akinetic rigid syndrome

Akinetic rigid Akinetic rigid syndromesyndrome

PresenterPresenter

Dr.Amit R.Barua.Dr.Amit R.Barua.

9/119/11

IntroductionIntroduction

Akinetic rigid syndromeAkinetic rigid syndrome implies= implies= :Slowness of initiation of movement specially :Slowness of initiation of movement specially

involving upper body (involving upper body (akinesia)akinesia) ::RigidityRigidity. .

{characterized by abnormal stiffness due to {characterized by abnormal stiffness due to increased tone of both the agonist and increased tone of both the agonist and antagonist muscles (antagonist muscles (lead pipelead pipe) ) or or cogwheel rigiditycogwheel rigidity, due to superimposed or , due to superimposed or underlying tremor.} underlying tremor.}

IntroductionIntroduction

It may additionally be accompaniedIt may additionally be accompanied by= :Slowness of movement (by= :Slowness of movement (bradykinesiabradykinesia) )

:Thought (:Thought (bradyphreniabradyphrenia), ), ::Difficulty initiating movementDifficulty initiating movement, , ::Progressive fatiguingProgressive fatiguing, , ::Diminishing amplitude of repetitive Diminishing amplitude of repetitive

alternatingalternating movements movements with or without tremor with or without tremor at restat rest, (usually 3-5 Hz) and on posture (usually , (usually 3-5 Hz) and on posture (usually 4-8 Hz) 4-8 Hz)

::Postural instabilityPostural instability. .

AetiologyAetiology Akinetic-rigid syndromesAkinetic-rigid syndromes can be caused by can be caused by diverse etiologies. diverse etiologies.

It is vital to separate It is vital to separate idiopathic Parkinson's diseaseidiopathic Parkinson's disease from other from other neurodegenerative diseases and causes of secondary neurodegenerative diseases and causes of secondary parkinsonism as it has significant therapeutic implications. parkinsonism as it has significant therapeutic implications.

Often the history may be nonspecific and all investigations may Often the history may be nonspecific and all investigations may be normal. be normal.

The diagnosis may thus rest entirely on clinical features. The diagnosis may thus rest entirely on clinical features.

The etiological diagnosis of Akinetic rigid syndrome The etiological diagnosis of Akinetic rigid syndrome has critical therapeutic and prognostic implications.has critical therapeutic and prognostic implications.

Neurodegenerative disordersNeurodegenerative disorders

Commonest aetiology >40yrs.Commonest aetiology >40yrs.

1.Symmetrical neurodegenerative disorders1.Symmetrical neurodegenerative disorders..

:MSA.:MSA.

:PSP.:PSP.

2.Asymmetrical neurodegenerative disorders.2.Asymmetrical neurodegenerative disorders.

:PD.:PD.

:CBD.:CBD.

2.Asymmetrical neurodegenerative 2.Asymmetrical neurodegenerative disordersdisorders

Idiopathic PDIdiopathic PD characterized by= characterized by= Insidious onset akinesia and rigidityInsidious onset akinesia and rigidity (either lead (either lead

pipe or cogwheel), pipe or cogwheel), Generally involving one limb at onset, which Generally involving one limb at onset, which

involves axial musculatureinvolves axial musculature and becomes and becomes bilateral.bilateral.

Clinical asymmetry is retained as the disease Clinical asymmetry is retained as the disease progresses over 10 or more years. progresses over 10 or more years.

Asymmetrical neurodegenerative Asymmetrical neurodegenerative disordersdisorders

Akinesia/BradykinesiaAkinesia/Bradykinesia 50%50% of IPD patients of IPD patients complain of complain of slowing slowing

upup at presentation. at presentation.

The slowness of IPD involves both The slowness of IPD involves both initiation and executioninitiation and execution of movements, of movements, particularly sequential and volitional particularly sequential and volitional actions.actions.

Writing becomes smaller (Writing becomes smaller (micrographiamicrographia). ).


Akinesia/BradykinesiaAkinesia/Bradykinesia Gradually they developGradually they develop difficulty washing, difficulty washing,

feeding, dressing and turning in bed, but these feeding, dressing and turning in bed, but these functions are rarely affected at presentation. functions are rarely affected at presentation.

Loss of facial expression occurs earlyLoss of facial expression occurs early, while= , while= ::Decreased eye blinking, Decreased eye blinking, :Hypophonia,:Hypophonia, :Impaired swallowing leading to sialorrhoea :Impaired swallowing leading to sialorrhoea are are

later featureslater features. .


RigidityRigidity The rigidity of IPD is, constant throughout a full The rigidity of IPD is, constant throughout a full

range of passive movement (range of passive movement (lead pipe rigiditylead pipe rigidity.) .)

If a postural tremor is superimposed it takes on a If a postural tremor is superimposed it takes on a ratchet characteristic described as ratchet characteristic described as "cogwheel" "cogwheel" rigidity.rigidity.

The majority of patients The majority of patients have rigidity on have rigidity on examination, especially if synkinesisexamination, especially if synkinesis of the of the opposite limb is performed; the increased tone is opposite limb is performed; the increased tone is often unilateral at presentation spreading to often unilateral at presentation spreading to become bilateral over three to five years, but become bilateral over three to five years, but maintaining an asymmetrical emphasis. maintaining an asymmetrical emphasis.


RigidityRigidity

The The rigidity initially targets limbsrigidity initially targets limbs and and later later spreads axially predominantlyspreads axially predominantly involving involving limb and trunk flexorslimb and trunk flexors leading to leading to characteristic stooped posturingcharacteristic stooped posturing with trunk, with trunk, neck and arm flexion and foot inversion. neck and arm flexion and foot inversion.

The rigidity is abolished by sleep.The rigidity is abolished by sleep.

In 80% of cases responds well to In 80% of cases responds well to dopaminergic agents. dopaminergic agents.


