51 year old male with gaze palsy

 NEUROLOGY DIARIES:

51 year old male ,Carpenter by occupation came with the chief complaints of 

Inability to look at bright lights (R eye>Left eye) since 6months

Decreased eyeball movements and slowing of activities since 6months

Low volume of speech since 6months

Stiffness of right upper limb since 6months

HOPI:Patient was apparently asymptomatic 6 months ago.Since 6months patient has c/o inability to look at bright lights,Photophobia since 6 months,more in right eye compared to left eye associated with watering from eyes on sun exposure,not associated with redness of eyes or pain in eyes.No c/o discharge,burning sensation of eyes.c/o diplopia on looking at objects occasionally.

Patients wife notices that patient has decreased eyeball movements in both eyes and patient is turning his entire head to look sideways and above and downwards.

C/o slowness of activities with right upper limb such as eating and combing hair since 6months.Patient is taking food to the mouth slowly and is also making a odd posture involuntarily while eating food with excess flexi on at wrist and elbow due to which patient has difficulty eating food associated with stiffness of right upper limb.

No c/o difficulty in getting up from squatting position except for the feeling of loss of balance while getting up.No difficulty climbing stairs but patient often flexes his neck to look at the ground while walking as patient is not able to see the ground with his head looking straight.

No c/o slippage of slippers,difficulty lifting the neck off the pillow,rolling over in bed

No c/o tingling,numbness,pins and needle sensations

No c/o unsteadiness walking in the dark,patient is able to feel the clothes.

No c/o neck pain,back pain,LOC,delusions,head injury,hallucinations,emotional or behavioural disturbances.

C/o altered smell perception

Patients wife complained that she is able to see dropping of right eyelid and right eye is appearing smaller compared to left eye.

No difficulty in closing or opening the eyelids.No difficulty in hearing,swallowing,deviation of mouth,able to sense facial sensations.

C/o low volume of speech since 6months.

Able to move neck in all directions and able to roll tongue over and push food backwards.

C/o feeling of swaying and loss of balance while walking.H/o fall 1month ago while climbing stairs.

No c/o spillage of food while taking to the mouth

No c/o bowel,bladder disturbances,sweating,palpitations,chest pain,giddiness,headache ,vomitings,diarrhoea,fever

No c/o sleep disturbances,involuntary movements.

PAST HISTORY:

H/o right hemiparesis 10 years ago(?ischemic stroke) which recovered over 5 years

K/c/o HTN used medication of few years and stopped.

Not a k/c/o DM,CKD,CAD,TB,epilepsy

PERSONAL HISTORY:Mixed diet

Appetite and sleep normal

Bowel and bladder regular

FAMILY HISTORY:

No significant family history 

CNS EXAMINATION:

HMF:

MMSE:18/30

FAB score:4

Cranial nerves

1.Patient not able to perceive smell with both nostrils

                                R                       L

2.Visual acuity      6/18                 6/18(with spectacles)

                              6/36                 6/24(without spectacles)

   Filed of vision    :patient not cooperative for confrontation test

 Colour vision      Normal

 Fundus:                Bilateral temporal pallor

3,4,6: right eye ptosis+

          Impaired vertical eye movements 

          Slow horizontal saccades

          DLR,ILR normal

          AR:absent

          No nystagmus 

Other cranial nerves:normal

MOTOR SYSTEM: R               L

Bulk             Normal and equal on both sides

Tone   UL        Rigidity+          Normal 

                  (Increased with synkinesis)

           LL        Normal              Normal

Power :5/5 all 4 limbs

Reflexes: corneal,conjunctival,abdominal +

     B    T      S      K     A     Plantar

R 3+   3+    3+    3+    2+     Flexion

L 3+    3+    3+    3+    2+     Flexion

Primitive reflexes:Meyers sign+

No palmomental reflex,no grasp reflex 

GAIT: abnormal tonic posture of right upper limb with reduced arm swing on right side

No involuntary movements.

SENSORY SYSTEM:

Pain,Fine touch,vibration,position sense normal in all 4 limbs

CEREBELLAR SIGNS:absent

Patient is not able to do dysdidokokinesia due to the presence of applause sign.

ANS:normal

Spine ,cranium :normal

EXTRA PYRAMIDAL SYSTEM:

Finger tap test:Bradykinesia +

No axial rigidity on shoulder shaking test

Right upper limb Assymetrical rigidity

Reduced arm swing on right side

Dystonic posture of right upper limb

Mask like facies

Eyelid closing apraxia+(unable to sustain eye closure on command but blinking is normal)

Vertical gaze palsy

Slow horizontal saccades

Hypophonia

Blink rate 2/min

Meyers sign+

Procerus sign of frontalis overactivity+

Applause sign of frontal disinhibition +

Retroplusion test/pull test positive 

Rocket sign absent

Final Diagnosis: PROGRESSIVE SUPRANUCLEAR PALSY-OCCULOMOTOR DYSFUNCTION VARIENT

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder with clinical features that include postural instability with frequent falls, bulbar palsy, truncal rigidity, progressive cognitive impairment and changes to eye movements, primarily in the form of vertical supranuclear palsy. 

Photophobia, which is described by patients as an acute sensitivity to light that can be painful in nature, has also been noted as a frequent symptom in PSP

Reference link: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8299976/

VARIENTS OF PSP: