES ARE SUFFICIENT FOR
A DIAGNOSIS OF PROBABLE DLB, ONE FOR POSSIBLE DLB)
 Fluctuating cognition with pronounced variations in
attention and alertness
 Recurrent visual hallucinations that are typically well
formed and detailed
 Spontaneous features of parkinsonism

83. SUGGESTIVE FEATURES
 REM sleep behavior disorder
 Severe neuroleptic sensitivity
 Low dopamine transporter uptake in basal ganglia
demonstrated by SPECT or PET imaging
 (If one or more of these is present in the presence of one
or more core features, - probable DLB .
 In the absence of any core features, one or more
suggestive features is sufficient for possible DLB.
 Probable DLB should not be diagnosed on the basis of
suggestive features alone.)

84. SUPPORTIVE FEATURES (COMMONLY PRESENT BUT NOT
PROVEN TO HAVE DIAGNOSTIC SPECIFICITY)
 Repeated falls and syncope
 Transient, unexplained loss of consciousness
 Severe autonomic dysfunction, e.g., orthostatic hypotension, urinary
incontinence
 Hallucinations in other modalities
 Systematized delusions
 Depression
 Relative preservation of medial temporal lobe structures on CT/MRI
scan
 Generalized low uptake on SPECT/PET perfusion scan with reduced
occipital activity
 Abnormal (low uptake) MIBG myocardial scintigraphy
 Prominent slow wave activity on EEG with temporal lobe transient
sharp waves

85. A DIAGNOSIS OF DLB IS LESS LIKELY
 If -CVA evident as focal neurologic signs or on brain
imaging
 In the presence of any other physical illness or brain
disorder sufficient to account in part or in total for the
clinical picture
 If parkinsonism only appears for the first time at a stage
of severe dementia

86. TEMPORAL SEQUENCE OF SYMPTOMS
 diagnosed when dementia occurs before or concurrently
with parkinsonism (if it is present).
 Parkinson disease dementia (PDD) - dementia that occurs
in the context of well-established Parkinson disease.
 In research studies in which distinction needs to be made
between DLB and PDD, the existing 1-year rule between
the onset of dementia and parkinsonism - DLB continues
to be recommended.

87. CLINICAL MANAGEMENT
 Motor parkinsonism-
-Levodopa at low doses & titrate up.
-Anticholinergics should be avoided.
 Neuropsychiatric symptoms.--cholinesterase inhibitors
(CHEIs) or atypical antipsychotic

92. “ATYPICAL” ATYPICAL PARKINSONISM
 Misdiagnosis PD with AP , as well as FTD,AD,PPA
 Mimickers of Atypical PD
Eg:
SCAS/ FTAX- mimic MSA
Neimann Pick C,CTX,prion d/s,mitochondrial- mimic AP
phenotype
- Age of onset
- Tempo of progression
- Family history
- Clinical exam + associated features

94. SUMMARY
 Careful clinical examination
 Expanding phenotypic spectrum of AP & expanding
pathological spectrum of classic AP phenotypes –
diagnostic challenge
 AP mimickers
 Investigations may be supportive, but their sensitivity
and specificity are low.
 There are currently no biomarkers available.
 There are currently no neuroprotective treatments
available.
 symptomatic and supportive treatments with usually no
sustained effect.
 Further research required

95. THANK YOU