TremorTremor 40% of patients40% of patients will complain of tremulousness of hand at rest, will complain of tremulousness of hand at rest,

which improves with action. which improves with action. 80% will have an asymmetrical 3-5 Hz rest tremor80% will have an asymmetrical 3-5 Hz rest tremor evident on evident on

examination.examination. A 4-8 Hz postural tremorA 4-8 Hz postural tremor is as frequent as the characteristic is as frequent as the characteristic

rest tremor. rest tremor. The tremor of IPDThe tremor of IPD can affect the eyelids, jaw,chin and legs in can affect the eyelids, jaw,chin and legs in

addition to arms.addition to arms.

Parkinsonian tremorParkinsonian tremor is not associated with in-coordination. is not associated with in-coordination.

IIncreasedncreased by anxiety and by anxiety and abolishedabolished by sleep. by sleep.

Limb activity attenuates rest but increases postural tremorLimb activity attenuates rest but increases postural tremor..


TremorTremor IPD presenting as an IPD presenting as an isolated postural tremorisolated postural tremor, can be , can be

differentiated from differentiated from essential tremoressential tremor by= by= 1.Late onset. 1.Late onset.

2.Starting in one limb and a latency of a few seconds 2.Starting in one limb and a latency of a few seconds when the arm is extended. when the arm is extended.

Also the breakthrough Also the breakthrough "rest" component of severe "rest" component of severe essential tremor can be discriminated from the rest essential tremor can be discriminated from the rest tremor of IPDtremor of IPD as its amplitude is reduced and the as its amplitude is reduced and the frequency is similar to that of the postural component. frequency is similar to that of the postural component.

In IPD, the rest tremor has a higher amplitude and lower In IPD, the rest tremor has a higher amplitude and lower frequency than the postural component. frequency than the postural component.


TremorTremor Around 50% of rest tremors respond well to Around 50% of rest tremors respond well to

dopaminergic and anticholinergic agents.dopaminergic and anticholinergic agents.

Rarely a minority are refractory. Rarely a minority are refractory.

Postural tremors of IPD, like Essential tremor, Postural tremors of IPD, like Essential tremor, may respond to both may respond to both β blockers and ethanolβ blockers and ethanol..


Gait & Postural reflexesGait & Postural reflexes The patient usually complains ofThe patient usually complains of== ::Reduced speed of walkingReduced speed of walking with shortening of steps, with shortening of steps, occasionally dragging of one leg. occasionally dragging of one leg. ::Reduced arm swingReduced arm swing on the affected side. on the affected side.

::Later a tendency to shuffleLater a tendency to shuffle develops, followed by= develops, followed by= #G#Gait ait initiation difficulties, initiation difficulties, #Freezing , #Freezing , #Festination. #Festination. (involuntary acceleration while walking) (involuntary acceleration while walking)


Gait & Postural reflexesGait & Postural reflexes They also have difficulties in turning and rising They also have difficulties in turning and rising

from a chair/bed.from a chair/bed.

While early slowing of gait responds to dopaminergic While early slowing of gait responds to dopaminergic agents the later complications do not, though they agents the later complications do not, though they may be helped by the presence of external cues=may be helped by the presence of external cues=

:Such as walking across lines,:Such as walking across lines,

:Listening to a metronome ticking,:Listening to a metronome ticking,

:Marching music on a walkman tape. :Marching music on a walkman tape.


Ocular movementsOcular movements Ocular movements especially pursuits and reflexive Ocular movements especially pursuits and reflexive

saccades are preserved till late saccades are preserved till late unlike PSPunlike PSP. .

However formal testing of remembered saccades to However formal testing of remembered saccades to an occluded target or anti-saccades reveals slowing. an occluded target or anti-saccades reveals slowing.

A frank A frank supranuclear gaze problem is rarely seensupranuclear gaze problem is rarely seen. .

Restricted voluntary up gaze and convergence can, Restricted voluntary up gaze and convergence can, however,be seen and, indeed, is present in a minority however,be seen and, indeed, is present in a minority of elderly normal subjects. of elderly normal subjects.


DystoniaDystonia Cases Cases particularly proneparticularly prone to exhibit limb to exhibit limb

dystonia=dystonia=

:1.Younger onset IPD, :1.Younger onset IPD,

:2.PD associated with :2.PD associated with parkinparkin mutations. mutations.

Asymmetrical neurodegenerative Asymmetrical neurodegenerative disordersdisordersDysautonomiaDysautonomia

PD patients frequently admit toPD patients frequently admit to==

:1.Impotence,:1.Impotence,

:2.slowed bowel motility(associated constipation :2.slowed bowel motility(associated constipation and abdominal pain.) and abdominal pain.)

::3.Symptomatic OH3.Symptomatic OH may develop as the may develop as the disease progresses.disease progresses.

Asymmetrical neurodegenerative Asymmetrical neurodegenerative disordersdisordersDysautonomiaDysautonomia

Urinary hesitancyUrinary hesitancy may reflect IPD or may reflect IPD or prostatism, but urgency and prostatism, but urgency and incontinenceincontinence are are usually caused by bladder instability.Increased usually caused by bladder instability.Increased sweating sweating "seborrhoea"seborrhoea" and " and facial flushingfacial flushing are are late featureslate features. .

Dopaminergic agents are ineffective and worsenDopaminergic agents are ineffective and worsen hypotension. hypotension.

Anticholinergic drugs such as tolterodine worsen Anticholinergic drugs such as tolterodine worsen constipation but can help bladder instability. constipation but can help bladder instability.

Constipation mayConstipation may respond to senna laxatives respond to senna laxatives or lactulose.or lactulose.


CognitionCognition Frank dementia is unusual at presentationFrank dementia is unusual at presentation though though

slowness with executive functions such as sorting, slowness with executive functions such as sorting, fluency and problem solving can be evident. fluency and problem solving can be evident.

