Clinical Presentation: Case History # 3

This 46-year old white woman is referred by her family physician for re-evaluation and treatment plan. Ms. Smith was well until 1974, when she had a bout of apparent optic neuritis  (Q.1) and received retro-orbital steroids. In 1974 she developed some facial numbness and decreased control of her extremities. At that time she also complained of difficulties climbing steps. These symptoms slowly subsided and the patient felt quite well till 1982  (Q.2) when she developed some thoracic sensory abnormalities and decreasing visual capacity due to decreased visual acuity and abnormal visual fields. Following that attack the patient underwent the course of oral steroids with significant improvement of symptoms. Neurologic examination in 1986 was notable for evidence of bilateral optic nerve involvement  (Q.3), a positive Romberg's, incoordination in lower extremities with proprioceptive deficit, and a spastic paraparesis in the lower extremities.  (Q.4) The patient also had a depressed and labile affect at that time. The patient was last seen by a neurologist in 1986 and failed to return for the follow up appointment due to relative stabilization of her course.

Today, 10 years later, we see this patient back in clinic due to dramatic decline in her neurologic status. Ms. Smith states that she remained ambulatory until December of 1995 when she collapsed on the street and had to be admitted to the hospital. Currently the patient resides in a nursing home. Today the patient complains of discomfort in her legs and knees due to permanently flexed posturing  (Q.5) She notes increased tremors with stress and has difficulty feeding herself under this type of situation.

Neurologic Exam: Cranial nerve II -- the patient has visual acuity of 20/400 O.D., and between 20/400 and 20/200 O.S. There is a striking bilateral optic pallor. Extraocular movements reveal impaired conjugate gaze to either side with occasional slight tendency for upbeat nystagmus.

Coordination is significant for marked ataxia in the upper extremities with intention tremor being very prominent.  (Q.6) She has only mild titubation at present.

Motor function exam reveals distal weakness in both upper extremities at about 4/5 strength. In the lower extremities the patient has a paraparesis with the right lower extremity more affected than the left. Strengths in the right lower extremities are: iliopsoas 3-/5, quadriceps and hamstrings 4-/5, muscles of dorsiflexion trace/5. In the left lower extremity the patient has 4/5 strength in the iliopsoas, quadriceps and hamstrings, 4/5 strength in the distal musculature, and 3+/5 in the dorsiflexion.

Deep tendon reflexes are brisk throughout with bilateral extensor toe signs. Sensory exam reveals distal loss of vibratory sense in all four extremities which is mild. Pin prick is intact. No sensory level is detected on the abdomen or thorax.