Case Presentation

Dr. Md Rashedul Islam FCPS, MRCP(UK)

Registrar, Neurology, BIRDEM

A 60 years old diabetic male hailing from Baridhara,Dhaka got admitted in BIRDEM General Hospital under department of Neurology, on 04.05.14 with the complaints of :

• Difficulty in walking for 1 year• Slurring of speech for 10 months

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According to the statement of the patient, he was reasonably well 1 year back. Then he developed walking difficulty which was progressive, associated with problems with maintaining balance & recurrent history of fall. It was not associated with low back pain, tingling of feet, incontinence of urine, head injury, fever, weight loss.

H/O Present illness

He also gave history of slurring of speech for 10 months which was progressive, monotonous, hypophonic, indistinct. He had difficulty in pronouncing consonants but no fatigability, swallowing difficulty was present. On detailed query, he gave history of memory disturbance, behavioral problems, insomnia, clumsiness & impaired hand writing.

H/O PAST ILLNESS: Nothing contributory

SOCIOECONOMIC HISTORY: He belongs to a upper class family

PERSONAL HISTORY: He is non alcoholic, non smoker.

FAMILY HISTORY Nothing significant

TREATMENT HISTORY: Tab. Metformin

Tab. Vit B complex

General examination:

Appearance: Masked face Startled expression

General examination:Built: Overweight(BMI- 27.4 kg/m2) Decubitus: on choiceOedemaAnaemiaJaundiceCyanosisDehydrationClubbing AbsentKoilonychiaLeukonychia

General examination:

Neck vein: not engorgedThyroid: not enlargedLymph node: not palpableSkin pigmentation & body hair distribution:

normalPulse: 86 b/minBP: 130/80 mmHgTemp:98 FRR: 16 breaths/min

Higher psychic function :

Mild cognitive dysfunction(MMSE- 27/30)

Slowed cognitive processing, sequencing,

planning.

Apathy

Speech: Spastic type of dysarthria

Cranial nerves :

Slow vertical saccades

Vertical gaze palsy was improved with

vestibular ocular reflex & Bell phenomenon

GCS: 15/15

NERVOUS SYSTEM EXAMINATION

Speech

Muscle Rt. UL Lt. UL Rt. LL Lt. LL

Bulk Normal Normal Normal Normal

Tone Increased

Increased

Increased

Increased

Power 4/5 4/5 4/5 4/5

Involuntary movement

Absent Absent Absent Absent

MOTOR FUNCTION:

ReflexReflex B T S K A Abd. PlantarPlantar

RightRight ++ ++ ++ ++ ++ N FlexorFlexor

LeftLeft ++ ++ ++ ++ ++ N FlexorFlexor

Sensory system:

Pain Temp Touch

Vibration

Position sense

Right upper limb

N N N N N

Right lower limb

N N N N N

Left upper limb

N N N N N

Left lower limb

N N N N N

• Sign of Meningeal irritation - Absent

• Cerebellar sign : Absent

• Gait – Broad based unstable gait

• Others-

Bradykinesia

Axial rigidity was more pronounced that appendicular

Systemic examinations

Cardiovascular systemRespiratory system: Alimentary system: no abnormality detected Genitourinary system:

SALIENT FEATURE

A 60 years old diabetic gentleman presented with progressive walking difficulty for 1 year with problems of maintaining balance, recurrent history of fall. It was not associated with low back pain, tingling of feet, incontinence of urine. He also had progressively slurring, monotonous, hypophonic, indistinct speech with difficulty in pronouncing consonants but no fatigability.

He also had history of features of dementia, insomnia, clumsiness & impaired hand writing. On examination, he had masked face, mild cognitive impairment, Spastic dysarthria, vertical gaze palsy & broad based, unstable gait. Bradykinesia, marked postural instability, predominant axial rigidity, diminished muscle power was also evident. Other systemic examination was normal.