Later, significant subcortical dementia occurs in Later, significant subcortical dementia occurs in 25%25% of the patients. of the patients.

Associated Alzheimer's disease may give rise to a Associated Alzheimer's disease may give rise to a dementia with cortical features and Diffuse Lewy Body dementia with cortical features and Diffuse Lewy Body disease is differentiated by characteristic fluctuating disease is differentiated by characteristic fluctuating confusion, visual hallucinations and paranoid confusion, visual hallucinations and paranoid delusions.delusions.

Dopaminergic and anticholinergicDopaminergic and anticholinergic agents are agents are ineffective and indeed may cause or exacerbate ineffective and indeed may cause or exacerbate confusion, hallucinations and psychosis. confusion, hallucinations and psychosis.

Asymmetrical neurodegenerative disordersAsymmetrical neurodegenerative disordersCognitionCognition

Anticholinesterase inhibitors such as Anticholinesterase inhibitors such as Rivastigmine may be helpfulRivastigmine may be helpful. .

Mild apathy and frustration are common at disease Mild apathy and frustration are common at disease onset.onset.

Majority of IPD patients are mildly depressed.Majority of IPD patients are mildly depressed.

Panic attacks and anxiety can be presenting Panic attacks and anxiety can be presenting features of IPD.features of IPD.

Asymmetrical neurodegenerative disorderAsymmetrical neurodegenerative disorder

Diffuse Lewy Body diseaseDiffuse Lewy Body disease Diffuse Lewy body disease presents with an Diffuse Lewy body disease presents with an akinetic akinetic

rigid staterigid state in around in around 24-40% patients.24-40% patients.

Approximately 90%Approximately 90% will develop akinetic rigid will develop akinetic rigid syndrome in the course of illness.syndrome in the course of illness.

The other features are The other features are hypophonic speech, masked hypophonic speech, masked facies, stooped posture and festinating gait.facies, stooped posture and festinating gait.

These akinetic rigid features are These akinetic rigid features are responsive to responsive to levodopa simulating idiopathic PD levodopa simulating idiopathic PD


Diffuse Lewy Body diseaseDiffuse Lewy Body disease However, However, rest tremor is uncommon in DLBDrest tremor is uncommon in DLBD..

MyoclonusMyoclonus may be present in 18% patients in may be present in 18% patients in contrast to PD.contrast to PD.

There is presence of syncope, repeated falls.There is presence of syncope, repeated falls. However, However, early presence of cognitive deficitsearly presence of cognitive deficits within within

a year of diagnosis a year of diagnosis as opposed to PDas opposed to PD . .

After Alzheimer's disease, it is the After Alzheimer's disease, it is the second most second most frequent cause of dementia. frequent cause of dementia.


Diffuse Lewy Body diseaseDiffuse Lewy Body disease The cognitive deficits in DLBD are The cognitive deficits in DLBD are fronto-fronto-

subcortical in nature.subcortical in nature. Involving=Involving= psychomotor speed, psychomotor speed, verbal fluency, problem solving. verbal fluency, problem solving.

Visuospatial abilities as opposed to early Visuospatial abilities as opposed to early involvement of language and memory functions involvement of language and memory functions in Alzheimer's disease.in Alzheimer's disease.


Diffuse Lewy Body diseaseDiffuse Lewy Body disease The dementia in DLBD is associated The dementia in DLBD is associated

withwith= :Prominent visual hallucinations, = :Prominent visual hallucinations, :Fluctuating mental state.:Fluctuating mental state.

The The visual hallucinationsvisual hallucinations are= are= : Well-formed,: Well-formed, :Detailed animate figures evoking emotional :Detailed animate figures evoking emotional responses.responses.

Systemized Systemized delusionsdelusions are also common. are also common.


Diffuse Lewy Body diseaseDiffuse Lewy Body disease Depression may be present in 50-60%Depression may be present in 50-60% of the of the

patients.patients.

REM sleep behavior disordersREM sleep behavior disorders are also frequent. are also frequent.

DLBD Vs PDDLBD Vs PD DLBD had older age of onset (68 DLBD had older age of onset (68 vsvs 62), 62), Shorter disease duration, Shorter disease duration, Less frequent rest tremor (55% Less frequent rest tremor (55% vsvs 85%) 85%) Response to levodopa is less frequent (70% Response to levodopa is less frequent (70% vsvs

100%).100%). (Louis & Klatka et al.,1997) (Louis & Klatka et al.,1997)


Diffuse Lewy Body diseaseDiffuse Lewy Body disease

The most useful feature may the The most useful feature may the predominant signs at presentation as predominant signs at presentation as DLBD presents with DLBD presents with cognitive and cognitive and psychiatric signs in 72% cases as psychiatric signs in 72% cases as compared to PD (6%).compared to PD (6%).

(Louis &Klatka et al.,1997)(Louis &Klatka et al.,1997)


Corticobasal degenerationCorticobasal degeneration Corticobasal degeneration (CBD) is strikingly Corticobasal degeneration (CBD) is strikingly

asymmetrical in its presentation, mimicking IPD.asymmetrical in its presentation, mimicking IPD.

:Apraxia of the affected limb.:Apraxia of the affected limb.

Difficulty recognizing objects by tactile impressions Difficulty recognizing objects by tactile impressions alone due to cortical sensory loss.alone due to cortical sensory loss.

Simultagnosia Simultagnosia Dysgraphaesthesia, despite preservation of light Dysgraphaesthesia, despite preservation of light

touch and pin prick sensation. touch and pin prick sensation.


Corticobasal degenerationCorticobasal degeneration The other manifestations in CBD include=The other manifestations in CBD include= :1. limb myoclonus that may be stimulus sensitive, :1. limb myoclonus that may be stimulus sensitive, :2.Asymmetric postural limb tremor, :2.Asymmetric postural limb tremor, :3.Limb dystonia. :3.Limb dystonia. :4.Early and severe dysphagia and dysarthria.:4.Early and severe dysphagia and dysarthria.

Supranuclear gaze difficultiesSupranuclear gaze difficulties have been described with have been described with CBD, however they are less common than PSP.CBD, however they are less common than PSP.

Pyramidal signs(extensor plantar responses.)Pyramidal signs(extensor plantar responses.)

Cerebellar signs and dysautonomia Cerebellar signs and dysautonomia are not typically seen in are not typically seen in CBD as opposed to MSA. CBD as opposed to MSA.


Corticobasal degenerationCorticobasal degeneration Dementia is usually a late feature of CBDDementia is usually a late feature of CBD, , but an early frontal dementia disease can occasionally but an early frontal dementia disease can occasionally

be seen.be seen.

Dysphasia occursDysphasia occurs early and frequently. early and frequently.

Frontal release signsFrontal release signs can occasionally be seen. can occasionally be seen.

Cases with atypical presentation Cases with atypical presentation simulating fronto simulating fronto temporal dementia and primary progressive aphasiatemporal dementia and primary progressive aphasia have also been described. have also been described.


Corticobasal degenerationCorticobasal degeneration A poor levodopa response is invariableA poor levodopa response is invariable in this disorder and in this disorder and

amantadine, is also usually ineffective. amantadine, is also usually ineffective. Treatment is essentially supportiveTreatment is essentially supportive..

Thus the main features that differentiate CBD from PD areThus the main features that differentiate CBD from PD are= = ideomotor apraxia, ideomotor apraxia, alien hand, alien hand, limb dystonia, limb dystonia, cortical sensory loss, cortical sensory loss, lack of gait difficulties lack of gait difficulties lack of response to levodopa.lack of response to levodopa.

Symmetrical neurodegenerative disorderSymmetrical neurodegenerative disorderMultiple system atrophyMultiple system atrophy

Multiple system atrophy most commonly begins in the Multiple system atrophy most commonly begins in the early early 50s(median survival of 9.3 years.) 50s(median survival of 9.3 years.)

Parkinsonism occurs in 90% of patientsParkinsonism occurs in 90% of patients with multiple system with multiple system atrophy and is usually symmetrical unlike IPD.atrophy and is usually symmetrical unlike IPD.

Cerebellar or pyramidal signs each occur in about halfCerebellar or pyramidal signs each occur in about half of of the patients.the patients.

Rest tremor is seen in MSA but is far less frequent thanRest tremor is seen in MSA but is far less frequent than in in IPD.IPD.

Limb ataxia or intention tremorLimb ataxia or intention tremor can be present in MSA, can be present in MSA, particularly in later stages, which is never seen in IPD. particularly in later stages, which is never seen in IPD.


Autonomic dysfunction failure occurs in virtually Autonomic dysfunction failure occurs in virtually all patients and it may precede the motor all patients and it may precede the motor disturbancedisturbance by months or even some years. by months or even some years.

In men, impotence is often the first symptom and In men, impotence is often the first symptom and incontinence, more than retention, is commonincontinence, more than retention, is common in in both sexes.both sexes.

Symptomatic postural hypotension is commonSymptomatic postural hypotension is common and unlike PD occurs earlyand unlike PD occurs early. .


Respiratory stridor affects about 30%Respiratory stridor affects about 30% of patients at of patients at some stage and when combined with parkinsonism is some stage and when combined with parkinsonism is highly suggestive of multiple system atrophy.highly suggestive of multiple system atrophy.

Severe apnoeic attacks and hypoventilationSevere apnoeic attacks and hypoventilation can can become severe enough to warrant a tracheostomy.become severe enough to warrant a tracheostomy.

Speech maySpeech may differ qualitatively from the differ qualitatively from the hypophonic hypophonic monotony of Parkinson's disease, incorporating monotony of Parkinson's disease, incorporating quivering, strained or slurring components.quivering, strained or slurring components.

Many patients develop severe aphonia, anarthria and Many patients develop severe aphonia, anarthria and dysphagia, which are rare in Parkinson'sdysphagia, which are rare in Parkinson's disease. disease.


Unlike PD extraocular smooth pursuit movements are Unlike PD extraocular smooth pursuit movements are hypometric to pursuit with saccadic intrusion and hypometric to pursuit with saccadic intrusion and may also show sustained nystagmus.may also show sustained nystagmus.

Square wave jerksSquare wave jerks may also be evident on central may also be evident on central fixation. fixation.

Voluntary saccades are usually full and supranuclear Voluntary saccades are usually full and supranuclear gaze problems are noted occasionally. gaze problems are noted occasionally.


Hyper reflexiaHyper reflexia in conjunction with spasticity and a positive in conjunction with spasticity and a positive Babinski response is only seen in MSA and not IPD.Babinski response is only seen in MSA and not IPD.

The flexed posture of MSA patients can be extreme and unlike The flexed posture of MSA patients can be extreme and unlike patients of IPD they become patients of IPD they become bent doublebent double with their chins on with their chins on their chest.Limb dystonia can also be a feature of MSA.their chest.Limb dystonia can also be a feature of MSA.

While frontal executive problems are found in MSA, While frontal executive problems are found in MSA, frank frank dementiais are rare, as are the hallucinations, confusion and dementiais are rare, as are the hallucinations, confusion and psychosis associated with cortical Lewy body disease. psychosis associated with cortical Lewy body disease.


Responds poorly to L-Dopa.Responds poorly to L-Dopa. Autonomic function testsAutonomic function tests can document disturbed control of blood can document disturbed control of blood

pressure and heart rate, but taken in isolation they are not particularly pressure and heart rate, but taken in isolation they are not particularly helpful in determining whether this is due to multiple system atrophy or due helpful in determining whether this is due to multiple system atrophy or due to Parkinson's disease, as similar results can be obtained in both diseases.to Parkinson's disease, as similar results can be obtained in both diseases.

