Parkinson Plus Syndrome - Multiple System Atrophy: Case Report. Poster used in CMC MAC 2021. ABSTRACT A 61yr/Male, K/C/O T2DM & Parkinson’s disease(PD) on T.Metformin and T.Syndopa for 3 years, presented to us with complaints of unsteadiness of gait, dysarthria, bilateral upper limb tremor. These symptoms started gradually and has been there for last 3 years and it is progressive. Initially, it started as inability to write and difficulty in mixing food due to tremulousness of both hands which worsens with activity. Then it progressed to slurred speech and then to gait unsteadiness. Patient also has urinary incontinence for last 2 years. Patient’s symptoms are more in severity for last 6 months. On examination, patient was having orthostatic hypotension, cogwheel rigidity on bilateral wrist movement and pendular knee jerks. All cerebellar signs were present bilaterally including finger nose test abnormality, past pointing, dysdiadochokinesia, heel shin test abnormality, gross truncal ataxia, wide based gait, impaired tandem walking, gaze evoked nystagmus, scanning speech. Presence of cerebellar signs, autonomic disturbances, poor response to syndopa, rapid progression, lack of resting tremor at presentation, symmetrical involvement, early speech and gait involvement - are usually NOT seen in PD. So, evaluated further. MRI Brain T2 showed classical cruciform hyperintensity in pons (Hot cross bun sign) & diffuse cerebellar atrophy. Hence the diagnosis MULTIPLE SYSTEM ATROPHY (MSA-C) - Shy Drager Syndrome. This shows the importance of identifying atypical features in PD.

1. GERONTOCON 2021
Parkinson-Plus Syndrome – Multiple System Atrophy (MSA-C)
Dr.K.Manievelraaman, Junior Resident, Institute of Internal Medicine,
Madras Medical College & Rajiv Gandhi Government General Hospital, Chennai – 03.
 Parkinson-plus syndrome is a neurodegenerative disorder
characterized by features of parkinsonism ( tremor, rigidity,
bradykinesia and gait abnormality ) along with some
additional characteristics that distinguish it from idiopathic
Parkinson’s Disease (PD). It includes PSP, CBD, MSA & DLB.
 MSA (Shy-Drager syndrome) - subdivided mainly as follows:
• Parkinsonian MSA-P (striatonigral degeneration)
• Cerebellar MSA-C (olivopontocerebellar atrophy)
 There is no definitive therapy for MSA. Only symptomatic &
supportive therapy can be provided. Parkinsonian features
may respond to Levodopa, but other features usually don’t.
Overall, it has poor prognosis with mean survival of 7 to 9
years. It’s closest DD is SCA-2.
 FEATURES SUGGESTING DIAGNOSIS OF MSA:
 Presence of cerebellar signs
 Presence of autonomic disturbances
 Poor response to Dopamine
 Rapid progression
 Lack of resting tremor at presentation
 Lack of asymmetry
 Early speech and gait involvement
 Presence of Cruciform hyperintensity
in T2 MRI (Hot-cross bun sign
indicating selective degeneration
of pontocerebellar pathways)
 Diffuse cerebellar atrophy
 A 66-year-old male, farmer, K/C/O T2DM and Parkinson’s
disease on T.Metformin and T.Syndopa for 3 years,
presented to us with complaints of unsteadiness of gait,
dysarthria, bilateral upper limb tremor. These symptoms
started gradually and has been there for last 3 years and it
is progressive.
 Initially, it started as inability to write and difficulty in
mixing food due to tremulousness of both hands which
worsens with activity. Then it progressed to slurred speech
and then to gait unsteadiness.
 Patient also has urinary incontinence for last 2 years.
 Patient’s symptoms are more in severity for last 6 months
 The patient is conscious, oriented, afebrile and comfortable
at rest.
 HR: 88/min, RR: 16/min, SpO2: 98% in RA
 BP: on lying flat: 130/80 mm Hg
on standing: 104/72 mm Hg
NERVOUS SYSTEM EXAMINATION:
 Higher mental functions: Normal
 Cranial nerves examination: Normal
 Motor system:
 Power & Bulk: Normal
 Tone: Mild cogwheel rigidity present in B/L wrist
movements (becoming more apparent on voluntary
movement of contralateral limbs – Activated rigidity
 Sensory examination : Normal
 Reflexes:
 DTR: UL- Normal, LL- Pendular Knee jerk present
 Superficial reflexes: Normal, B/L Plantar flexor
 Cerebellum examination:
 Bilateral Finger-nose-test abnormal
 Bilateral Finger-finger-nose-test abnormal
 Bilateral Past pointing present
 Bilateral Dysdiadochokinesia present
 Bilateral Heel shin test abnormal
 Gross truncal ataxia present
 Wide based gait present; Tandem walking impaired
 Bilateral gaze evoked nystagmus present
 Scanning speech present
 Cranium / Spine: Normal, No meningeal signs
 CVS, RS, ABDOMEN EXAMINATION: Normal
INTRODUCTION
 Parkinson’s Disease (PD) is the second most common
neurodegenerative disorder. It’s seen in elderly population
and its prevalence is increasing globally. It is characterized
by well-known triad of resting tremor, bradykinesia, rigidity
along with many other non-motor manifestations.
 It is important to identify the atypical features which
distinguishes idiopathic PD from Parkinson-Plus Syndromes
and this distinction has therapeutic and more importantly
prognostic implications.
 This is the case report of one such classical case of
Parkinson-Plus Syndrome.
HISTORY
EXAMINATION
 CBC, RFT, LFT, Electrolytes – Normal
 HbA1c – 7.2
 TFT: Normal
 Vitamin B12 levels - Normal
BASIC INVESTIGATIONS
MRI BRAIN – T2 WEIGHTED
DISCUSSION
DIAGNOSIS:MULTIPLE SYSTEM ATROPHY (MSA-c)
CONCLUSION
Although at the outset it appears to be similar to PD, as the
disease unfolds, it becomes apparent that MSA is a different
disease & different outcomes. Pathologically, MSA is a
synucleinopathy with GLIAL cytoplasmic inclusions.
References:
1. Harrison’s Principles of Internal Medicine
2. Bradley’s Neurology in Clinical Practice
CMC MAC 2021