Clinical Sciences/Health Conditions
Ivy Ren, MD
Resident Physician
Icahn School of Medicine at Mount Sinai
New York, New York, United States
Katia Rios Rojas, MD
Resident Physician
Icahn School of Medicine at Mount Sinai
New York, New York, United States
Alyssa M. Volmrich, MD
Assistant Professor
Icahn School of Medicine At Mount Sinai
New York, New York, United States
Gerstmann Syndrome
Case Description:
30-year-old high-functioning business analyst with Raynaud's and systemic lupus erythematosus (SLE) presented with days of progressive confusion, right upper extremity (RUE) weakness, and numbness. On admission, she had anomia of low-frequency objects, expressive aphasia with paraphasic errors, mild right pronator drift, and 4/5 RUE strength. Neurology additionally identified right-left confusion, acalculia, and agraphia with deficits in grapheme-phoneme correspondence and phonemic awareness. MRI and CT head revealed subacute left frontal and parietal infarcts. CTA revealed severe left ICA stenosis. DAPT was initiated for suspected accelerated atherosclerosis from SLE. She was then admitted to the Acute Rehabilitation service for Gerstmann syndrome (GS), where she made significant functional gains.
Discussions:
GS is a rare disorder characterized by acalculia, agraphia, finger agnosia, and right-left disorientation. Lesions of the dominant inferior parietal lobule, often involving the angular gyrus, are typically responsible. Early recognition facilitates identification of underlying etiology and early rehabilitation to optimize outcomes. This case illustrates an atypical rehab patient since she has more Speech Language Pathology needs than Physical Therapy or Occupational Therapy needs. Because GS typically has minimal weakness or ataxia or other severe physical manifestations, we had to strongly advocate for acute rehabilitation authorization, during which her right-left orientation and agraphia improved. Since discharge, she continues to progress with outpatient SLP, OT, and neuropsychology though still has lingering acalculia. This is consistent with literature, which finds acalculia has delayed recovery. Without her Acute Rehabilitation admission, she would have had less therapy acutely, less time to coordinate support at home, and would have waited longer to begin outpatient therapy.
Conclusions:
Although GS patients do not have the PT needs seen in typical acute rehabilitation patients, timely targeted therapy for cognitive and functional deficits in acute rehabilitation is essential for meaningful recovery of higher cortical functions and daily activities.