In contrast, an abnormal external sphincter electromyogram reflecting loss In contrast, an abnormal external sphincter electromyogram reflecting loss of anterior horn cells in Onuf's nucleus in the sacral cord is highly suggestive of anterior horn cells in Onuf's nucleus in the sacral cord is highly suggestive of multiple system atrophy of multiple system atrophy

The presence of putaminal hypo intensity relative to globus pallidus on T2 The presence of putaminal hypo intensity relative to globus pallidus on T2 weighted MRI or slit hyper intensity of the lateral putaminal margin, with or weighted MRI or slit hyper intensity of the lateral putaminal margin, with or without cerebellar and putaminal atrophy may suggest MSA.without cerebellar and putaminal atrophy may suggest MSA.

MRI may show=MRI may show= : A hyper intense rim at the lateral edge of the dorsolateral putamen, : A hyper intense rim at the lateral edge of the dorsolateral putamen, :‘Hot cross bun' sign in the pontine basis (PB).:‘Hot cross bun' sign in the pontine basis (PB).

However neuro imaging may entirely be normal. However neuro imaging may entirely be normal.

Symmetrical neurodegenerative disorderSymmetrical neurodegenerative disorderProgressive Supranuclear Palsy (Steele-Progressive Supranuclear Palsy (Steele-

Richardson-Olszewski Syndrome) Richardson-Olszewski Syndrome) Commonly misdiagnosed as PD.Commonly misdiagnosed as PD.

The median age of onset is 55-70 years.The median age of onset is 55-70 years.

Involves the trunk and neck rather than the limbsInvolves the trunk and neck rather than the limbs , , causing early postural and gait instability with falls and causing early postural and gait instability with falls and a dystonic posture where the a dystonic posture where the trunk is flexedtrunk is flexed but the but the neck extendedneck extended..

Any elderly patient with truncal or neck rigidity either in Any elderly patient with truncal or neck rigidity either in the absence of or with only mild limb involvement and the absence of or with only mild limb involvement and impaired postural reflexes leading to backward falls impaired postural reflexes leading to backward falls should be suspected of having PSP.should be suspected of having PSP.


Richardson-Olszewski Syndrome) Richardson-Olszewski Syndrome)

Later, limb rigidity and brady kinesia Later, limb rigidity and brady kinesia develop in a symmetrical fashion but rest develop in a symmetrical fashion but rest tremor is uncommon.tremor is uncommon.

Hyper reflexia and extensor plantars.Hyper reflexia and extensor plantars.



Voluntary gaze problems are pathognomonicVoluntary gaze problems are pathognomonic but may be a but may be a late feature or even absent. late feature or even absent.

PSP accounts for 75% cases of palsies of supra nuclear PSP accounts for 75% cases of palsies of supra nuclear gazegaze..

Difficulty with reading because of problems scanning the Difficulty with reading because of problems scanning the printed text may be a presenting feature.printed text may be a presenting feature.

Impaired volitional saccades maybe brought out by asking the Impaired volitional saccades maybe brought out by asking the patient to look up and down voluntarily or left and right, in the patient to look up and down voluntarily or left and right, in the absence of a target.absence of a target.

This failure of voluntary down or lateral gaze corrects when This failure of voluntary down or lateral gaze corrects when the patient's eyes are centrally fixated and their head passively the patient's eyes are centrally fixated and their head passively tilted backwards or rotated.tilted backwards or rotated.

Reflex saccades and pursuit eye movements (become slow Reflex saccades and pursuit eye movements (become slow with saccadic intrusions) are impaired at a later stage. with saccadic intrusions) are impaired at a later stage.


Richardson-Olszewski Syndrome) Richardson-Olszewski Syndrome) Later, Later, nystagmus and frank oculoparesis maynystagmus and frank oculoparesis may occur causing the patient to occur causing the patient to

complain of diplopia. Square wave jerk on central fixation become evident.complain of diplopia. Square wave jerk on central fixation become evident.

In PSP, the In PSP, the optokinetic reflex is lost earlyoptokinetic reflex is lost early with a sustained deviation of the eyes with a sustained deviation of the eyes rather than saccades occurring on viewing a striped rotating drum.rather than saccades occurring on viewing a striped rotating drum.

The combination of neck extension and a down gaze palsy predisposes PSP patients The combination of neck extension and a down gaze palsy predisposes PSP patients to falls, particularly when descending stairs. to falls, particularly when descending stairs.

"sloppy tie sign"sloppy tie sign" as the patient cannot look down to keep food from his plate on his fork " as the patient cannot look down to keep food from his plate on his fork

while conveying it up to his mouth and so drops it on his tie.while conveying it up to his mouth and so drops it on his tie.

Also a staring expression maybe caused by eyelid retraction. This characteristic Also a staring expression maybe caused by eyelid retraction. This characteristic pattern of involvement differentiates it from pattern of involvement differentiates it from IPD where ocular movements are IPD where ocular movements are initially spared. initially spared.



The bulbar involvement is early with The bulbar involvement is early with initially a nasal spastic dysarthria (leads to initially a nasal spastic dysarthria (leads to growling and groaning) and hypophonia, growling and groaning) and hypophonia, followed by dysphagia.followed by dysphagia.

Urinary urgency, urge incontinence and Urinary urgency, urge incontinence and constipation are frequently present, though constipation are frequently present, though postural hypotensionpostural hypotension is rare. is rare.


Richardson-Olszewski Syndrome)Richardson-Olszewski Syndrome) Cognitive impairment in PSP mostly involves Cognitive impairment in PSP mostly involves

frontal lobe functions.frontal lobe functions.

