Present Illness: The patient is a 69 year old right-handed female who presented with complaints of proximal muscle weakness, memory loss, and vertigo. Her initial symptoms began approximately five years ago with difficulty climbing stairs, and rising from a seated position. These progressed slowly, and over the past few years she had difficulty lifting heavy objects, but she was still able to ambulate. About four years ago her husband began to notice intermittent episodes of confusion and progressive memory loss. She often misplaced items, repeated questions, and got lost in familiar environments. Recently, she lost interest in many activities which she previously enjoyed. Over the past two years, she had intermittent episodes of vertigo which lasted about five minutes and occurred two times per week. They were not positional and there was no associated nausea, diplopia, dysarthria, dysphagia, tinnitus, or hearing loss.
She first came to medical attention after she fell at a church function. She was walking when she had a brief episode of vertigo, and fell to the ground. She was unconscious for a few seconds, and was then confused for about 10 minutes. There were no associated autonomic symptoms, incontinence, limb movements, or tongue-biting. She was hospitalized and underwent the following diagnostic evaluation:
Past Medical History: She had a history of "fluid" on the lungs 20 years ago. She was hospitalized for one week and a chest xray was abnormal. She was told that the abnormality was related to pulmonary disease as a child, following measles and whooping cough. A PPD was negative and she was followed by a pulmonologist who felt that she may have had sarcoid, but without evidence of active disease.
Past Surgical History: Total abdominal hysterectomy secondary to endometriosis.
Allergies: Sulfa
Medications: Premarin .625 mg/day
Family History: Peripheral vascular disease, otherwise unremarkable.
Social History: No tobacco, alcohol, or illegal drug use.
Review of Systems: No weight loss, cough, hematuria, melena, or headache.
General: The patient appeared thin and mildly disheveled in appearance. The general examination was within normal limits including a normal cardiac, pulmonary, and breast exam. Pertinent negatives included a supple neck and absence of lymphadenopathy or skin abnormalities.
Vital Signs: BP=156/72, HR=72, T=99.1, RR=12.
Mental Status: The patient was awake, alert, and cooperative. Mini-mental status examination score was 26/30. Speech was fluent with no deficits of repetition or comprehension. No dysarthria. No ideomotor apraxias.
Cranial Nerves:
Cranial Nerves | Findings |
|---|---|
| I | Not tested |
| II | Intact with normal discs bilaterally. |
| III / IV / VI | Extraocular movements were intact with normal OKNs. |
| V | Intact. |
| VII | No facial weakness. |
| VIII | Normal Weber, Rinne, and Barany. |
| IX / X | Intact. |
| XI | Sternocleidomastoid 4/5 bilaterally. |
| XII | Intact. |
Motor: Decreased muscle bulk throughout. Minimal cogwheeling present in both upper extremities. Strength is symmetric throughout: 4/5 in the proximal upper and lower extremities; 4+/5 in the distal upper extremities with the flexors being weaker than the extensors; 4+/5 in the distal lower extremities.
Reflexes: Symmetric and brisk in the biceps, brachial radialis, and patellars. 1+ in the ankles bilaterally. Plantar reflexes are flexor. Absent jaw jerk. Positive glabellar, negative snout and palmomental.
Sensation: Intact to pinprick, light touch, vibration, and proprioception.
Coordination: Mild bilateral dysdiadochokinesia and loss of check on the left; otherwise intact.
Gait: Unsteady, mild decreased arm swing bilaterally. Difficulty with turning. No festination, shuffling, retropulsion or en block turning.
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