1. An Unusual Gait Abnormality: Placing hands over head or pulling up pants improves walking Tara Kimbason, PGY 3 Feb 21, 2015

2. Objectives To learn from an unusual gait abnormality case Discuss ways to approach a challenging case Discuss diagnosis and review of the condition

3. Disclosures The authors report no disclosures Tara Kimbason, PGY 3 Mentor: Efrain Perez-Vargas, MD PD: Jonathan Hosey, MD

4. History An 83-year-old right-handed male with h/o well-controlled essential tremors who complains of walking problems that he noticed 5 years ago with slowness and decreased balance Associated with upper and lower extremity weakness, fatigue, intermittent clumsy hands and rigidity “Extreme fatigue" with walking - feels like “a car without an engine” - takes frequent rest periods - worsens with wearing long pants or placing his wallet or keys in his pockets - improves with placing his hands over his head or pulling the waistband off of his body

5. History No head/neck trauma, or head/neck/back pain No difficulty initiating or stopping gait; no falls No visual disturbance, dysarthria, dysphagia No autonomic symptoms No sleep-wake dysfunction No dementia or hallucinations PMHx: essential tremors, depression, hypertension Medications: propranolol, SSRI Allergies: NKDA Social Hx: no smoking or EtOH abuse Family Hx: no h/o vascular, autoimmune, neurologic disorders

6. Focused Neurologic Examination

7. Neurologic Examination: other pertinent negatives Clear mentation and memory No language/speech dysfunction No cogwheel rigidity or dystonia No weakness or sensory deficit

8. Phenomenology A slowly progressive gait difficulty Bradykinesia LE more affected > UE Asymmetry of mild incoordination Postural instability Reduced step length and height Gait improves with sensory tricks

9. Differential Diagnosis Parkinsonism:Idiopathic Parkinson’s disease Progressive supranuclear palsy Multiple system atrophy Corticobasal degeneration Other parkinsonian syndromes Multi-infarct states:Vascular parkinsonism Gait ignition failure NPH Frontal lobe lesions (mass, infarcts) Idiopathic Functional gait disorder with sensory trick Gait apraxia

10. Investigations and Results Thyroid function - wnl MRI of brain and spine - small vessel ischemic changes in white matter - no spinal stenosis or cord enhancement EMG of lower extremities - no evidence of myopathy or motor neuron involvement

11. Parkinsonism Diagnostic Criteria 1.Tremor at rest 2.Bradykinesia* 3.Rigidity 4.Loss of postural reflexes* 5.Flexed posture 6.Freezing (motor blocks) Definite: 2+ (include tremor or bradykinesia) Probable: tremor or bradykinesia Possible : 2+ (3 to 6)

12. Diagnosis Parkinsonian gait Impairment of scaling function and defective internal cueing -amplitude reduction -reduced step height -reduction/abolishment of automatic synkinetic arm swing Probable Parkinson disease Asymmetrical arm swing and step-length Responded to levodopa- carbidopa Gait is more variable due to lack of automaticity

13. Gait Abnormality in Parkinson Disease A dopaminergic deficiency plays a major role in levodopa-responsive cases, possibly in regions outside the putamin – improve bradykinesia and rigidity but not freezing BG dysfunction  failure in generating adequate movement amplitude - “sequence effect” Precise pathophysiology of freezing gait unknown (dysfunction in organized network involving frontal lobe, NE deficiency)

14. Conclusion Diagnosis can be challenging with atypical presentation Correctly diagnosing neurologic cases requires a combination of excellent observational skills, history taking, examinational skills, and appropriate diagnostic evaluations

15. References Fasano, Alfonso MD, PhD; Bloem, Bastiaan R. MD, PhD. CONTINUUM: Lifelong Learning in Neurology: Movement Disorders. October 2013 - Volume 19 - Issue 5, - p 1344–1382 Fahn S and J. Jankovic Principles and Practice of Movement Disorders. Churchill Livingston Elsevier Press 2007. 80-79-82 Nadeau SE. Gait apraxia: further clues to localization. Eur Neurol. 2007;58(3):142- 145 Denes G, mantovan MC, Gallana A, Cappelletti JY. Limb-kinetic apraxia. Mov Disord. 1998 may; 13(3):468-476. PubMed PMID: 9613739 Quencer K, Okun MS, Crucian G, Fernandez HH, Skidmore F, Heilman KM. Limb- kinetic apraxia in Parkinson disease. Neurology 2007;68:150–151. Landau WM, Mink JW. Is decreased dexterity in Parkinson disease due to apraxia? Neurology 2007;68:90–91. Leiguarda R, Marsden CD. Limb apraxias: higher-order disorders of sensorimotor integration. Brain 2000;123:860–879. Leiguarda R. Apraxias as traditionally defined. In: Freund H-J, Jeannerod M, Hallett M, Leiguarda R, eds. Higher-order motor disorders. Oxford: Oxford University Press 2000;303–338. Zadikoff C, Lang AE. Apraxia in movement disorders. Brain 2005;128:1480–1497. Jose M. Ferro, Andrew Kertesz and Cynthia M. Shewan. Apraxia and aphasia: The functional ‐ anatomical basis for their dissociation. Neurology January 1984 34:1

16. Questions? Thank You