Depression and frontal lobe impairments occur early in Depression and frontal lobe impairments occur early in the disease.the disease.

Memory,language and praxis deficits are seen Memory,language and praxis deficits are seen later in later in the disease.the disease.

Perseveration may be particularly obvious in speech, Perseveration may be particularly obvious in speech, with repetition of syllables and words (palilalia) or even with repetition of syllables and words (palilalia) or even of whole phrases (palilogia). of whole phrases (palilogia).


Richardson-Olszewski Syndrome)Richardson-Olszewski Syndrome) Limb apraxia is generally symmetrical and patients show Limb apraxia is generally symmetrical and patients show

difficulties imitating postures (difficulties imitating postures (ideomotor apraxiaideomotor apraxia)) and and pantomiming patterns of limb movements (pantomiming patterns of limb movements (ideational apraxiaideational apraxia).).

Eyelid opening apraxia can occasionally be present, patients Eyelid opening apraxia can occasionally be present, patients taking seconds or even minutes to open their eyes volitionally taking seconds or even minutes to open their eyes volitionally after eye closure. Personality changes are common in PSP and after eye closure. Personality changes are common in PSP and emotional lability can cause patients to oscillate inappropriately emotional lability can cause patients to oscillate inappropriately between episodes of laughing and crying. between episodes of laughing and crying.

In end stage of the disease grasp and pout reflexes can be In end stage of the disease grasp and pout reflexes can be elicited.elicited.

A poor levodopa responseA poor levodopa response is usual though high doses can be is usual though high doses can be beneficial initially. As with MSA, amantadine can be useful beneficial initially. As with MSA, amantadine can be useful where levodopa is ineffective or poorly tolerated. where levodopa is ineffective or poorly tolerated.

Symmetrical neurodegenerative disorderSymmetrical neurodegenerative disorder

Vascular ParkinsonismVascular Parkinsonism Cerebrovascular disease is an important cause of an apparent Cerebrovascular disease is an important cause of an apparent

akinetic rigid syndrome in the elderly and akinetic rigid syndrome in the elderly and responsible for 3-6% responsible for 3-6% of cases of parkinsonism.of cases of parkinsonism.

An extra pyramidal syndrome characterized by =lower body An extra pyramidal syndrome characterized by =lower body parkinsonism may occur as a complication of cerebro vascular parkinsonism may occur as a complication of cerebro vascular disease, even in the absence of recognized strokes. disease, even in the absence of recognized strokes.

These patients are generally elderly and often hypertensive.These patients are generally elderly and often hypertensive.

The gait is short-stepped but often The gait is short-stepped but often wide-based unlike PDwide-based unlike PD and and postural stability may be impaired.postural stability may be impaired.


Vascular ParkinsonismVascular Parkinsonism Start hesitation and freezing may occur, as in Start hesitation and freezing may occur, as in

Parkinson's disease, but above the waist there is a Parkinson's disease, but above the waist there is a striking absence of true parkinsonism.striking absence of true parkinsonism.

Thus, the voice and facial expression are lively and Thus, the voice and facial expression are lively and well modulated, the arms are used freely in well modulated, the arms are used freely in spontaneous gesture, rest tremor is absent, there is spontaneous gesture, rest tremor is absent, there is no fatigue or decrement of alternating movements and no fatigue or decrement of alternating movements and the posture is usually upright.the posture is usually upright.

An acute onset, more rapid or stepwise progression, An acute onset, more rapid or stepwise progression, presence of multiple vascular risk factors and absence presence of multiple vascular risk factors and absence of sustained response to levodopa therapy are useful of sustained response to levodopa therapy are useful differentiating features from Parkinson' disease. differentiating features from Parkinson' disease.


Vascular ParkinsonismVascular Parkinsonism Imaging often showsImaging often shows== :BG lacunae or white matter change,:BG lacunae or white matter change, :Sometimes hydrocephalus, :Sometimes hydrocephalus, :Rarely cerebral tumor can give rise to a :Rarely cerebral tumor can give rise to a

broadly similar picture.broadly similar picture.

However it is important to recognize that people However it is important to recognize that people with Parkinson's disease can have concomitant with Parkinson's disease can have concomitant cerebro vascular disease.cerebro vascular disease.

Hereditary Causes of Akinetic Rigid SyndromeHereditary Causes of Akinetic Rigid Syndrome

Diseases with Akinetic rigid syndrome Diseases with Akinetic rigid syndrome as a major componentas a major component Dopa responsive dystonia (DYT5). Dopa responsive dystonia (DYT5).

:Usually presents in childhood and adolescence with foot :Usually presents in childhood and adolescence with foot dystonia, diurnal fluctuations and frequent falls. dystonia, diurnal fluctuations and frequent falls.

:Exquisitely responsive to small doses of levodopa,:Exquisitely responsive to small doses of levodopa,

:Do not develop motor fluctuations even in advanced :Do not develop motor fluctuations even in advanced disease.disease.

Hereditary Causes of Akinetic Rigid SyndromeHereditary Causes of Akinetic Rigid Syndrome Diseases with Akinetic rigid syndrome Diseases with Akinetic rigid syndrome as a major componentas a major component

Rapid onset dystonia parkinsonismRapid onset dystonia parkinsonism :A rare autosomal dominant disorder with acute -:A rare autosomal dominant disorder with acute -

subacute onset over hours to weeks. subacute onset over hours to weeks. :The typical symptoms are combined dystonia, levodopa :The typical symptoms are combined dystonia, levodopa

unresponsive parkinsonism, bulbar and arm involvement unresponsive parkinsonism, bulbar and arm involvement as compared to legs. as compared to legs.

X-linked dystonia parkinsonism (Lubag's diseaseX-linked dystonia parkinsonism (Lubag's disease) ) has been described in middle-aged patients from has been described in middle-aged patients from Philippines.Philippines.

