UPPER - PowerPoint Presentation

Slide1

UPPER

MOTOR NEURON HEMIPLEGIA-PARAPLEGIA

DR NASIR FAROOQ BUTTASSISTANT PROFESSORDEPARTMENT OF MEDICINE KING EDWARD MEDICAL UNIVERSITYMAYO HOSPITAL LAHORE Slide2

UPPER MOTOR NEURONS

(UMN) start from cerebral cortex, passes through internal capsule,

corticospinal tracts, and ends at cranial nerve nuclei in brain stem or anterior horn cells of spinal cord at different levels. The axons of upper motor neurons are contained within the pyramidal system, which is composed of the corticospinal (pyramidal) and corticobulbar tracts. LOWER MOTOR NEURONS (LMN) start from nuclei of cranial nerves or anterior horn cells of spinal cord, extend through cranial /spinal nerves and ends at motor end plate of concerned muscle.Slide3

.

.Slide4

UPPER MOTOR

NEURONS LESION (UMNL).Slide5

Pyramidal Drift Of An Upper Limb

Normally

, the outstretched upper limbs are held symmetrically, when the eyes are closed. With a pyramidal lesion, when both upper limbs are held outstretched, palms uppermost, the affected limb drifts downwards and medially. The forearm tends to pronate and the fingers flex slightly. This sign is often first to emerge, sometimes before weakness and/or reflex changes become apparent.Slide6

Pyramidal Drift Of An Upper LimbSlide7

Weakness And Loss Of Skilled Movement

A unilateral pyramidal lesion

above the decussation in the medulla causes weakness of the opposite limbs. When acute and complete, this weakness will be immediate and total, a hemiplegia, e.g. following an internal capsule infarct.

With

slowly progressive

lesions (e.g. a hemisphere

glioma

) a characteristic pattern of weakness emerges – a hemiparesis.

There is also

loss of skilled movement

– fine finger and toe control

diminishes

.Slide8

Changes In Tone And Tendon Reflexes

An

acute lesion of one pyramidal tract (e.g. internal capsule stroke) causes initially FLACCID paralysis with loss of tendon reflexes. Increase in tone follows, usually within several days due to loss of inhibitory effects of the corticospinal pathways and an increase in spinal reflex activity.

SPASTICITY

is

characterized by sudden changing resistance to passive movement – the clasp-knife effect.

Relevant

TENDON REFLEXES become exaggerated

;

CLONUS

may

emerge.Slide9
Slide10

Changes In Superficial Reflexes

Abdominal (and

cremasteric) reflexes are abolished on the side affected.The Babinski sign. Slide11

Patterns of UMN disorders

There are three main patterns:

HEMIPARESIS means weakness of the limbs on one side; it is usually caused by a lesion in the brain and occasionally in the cord.PARAPARESIS means weakness of both lower limbs and usually diagnostic of a cord lesion; bilateral brain lesions occasionally cause paraparesis.

TETRAPARESIS

(syn.

quadriparesis

) means weakness of four limbs.Slide12

Extrapyramidal system

The extrapyramidal system is a general term for

BASAL GANGLIA motor systems, i.e. corpus striatum (caudate nucleus + globus pallidus + putamen), subthalamic nucleus, substantia nigra and parts of the thalamus. In basal ganglia/ extrapyramidal disorders, two features (either or both) become apparent, in limbs and axial muscles:

Reduction in speed (

bradykinesia

, meaning slow movement) or

akinesia

(no movement), with muscle

rigidity

Involuntary

movements

(e.g. tremor, chorea,

hemiballismus

,

athetosis

, dystonia).Slide13

Extrapyramidal system

Extrapyramidal disorders are classified broadly into:

Akinetic-rigid Syndromes where poverty of movement predominates D

yskinesias

where there are involuntary

movements.