SALIENT FEATURE

Provisional Diagnosis

• Diabetes Mellitus Type 2 • Parkinson-plus syndrome possibly

progressive supranuclear palsy

Differential Diagnosis

• Other Parkinson-plus syndromesParkinsonism-dementia complexCorticobasal degeneration

• Pseudobulbar palsy due to bilateral hemishpere infarction

Investigations

CBC: Hb % - 14.2 mg/dl

WBC -7000 cu/mm Neu-65 % Lymph- 17.8 % Mono -5.9 % Eosino- 1.1% Platelet- 195000

ESR- 20mm in 1st hour

ALT: 28 iu/LAST: 32 iu/L

Lipid profile: TG: 136 mg/dl T. Chol : 122 mg/dl LDL: 55 mg/dl HDL: 40 mg/dl

Liver Function test:

Serum electrolytes Na: 141 mmol/l

K: 4.8 mmol/l Cl: 108 mmol/l HCO3: 23 mmol/l

Renal Function Test

SS. Urea: 21 mg/dl S. Creatinine: 1.0mg/dl

S. Urea: 32 mg/dl.

Sugar - Nil

Albumin – Nil

Ketone- Nil

Epi. cell: A few /HPF

Pus cell: 1-2 /HPF

RBC: Nil

Urine R/M/E

Chest X ray P/A view: Normal

ECG: Normal

MRI of Brain: Atrophy of dorsal midbrain giving rise to “mouse ears” appearance

MRI of Brain: Atrophy

of dorsal midbrain giving rise to “mouse ears” appearance

MRI of Brain:

Final diagnosis:

• Diabetes Mellitus Type 2 • Progressive supranuclear palsy

Management

• Counseling • Balanced diet• Tab Metformin• Tab. Ropirinole• Physiotherapy• Rehabilitation specialist consultation• Speech therapy• Consultation with opthalmologist

Follow up

Patient visited us after 2 months with improvement

of postural instability & reduced frequency of falls

Progressive supranuclear palsy (PSP)

Progressive supranuclear palsy

• Progressive supranuclear palsy (PSP), also known as Steele-Richardson-Olszewski syndrome, is a neurodegenerative disease that affects cognition, eye movements, and posture.

• It was first described as a clinicopathologic entity in 1964.

Pathologically, PSP is defined by the accumulation of neurofibrillary tangles in the brain. Different rates, localizations, and patterns of the accumulation of phosphorylated tau protein may account for the variation in clinical phenomena seen in patients with PSP.

Pathology

Bilateral loss of neurons and gliosis in the periaqueductal gray matter, superior colliculus, subthalamic nucleus, red nucleus, pallidum, dentate nucleus, and pretectal and vestibular nuclei, and to some extent in the oculomotor nucleus.

Etiology

The cause of PSP remains unknown. Most cases appear to be sporadic. Both environmental and genetic influences have been postulated.

HistoryThe cardinal manifestations:• Supranuclear ophthalmoplegia• Pseudobulbar palsy• Prominent neck dystonia• Parkinsonism• Behavioral, cognitive, and gait disturbances that

cause imbalance• Frequent falls/impaired postural reflexes

History

• Focal or segmental dystonia in the form of limb dystonia or blepharospasm

• Micturition disturbances, including urinary incontinence

• Progressive apraxia of speech, nonfluent aphasia, or a combination thereof

• Photophobia

Physical examination• Poor postural reflexes, axial rigidity greater than

appendicular rigidity, and dysarthria• Absence of cogwheeling or tremor• Widely based and unstable gait• Bradykinesia with masked facies and a startled

expression• Retrocollis may be present

Visual signs

• Supranuclear ophthalmoplegia • Downgaze typically involved

before upgaze• Impairment of convergence eye

movements• Eyelid retraction, eyelid opening

or closing apraxia, blepharospasm, or lid lag

Diagnostic Considerations

The diagnosis of progressive supranuclear palsy (PSP) is clinical. The key features typically develop over time; although the full-blown picture may be relatively easy to recognize, the early or restricted cases are much more challenging.

Diagnostic aids

Atrophy of the dorsal mesencephalon (superior colliculi, red nuclei) giving rise to a "mouse ears"

Diagnostic aids

CSF contains both extended and truncated tau forms, and the truncated-to-extended ratio is significantly lower in PSP than in other neurodegenerative disorders

Management

• Treatment of progressive supranuclear palsy (PSP) is challenging at best.

• No medication is effective• Dopamine agonists,

tricyclic antidepressants, may provide modest symptomatic improvement.

Management

• Only a few patients respond to dopaminergic or anticholinergic drugs, and responses often are short-lived and incomplete.

• Electroconvulsive therapy (ECT) may ameliorate motor symptoms in some patients with PSP.

Management

• Rehabilitation• Treatment of the sleep

difficulties• Observing the decline of

these patients and the limitations of treatment is a frustrating ordeal for all involved.

Thank you