Dementia-parkinsonism -amyotrophy complexDementia-parkinsonism -amyotrophy complex is an is an autosomal dominant disorder characterized by frontal autosomal dominant disorder characterized by frontal lobe type of behavior disorder, cognitive impairment and lobe type of behavior disorder, cognitive impairment and amyotrophy.amyotrophy.

Hereditary Causes of Akinetic Rigid SyndromeHereditary Causes of Akinetic Rigid Syndrome Diseases with Akinetic rigid syndrome Diseases with Akinetic rigid syndrome as a minor componentas a minor component

Wilson’s diseaseWilson’s disease In children and young adults WD is an important cause of In children and young adults WD is an important cause of

akinetic rigid syndrome.akinetic rigid syndrome.

It is an autosomal recessive disorder of copper metabolism It is an autosomal recessive disorder of copper metabolism due to defect excretion of copper by liver.due to defect excretion of copper by liver.

The patients with neurological WD present in second or third The patients with neurological WD present in second or third decade.decade.

It typically presents with dystonia, rigidity, tremor, bradykinesia, It typically presents with dystonia, rigidity, tremor, bradykinesia, drooling, dysarthria, dysphagia, ataxia, unsteady gait and drooling, dysarthria, dysphagia, ataxia, unsteady gait and mental deterioration.mental deterioration.

There may be characteristic wing beating tremor, though all There may be characteristic wing beating tremor, though all categories of parkinsonian tremors are seen. categories of parkinsonian tremors are seen.

Hereditary Causes of Akinetic Rigid SyndromeHereditary Causes of Akinetic Rigid Syndrome Diseases with Akinetic rigid syndrome Diseases with Akinetic rigid syndrome as a minor componentas a minor component

Huntington diseaseHuntington disease HD is a autosomal dominant disorder of trinucleotide repeats characterized HD is a autosomal dominant disorder of trinucleotide repeats characterized

by chorea, behavior changes and cognitive impairment.by chorea, behavior changes and cognitive impairment.

However HD often in patients younger than 20 years and rarely in adults However HD often in patients younger than 20 years and rarely in adults (Westphal variant) may present as an Akinetic rigid syndrome with dystonia.(Westphal variant) may present as an Akinetic rigid syndrome with dystonia.

Even in atypical HD in late stages akinesia and rigidity may be the dominant Even in atypical HD in late stages akinesia and rigidity may be the dominant features.features.

Additionally impairment of sustained voluntary movements, motor Additionally impairment of sustained voluntary movements, motor restlessness, gait abnormalities, abnormal saccadic eye movements and restlessness, gait abnormalities, abnormal saccadic eye movements and signs of bulbar dysfunction dysphagia and dysarthria may be seen in this signs of bulbar dysfunction dysphagia and dysarthria may be seen in this disease disease

Others=Others=

:Dentatorubral pallidoluysian atrophy (DRPLA), :Dentatorubral pallidoluysian atrophy (DRPLA),

:Hallervorden Spatz disease, :Hallervorden Spatz disease,

:Spinocerebeller ataxia (SCA) 2, :Spinocerebeller ataxia (SCA) 2, 33, 6 and 17, 6 and 17

:Neuroacanthocytosis. :Neuroacanthocytosis.

Secondary Akinetic Rigid Secondary Akinetic Rigid SyndromesSyndromes

(Drugs) (Drugs) Drugs that deplete presynaptic dopamine Drugs that deplete presynaptic dopamine

storesstores= :Reserpine = :Reserpine :Tetrabenazine :Tetrabenazine

Block dopamine receptorsBlock dopamine receptors= = :Phenothiazines (chlorpromazine), :Phenothiazines (chlorpromazine), :Butyrophenones (haloperidol), :Butyrophenones (haloperidol), :Thioxanthines (flupenthixol) :Thioxanthines (flupenthixol) :Benzamides (sulpiride) :Benzamides (sulpiride)

Neuroleptics causeNeuroleptics cause parkinsonian symptoms with parkinsonian symptoms with clinical signs in 20-40% patients clinical signs in 20-40% patients

Secondary Akinetic Rigid Secondary Akinetic Rigid SyndromesSyndromes

(Toxins)(Toxins) MPTP.MPTP. Ethanol.Ethanol. Methanol.Methanol.

Secondary Akinetic Rigid SyndromesSecondary Akinetic Rigid Syndromes

(Metabolic and autoimmune)(Metabolic and autoimmune)

Acquired liver diseases.Acquired liver diseases.

Hashimoto’s thyroiditis.Hashimoto’s thyroiditis.

EPM.EPM.

Autoimmune-APAS.Autoimmune-APAS.

SCLC.SCLC.

Secondary Akinetic Rigid Syndromes Secondary Akinetic Rigid Syndromes Infections and post-infectious Akinetic rigid syndromesInfections and post-infectious Akinetic rigid syndromes

Encephalitis lethergica.Encephalitis lethergica. SSPE.SSPE. JE.JE. PM-Mycoplasma pneumoniae,PM-Mycoplasma pneumoniae, TBM.TBM. Kuru,CJD,GSS.Kuru,CJD,GSS.

Secondary Akinetic Rigid SyndromesSecondary Akinetic Rigid Syndromes(Misc)(Misc)

Trauma=Dementia pugilistica.Trauma=Dementia pugilistica. Temporal/frontal lobe glioma.Temporal/frontal lobe glioma. Primary CNS lymphoma,Primary CNS lymphoma, Mesencephalic cyst.Mesencephalic cyst. Glioblastoma.Glioblastoma. Astrocytoma.Astrocytoma. Hydrocephalus.Hydrocephalus. Psychogenic.Psychogenic.