The

most common extrapyramidal disorder is

Parkinson’s disease.Slide14

Click to add title

Click to add textSlide15

Case Scenario

An 83-year-old right-handed woman was eating dinner when she suddenly slumped to the right and her speech became slurred and nonfluent. Her vascular risk factors were hypertension and dyslipidemia, and the only other past medical history of interest was long-term hypothyroidism and a curative mastectomy for breast cancer. The patient was on two antihypertensive medications and a statin, and was not receiving any antiplatelet medication. Her husband immediately called the emergency medical services.Slide16

Case Scenario

On Examination Pulse is 84/min (regular), BP: 180/105 mm Hg. Angle of mouth is deviated towards left (UMN type Right 7

th CN)Tone is decreased in right upper and lower limb,Power is 2/5 in right right upper and lower limb,Reflexex are absent in right right upper and lower limb.Right Planter is upgoing

.

Tone, power, reflexes are normal on left side with

downgoing

planter

What is your Diagnosis????Slide17

STROKE

This patient has had STROKE causing Right sided uncrossed Hemiplegia with UMN type Facial Nerve Palsy

which can be caused by a vascular event such as thrombosis, embolism or hemorrhage or by neaplasm of brain (aetiology). The patient is limited by hemiplegia (functional status)Slide18

Stroke

DEFINITION: Stroke is defined

as a syndrome of rapid onset of cerebral deficit (usually focal) lasting >24 h or leading to death, with no cause apparent other than a vascular one.Slide19
Slide20

Stroke

Ischaemic stroke/infarction (80%)

ThromboticCardio-embolicLarge artery stenosisSmall vessel diseaseHypoperfusion

Haemorrhagic

stroke

(17

%)

Intracerebral

hemorrhage

(12

%)

Subarachnoid hemorrhage

(5

%)

Other

(3%),

e.g. arterial dissection, venous

sinus thrombosis

,

vasculitisSlide21

Types of Stroke

Depending upon the rate of developing and reversibility, stroke can be of following types:

Transient Ischemic Attack (TIA): means a brief episode of neurological dysfunction due to temporary focal cerebral or retinal ischemia without infarction, e.g. a weak limb, aphasia or loss of vision, usually lasting seconds or minutes with complete recovery.

Stroke-in-Evolution

:

If neurological deficit increases by step wise progression or there is slow uninterrupted progression, it is called stroke-in-evolution. It may take 1-2 weeks to complete

Complete Stroke

: It means complete hemiplegia

RIND (Reversible

Ischemic Neurological

Deficit):

It is a term that is not widely used anymore.

It

means a stroke that the patient was able to fully recover from within a few weeks. Slide22

Types of Stroke

Depending upon the blood supply of affected area of brain, stroke can be of following types:

ANTERIOR CIRCULATION STROKE:Carotid systemPOSTERIOR CIRCULATION STROKE:

Vertebrobasilar

systemSlide23
Slide24
Slide25
Slide26

Risk Factor For Ischemic Stroke

Hypertension

DiabetesSmoking Lifestyle AlcoholHigh cholesterolAtrial fibrillation

Obesity

Severe carotid

stenosis

Sleep

apnoeaSlide27

Rarer Causes Of Stroke

Hyperviscosity

states.Thrombocythaemia, Polycythaemia Thrombophilia Anti-cardiolipin

and lupus anticoagulant antibodies

oral contraceptive.

Migraine

Vasculitis

Systemic lupus

erythematosus

(SLE),

Polyarteritis

Giant

cell arteritis

Granulomatous

CNS

angiitis

:Slide28

Rarer Causes Of Stroke

Amyloidosis.