CausesCauses

Electromagnetic, Physics, trauma, Radiation Causes Electromagnetic, Physics, trauma, Radiation Causes Hypothermia, accidental/exposureHypothermia, accidental/exposure

Infectious Disorders (Specific Agent) Infectious Disorders (Specific Agent) Encephalitis, Eastern equineEncephalitis, Eastern equine TetanusTetanus Creutzfeld-JakobCreutzfeld-Jakob disease disease Parkinsonism, Parkinsonism, postencephalitispostencephalitis type type

Metabolic, Storage Disorders Metabolic, Storage Disorders KernicterusKernicterus

Congenital, Developmental Disorders Congenital, Developmental Disorders MyoclonicMyoclonic dystonia/DYT11 (11q23) dystonia/DYT11 (11q23)

CausesCauses Hereditary, Familial, Genetic Disorders Hereditary, Familial, Genetic Disorders

OromandibularOromandibular facial facial dystoniadystonia syndrome syndrome Huntington's choreaHuntington's chorea Paraplegia, spastic hereditaryParaplegia, spastic hereditary CanavanCanavan disease disease DystoniaDystonia musculorummusculorum deformansdeformans LeukodystrophyLeukodystrophy, , KrabbeKrabbe MyotoniaMyotonia atrophicaatrophica ( (Steinert'sSteinert's disease) disease) MyotoniaMyotonia congenitacongenita ( (ThomsensThomsens d.) d.) OlivopontocerebellarOlivopontocerebellar degeneration degeneration PallidopyramidalPallidopyramidal hereditary syndrome hereditary syndrome Parkinsonism, juvenile, of HuntParkinsonism, juvenile, of Hunt Torsion Torsion dystoniadystonia, primary/idiopathic, primary/idiopathic Familial Basal ganglion calcification (Familial Basal ganglion calcification (Fahr'sFahr's syndrome) syndrome)

Causes Causes Usage, Degenerative, Necrosis, Age Related Usage, Degenerative, Necrosis, Age Related

Disorders Disorders NeurologicNeurologic degenerative disorder degenerative disorder Parkinson's disease (paralysis Parkinson's disease (paralysis agitansagitans)) Dementia, Dementia, LewyLewy-body type-body type Parkinsonism-dementia complexParkinsonism-dementia complex Primary lateral sclerosisPrimary lateral sclerosis Progressive Progressive supranuclearsupranuclear palsy palsy MetachromaticMetachromatic leukodystrophyleukodystrophy Shy-Shy-DragerDrager syndrome syndrome Gerstmann-Straussler-ScheinkerGerstmann-Straussler-Scheinker (GSS) Disease (GSS) Disease Multiple System Atrophy/Shy Multiple System Atrophy/Shy DragersDragers plus plus

CausesCauses

Relational, Mental, Psychiatric Disorders Relational, Mental, Psychiatric Disorders Schizophrenia, catatonic typeSchizophrenia, catatonic type Catatonia, lethal typeCatatonia, lethal type CatatoniaCatatonia

Anatomic, Foreign Body, Structural Anatomic, Foreign Body, Structural Disorders Disorders Basal ganglion hemorrhageBasal ganglion hemorrhage Basal ganglion mass lesionBasal ganglion mass lesion

CausesCauses

Vegetative, Autonomic, Endocrine Disorders Vegetative, Autonomic, Endocrine Disorders SeizuresSeizures Malignant hyperthermiaMalignant hyperthermia

Reference to Organ System Reference to Organ System Motor neuron diseaseMotor neuron disease ParkinsonianParkinsonian syndrome syndrome Basal ganglion lesion/disorderBasal ganglion lesion/disorder

Idiopathic, Unclassified Disorders Idiopathic, Unclassified Disorders Stiff-man syndromeStiff-man syndrome

CausesCauses

Drugs Drugs Monoamine Monoamine oxidaseoxidase inhibitor Administration/Toxicity inhibitor Administration/Toxicity PhenothiazinePhenothiazine dystonicdystonic/toxicity/toxicity Drug induced Drug induced DystoniaDystonia reaction/acute. reaction/acute. Monoamine Monoamine oxidaseoxidase inhibitor crisis inhibitor crisis Methaqualone (Sopor/Quaalude) Methaqualone (Sopor/Quaalude)

Administration/Toxicity Administration/Toxicity Camphor tree/camphora herbal/intake Camphor tree/camphora herbal/intake Hyperserotoninemia/serotonin syndromeHyperserotoninemia/serotonin syndrome

CausesCauses Poisoning (Specific Agent) Poisoning (Specific Agent)

Amphetamine/Speed toxidrome/Acute Amphetamine/Speed toxidrome/Acute Ecstacy drug/MDMA/MethylenedioxyMA Ecstacy drug/MDMA/MethylenedioxyMA Phencyclidine (Angel dust) intoxication Phencyclidine (Angel dust) intoxication Methamphetamine/Speed/Amphetamine chronic/abuse Methamphetamine/Speed/Amphetamine chronic/abuse Black widow spider bite Black widow spider bite Camphorated oils poisoning Camphorated oils poisoning Carbon monoxide poisoning/exposure Carbon monoxide poisoning/exposure Metaldehyde poisoning Metaldehyde poisoning Ammonia exposure/inhalation Ammonia exposure/inhalation Haff disease/poisoning Haff disease/poisoning Strychnine poisoning Strychnine poisoning Trichothecene mycotoxin/weapon exposure Trichothecene mycotoxin/weapon exposure Manganese poisoning Manganese poisoning MPTP/Meperidine designer drug/synthetic MPTP/Meperidine designer drug/synthetic Nux vomica/Strychnos herbal/intake Nux vomica/Strychnos herbal/intake

Organ Poisoning (Intoxication) Organ Poisoning (Intoxication) Neuroleptic malignant syndromeNeuroleptic malignant syndrome

akinetic rigid syndrome

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