HyperhomocysteinaemiaNeurosyphilisMitochondrial diseaseDrugs Sympathomimetic drugs

cocaine

Vasoconstrictors

neuroleptics

CADASIL (cerebral dominant

arteriopathy

with subcortical

infarcts and

leucoencephalopathy

)Slide29

Clinical Features of Stroke

SYMPTOMS:

Weakness of one half of bodyOnset – Sudden, can be gradualHeadache, vomiting, fitsUnconciousnessPast H/O TIAsH/O smooking, HTN, DM or IHD

Family H/O StrokeSlide30

Clinical Features of Stroke

SIGNS:

PULSE: Irregular (At. Fib), Slow (Raised ICP)BP: HighUnconciounessPupils: Small or Dilated

EYES:

Devited

towards on side (conjugate gaze)

Cranial Nerves

may be involved

Dysphasia, Dysarthria

Initially limbs may be

flaccid

with absent reflexes (Spinal Shock). This may persist for days, weeks or months and then Classical UMNL signs developSlide31

Clinical Features of Stroke

SIGNS:

Hemiplegic weakness of upper limb affects the shoulder abductor, elbow

extensors

,

wrist

and

finger

extensors

and

small

musles

of

hand

Hemiplegic

weakness

of

lower

limb

affects

hip

flexors

,

knee

flexors

and

dorsiflexors

and

evertors

of

the

footSlide32
Slide33

Clinical Features of Stroke

SIGNS:

Signs of meningial irritation (SOMI) – Subarachnoid HemorrhagesFundoscopy: Diabetic or

Hypertensive

Retinopathy

Carotid

Pulsation

:

weak

,

bruit

CVS

Exam

:

Murmur

eg

Mitral

stenosis

Homonymous

hemianopia

and

sensory

inattention

Speech

defectsSlide34
Slide35

Click to add title

Click to add textSlide36

Amaurosis

fugax

Refers to a transient loss of vision in one or both eyes due to embolism in retinal artery.This is a feature of TIA in carotid circulationSlide37

ISCHEMIC STROKE

Cerebral Thrombosis:

Occurs when patient is asleepAtherosclerotic narrowing of blood vesselMiddle cerebral art is commonly involvedPatient usually remain consciousHTN is a major risk factorClinical Features:

Pt

can not move one half of body

Dysphasia

Uncrossed hemiplegia

High BP

Carotid BruitSlide38

ISCHEMIC STROKE

Cerebral Embolism:

Sudden onsetPast history of TIAsSource of EmbolismCarotid artery atheroma (weak carotid pulse,bruit)Atrial Fibrillation or other arrythmias

Recent Myocardial Infarction

Valvular

Heart Disease

Infective Endocarditis

Clinical Features:

Sudden loss of power in one half of body

Conciouness

may be lost

Dysphasia

Uncrossed hemiplegia

Irregular Pulse

Signs of

valvular

heart disease

Carotid BruitSlide39

HEMORRHAGIC STROKE

Primary

Intracerebral Hemorrhage:H/O HTNInternal capsule is most common site. Other sites: subcortical white matter, pons, and cerebellum.Occurs when pt

is

engaged in some activity

Causes: HTN

,

Saccular

Aneurysm,

AV

Malformation, Bleeding Disorders

Clinical Features:

Sudden loss of power in one half of body

Headache, vomiting and loss of

Conciouness

Uncrossed hemiplegia

Fundoscopy

: PapilledemaSlide40

HEMORRHAGIC STROKE

Subarachnoid Hemorrhage:

Primary: Bleeding occurs directly into subarachnoid space.Causes: Saccular Aneurysm, AV Malformation, Bleeding

Disorders,

Vasculitis

Clinical Features:

Sudden onset of severe Headache with or without vomiting

SOMI are present

Focal neurological signs

Fundoscopy

:

Subhyloid

hemorrhage, Papilledema

Secondary

:

Extension

of

intracerebral

hemorrhage

into

subarachnoid

spaceSlide41

SPACE OCCUPYING LESION

Lesion which occupy the cranial cavity and displace or

destroy existing structuresCauses: Tumour, Abscess, Hematoma, TuberculomasSymptoms:H/O gradual onset of weakness developing over weeks to months.Headache and vomiting

There may be H/O trauma (Hematoma)

H/O waxing and waning of

concious

level (Subdural Hematoma)

H/O fever and ear discharge suggest an abscess.

Signs:

In addition to motor deficit, following signs may be present:

Bradycardia

Fundoscopy

……Papilledema

Investigations:

CT scan (with contrast), MRISlide42

Localization of Lesion

UNCROSSED HEMIPLEGIA

: Cranial nerves involvement and hemiplegia are on the same sideLesion is above brainstem on the side opposite to the hemiplegiaCROSSED HEMIPLEGIA: Cranial nerves are involved on one side and hemiplegia is on the

other

side

Lesion is

in

brainstem on the side

of cranial nerve involvement.

HEMIPLEGIA WITHOUT CRANIAL NERVES INVOLVEMENT:

Lesion is in spinal cord above C5 on the side of paralysis.Slide43

Localization of Lesion

UNCROSSED HEMIPLEGIA

: Lesion can be in Motor cortex, Corona Radiata or Internal CapsuleMotor cortex:Specific manifestation due to lesion of various lobes:Parietal Lobe

Temporal Lobe

Occipital Lobe

Frontal LobeSlide44

Localization of Lesion

PARIETAL LOBE

: Sensory cortex (Precentral Gyrus) demage only affects special sensory functionsLoss of cortical sensations:Two point discriminationAstereogonosis (inability to identify common objects by palpation with one hand while senses are intact)

Homonymous lower

quadrantanopia

Apraxia

(inability to perform a learned act in the absence of significant motor weakness.

Additional Features of Dominant Hemisphere

Gerstmann’s

synd

: Inability to calculate, write, distinguish between right and left, to recognize different fingers.

Alexia (inability to read), Bimanual

astereognosis

,

Sensory dysphasiaSlide45

Localization of Lesion

PARIETAL LOBE

: Additional Features of Non- Dominant HemisphereSensory inattention (when both side are touched separately patient can recognize them, but when they are touched simultaneously, side opposite to affected parietal lobe is ignored)Indifference to illness

Anosognosia

(

pt

is unaware of paralysis)

Autopagnosia

(

pt

ignores paralyzed body)

Spatial disorientation (

Pt

is unable to find his way around)Slide46

Localization of Lesion

TEMPORAL LOBE

: Auditory or Olfactory hallucinationAuditory or Visual hallucination Homonymous upper quadrantanopia of opposite sideDeja vu phenomenon (feeling of undue familiarity with surroundings)Automatism (lip smacking, swallowing, walking aimlessly or unconscious performance of highly skilled activity like driving a car)

OCCIPITAL LOBE

:

Visual

hallucination

Homonymous hemianopia of opposite sideSlide47

Localization of Lesion

PRE-FRONTAL LOBE :

Lack of initiative and concentrationLack of concern of consequences of any actionMicturition disturbanceGrasp Reflex is +vePRECENTRAL GYRUS (FRONTAL LOBE):Weakness of of opposite half of the bodyJacksonian

fits

Expressive, motor or non-fluent dysphasia if lesion is in the dominant hemisphere.Slide48

Localization of Lesion

UNCROSSED HEMIPLEGIA

: CORONA RADIATA:Although Weakness is more marked in one limb, whole half is involved to some extentINTERNAL CAPSULE:Motor and sensory fibere are very closely packed…so lesion cause

Dense complete hemiplegia

Facial nerve palsy of same side

Cortical sensory loss and hemianopia may occurSlide49

Localization of Lesion

CROSSED HEMIPLEGIA

: Midbrain:3rd /4th CN are involved on one side and hemiplegia of opposite sidePons:

5

th

, 6

th

, 7

th

and 8

th

CN nuclei are present in pons.

One or more of these CNs are paralyzed on one side and hemiplegia is on the other side.

Pupils are pinpoint but reactive to light

Hyperpyrexia may be present

Medulla Oblongata:

9

th

,

10

th

,

11

th

and

12

th

CN nuclei are present in

medulla.

One or more of these CNs are paralyzed on one side and hemiplegia is on the other side.Slide50

Localization of Lesion

SPINAL CORD.

Brown-Sequard syndrome (BSS) is a rare neurological condition characterized by a lesion in the spinal cord which results in weakness or paralysis (hemiparaplegia) on one side of the body and a loss of sensation (hemianesthesia

) on the opposite

side.

Causes:

Trauma

Non-traumatic: Tumor

(primary or

metastatic), Multiple sclerosis, Disk herniation,

Epidural

hematoma, Vertebral

artery

dissection, Transverse myelitis,

etc

Slide51
Slide52

Click to add title

Click to add textSlide53

MIDDLE CEREBRAL ARTERY

Supplies lateral surface of frontal,frontal,

perital and temporal lobesMost of the motor and sensory cortex Ecxept the part on medial side and control lower limb, internal capsuleTotal Occlusion:Uncrossed hemiplegiaHemianesthesiaGlobal DysphasiaHomonymous hemianopiaApraxia,

Agnosia

Partial occlusion

:

Any of the above features.

Lower limb is either not or minimally involvedSlide54

ANTERIOR CEREBRAL ARTERY

Supplies MEDIAL surface of hemisphereMotor and sensory cortex on medial side which control lower limb

Soon its origin it gives a penetrating branch (Heubner’s artery) which supply internal capsule (containing fibers of upper limb and face)Occlusion at the origin:Features are similar to total occlusion of MCBOcclusion of Heubner’s

artery

:

Weakness of upper limb and face

Occlusion

after the origin of

Heubner’s

artery

:

Weakness of

lower limb

Loss of cortical sensory function in lower limbSlide55

POSTERIOR

CEREBRAL ARTERY Branch of Basilar artery

Supplies occipital lobe which include visual cortexOcclusion:Visual field loss Homonymous hemianopiaVisual agnosia Disorders of reading Disorders of color vision

Memory impairment

Motor dysfunction Slide56

Click to add title

Click to add textSlide57

Click to add title

Click to add textSlide58

LATERAL MEDULLARY SYNDROME

Also called posterior inferior cerebellar artery (PICA) thrombosis and

Wallenberg’s syndrome) Acommon example of brainstem infarction presenting as acute vertigo with cerebellar and other signs. It follows Thromboembolism in the PICA or its branches, vertebral artery thromboembolism or dissection. Features depend on the precise structures damaged.

MEDIAL MEDULLARY SYNDROME

C

haracterized

by a set of clinical features resulting from occlusion of the anterior spinal artery. This results in the infarction of medial part of the medulla oblongata.Slide59
Slide60
Slide61

Investigations - STROKE

The purpose of investigations in stroke is:To

confirm the clinical diagnosis and distinguish between hemorrhagic and thromboembolic infarction;To look for underlying causes and to direct therapy;To exclude other causes, e.g. tumour.Sources of embolus should be soughtSlide62

Investigations of Stroke

Brain

Computed Tomography (CT) ScanMagnetic resonance imaging (MRI)Computed Tomography Arteriogram and Magnetic Resonance Arteriogram: Aneurysm, AV malformationCarotid Ultrasound

ECG and

Echocardiography

Lumber Puncture:

Subarachnoid

HemorrhageSlide63

Investigations - STROKE

Routine bloods (for ESR, polycythaemia

, infection, vasculitis, thrombophilia, syphilitic serology, clotting studies, autoantibodies)Blood Sugar LevelFasting Lipid profile Chest X-ray ECG and Echocardiography

Carotid Doppler studies

Lumber

Puncture:

Subarachnoid

HemorrhageSlide64

Investigations - STROKE

Imaging in Acute Stroke.

Non-contrast CT: will demonstrate haemorrhage immediately but cerebral infarction is often not detected or only subtle changes are seen initially.Slide65
Slide66
Slide67
Slide68
Slide69
Slide70
Slide71

CASE SCENARIO

A 40 year old male presented in OPD with C/O gradually progressive weakness of both legs for the last one month. He is also C/O backache. There is no H/O Trauma.There is H/O Numbness

and paresthesias in legs. He is unable to control his urine.Slide72

CASE SCENARIO

On Examination Pulse is 84/min (regular), BP: 180/105 mm Hg. Bulk is normal in both legs

Tone is increased in both legs,Power is 3/5 in right leg and 2/5 in left leg.Knee and Ankle Reflexex are Brisk bilaterally.

B

oth Planters are

upgoing

. Ankle Clonus is present on both sides.

Upper limbs are normal.

Tenderness at back of chest.

What is your Diagnosis????Slide73

This patient has SPASTIC PARAPLEGIA (Lesion) at T10 spinal level due to Cord compression (aetiology). It is complicated by bladder involvement (functional status)Slide74

SPASTIC PARAPLEGIA

HISTORY:

Ask about onset, duration and course of symptomsBack pain: whether localizedAsk about radicular painNumbness and parasthesia particularly below the level of lesionWeakness: whether gradual or suddenSphincter control and bladder sensationFunctional status: wheelchair transfers, walking aids, orthotic shoes and whether house has been modified for the patient’s disabilityTake a family history (hereditary spastic paraplegia)Take a history of birth anoxia (cerebral palsy)History of urinary infections, pressure sores and deep venous

thromboses

.Slide75

SPASTIC PARAPLEGIA

EXAMINATION:

Increased tone in both lower limbs, Hyperreflexia, Ankle clonusWeakness in both lower limbsThen proceed to:Check the sensory levelExamine the spine (spinal tenderness or deformity

)

Tell the examiner that you would like to do the

following:

Check sacral sensation

Examine

the hands to rule out involvement of upper limbs

Check

for cerebellar signs (multiple sclerosis,

Friedreich’s

ataxia).Slide76

SPASTIC PARAPLEGIA

EXAMINATION:

Try to localize the level of lesion using the following:UMNL signs in lower limb and LMNL signs in upper lims: lesion of

C5–T1.

UMNL signs in lower limb and

Wasting of small muscles of hand:

lesion of

C8–T1

).

Spasticity of the lower limb alone:

lesion of thoracic cord (T2–L1).

Flaccid

Paraplegia:

Lesion

below L1Slide77

SPASTIC PARAPLEGIA

Paraplegia means UMN type weakness of both lower limbs.

CAUSES:Spinal Cord CompressionTransverse MellitusSubacute combined degenration

of spinal cord

Friedreich’s

Ataxia

Amyotrophic Lateral Sclerosis (MND)

Spinal cord infarction

Hereditary

Spaatic

parapresis

Tabes

dorsalis

(

Neurosyphilis

)

Syringomyelia

Intracranial Lesions

Parasagital

Meningioma

Thrombosis of Sagittal SinusSlide78

SPINAL CORD COMPRESSION

CAUSES

A. EXTRADURAL:Vertebral Traumatic FractureMyelomaTuberculosis of SpineMetastases (breast, prostate, lungs)

Traumatic and degenerative lesions of disc

B. EXTRAMEDULLARY

:

Meningioma

AV Malformation

C. INTRAMEDULLARY

:

Spinal Cord TumorsSlide79

SPINAL CORD COMPRESSION

Symtoms

:H/O Backache, aggrevated by coughing, sneezing and strainingPain in the distribution of nerve rootH/O TraumaWeakness is usually gradually progressive except in trauma when it is of sudden onset.Numbness and paresthesia in lower limbsMicturation-urgency or

hesistancy

Signs:

Local examination of the vertebral column may reveal deformity or tenderness

Sensory loss with definite upper limitSlide80

SPINAL CORD COMPRESSION

Investigations:X- Rays of vertebral column may show reduce disc space, bony

errosions, osteolytic or osteosclerotic lesions, vertebral collapse or fracture.MRIRoutine InvestigationsSlide81

TRANVERSE MYELITIS

Inflamation of spinal cord

Can present with paraplegia or quadriplegiaRespiratory paralysis can occurCauses: Unknown 60 % (viral infections or post-infectious??)Remaining 40 % associated with autoimmune disorders such as multiple sclerosis, neuromyelitis optica

, systemic lupus erythematous,

Sjogren’s

syndrome

and

sarcoidosis

Slide82

TRANVERSE MYELITIS

4 classical

Symptoms:Backache/discomfort is first symptomsRapidly progressive weakness of lower limbsSensory symptoms such as numbness or tinglingSphincter disturbances (Bladder/bowel) are common

Signs

:

UMN signs in lower limbs

Sensory

Level

Investigations

:

Lumber Puncture with CSF Exam Lymphocytosis

X-Ray and MRI to rule out Cord compression Slide83

Subacute

Combined Degeneration Of Spinal Cord

Due to Vit B12 DeficiencyDegeneration of Posterior columns and pyramidal tractPeripheral NeuropathySymptoms:

Symptoms of anemia

Gradual progressive weakness of legs

Numbness, tingling and

hyperesthesias

Signs:

Pallor

Paraplegia, Ankle Jerk Absent, Up going planters.

Sense of position and vibration are lostSlide84

Subacute

Combined Degeneration Of Spinal Cord

Investigations:Peripheral Blood Film: Macrocytosis (MCV is raised), Hypersegmented NeutrophilsPancytopeniaMild unconjugated hyperbilirubinemia (du to ineffective erythropoisis.Serum B12 is low. Elevated LDH,

Bonemarrow

biopsy:

Megaloblasts

Schilling Test

Causes of

Vit

B12 Deficiency:

Nutritional

def

,, Pernicious anemia, Bacterial overgrowth and blind loop syndrome, Diverticulosis,

Diphyllobothrium

latum

infestation, diseases of terminal ileum (

Crohn’s

disease,

ileocecal

TB)Slide85

Friedreich’s

Ataxia

Familial Autosomal RecessiveSlowly progressive degeneration of Posterior columns, Pyramidal

tracts,

S

pinocerebellar

tracts and

P

eripheral

nerves

Age of onset: 8 – 16 years

Slowly progressive weakness of legs and unsteadiness on walking

UMN type weakness of legs

Absent Ankle jerk

Planters

up going

Sense of position and vibration are lostSlide86

Friedreich’s

Ataxia

Sensory Ataxia, Romberg’s sign may be +veCerebellar Signs (nystagmus, scanning speech, intension tremors) may be present

Associated features:

Pes

cavus

(high arched foot)

Kyphoscoliosis

Spina

Bifida

Hypertrophic Obstructive

Cardiomuopathy

(HOCM)Slide87

Pes

Cavus

Causes:Hereditary Motor and Sensory Neuropathy Type 1 (Charcot-Marie-Tooth disease) Friedreich's

AtaxiaSlide88

Causes Of Absent

Ankel

Jerk And Up Going Planters:Friedreich's ataxiaSubacute combined degeneration of the spinal cordMotor neurone diseaseSyphilis (

taboparesis

)

Combined

conus

medullaris

and

cauda

equina

lesions

Combination

of common problems e.g. diabetic peripheral neuropathy and cervical

spondylosisSlide89

CASE SCENARIO

A

65 year old male presented in OPD with complains of slowly progressive weakness of legs with numbness, tingling and hyperesthesias. He is a strict vegetarian. On

examination

he has

spastic

parapresis

with absent ankle jerk and

upgoing

planters. Sense of position and vibration are

lost.

His hemoglobin

is 6

gm

/dl.

What

is most likely diagnosis?Slide90

CASE SCENARIO

Answer:

Subacute Combined Degeneration Of Spinal CordSlide91