Neurology: Case of the Month

History and Physical — Patient 55

History

Present Illness: A 25 year old Hispanic woman presents to the Movement Disorders Clinic at the Methodist Hospital (Baylor College of Medicine) for evaluation of involuntary movements and progressive decline in activities of daily living. Her husband, who provides the history, relates that he first noticed abnormal movements of her head about four months ago, following a near-miss car accident when she turned around quickly to look at her children in the back seat. She began to experience an episodic leftward head tilt, and over the next one to two months, the movements became much more apparent and changed in character. At that time, the patient sought medical attention at a local emergency center, and an examining physician reported leftward head rotation with tongue protrusion, which was felt to be of voluntary origin. Now, the patient's husband reports that her movements have progressed to include episodic movements of her arms and legs as well, described as highly similar in character. He notes that she "sort of sticks out her tongue" every 30 seconds to one minute throughout the day. She has also developed significant difficulty with walking. According to her husband's description, the patient would appear to be walking normally, and then would suddenly arch her back and straighten her legs, resulting in her almost throwing herself into falls. She has actually had a number of falls at home, causing numerous cuts and bruises, but no serious injuries. The patient herself denies any precipitating or alleviating factors. She does not report any premonitory urge, suggestibility, or suppressibility of these movements. Her husband notes no clear alteration of consciousness during these events. Over the last several months, however, he has noticed that the patient seems to "act differently" as well, taking much less interest in her surroundings. He has had to stop work to help care for her, and is feeding and dressing her, and performing all child care duties.

Past Medical History: The patient developed monocular blindness approximately one year prior to the onset of her other symptoms, which has partially resolved. She has no other known medical problems.

Allergies: No known drug allergies.

Medications: Oral contraceptives.

Family History: There is no history of neurologic disease in the family. Her parents are both alive in their early fifties with no known medical problems. Her five siblings are also reported to be in good health. Her two children are ages 10 and 7, with no medical problems.

Social History: There is no history of tobacco or drug abuse, and the patient does not drink alcohol. There is also no history of known toxic exposures, recent foreign travel, or antecedent social stresses.

Review of Systems: There is no history of recent surgeries, psychiatric illnesses or symptoms, fevers or night sweats, weight loss or gain, or depressed mood. There are no cardiac or pulmonary symptoms, skin rashes, or change in skin pigmentation.

Physical Exam

General: This is a listless-appearing, well-nourished Hispanic female in no apparent distress.

Vital Signs: Blood pressure is 100/60 mmHg with a regular pulse of 64/min. She is afebrile.

Neck: Supple with full range of motion and no lymphadenopathy.

Chest: Clear to auscultation.

Cardiovascular: Regular rate and rhythm without murmurs, rubs or gallops appreciated.

Abdomen: Soft, non-tender, non-distended, with no organomegaly.

Extremities: No cyanosis, clubbing or edema.

Skin: No lesions or pigmentary abnormalities appreciated.

Neurological Examination

Mental Status: The interview was performed in Spanish, with the husband and a physician as interpreters. The patient has a flat affect, seldom making eye contact. She appears somewhat indifferent to her situation. She rarely speaks, but when she does talk, her expressive language abilities appear relatively intact. She was unable to complete detailed testing of mental status or praxis.

Speech: Fluent with good repetition and comprehension. Labial, lingual and guttural sounds were normal.

Cranial Nerves: Pupils are equal, round and reactive bilaterally to light. Extraocular movements are full with no nystagmus. Fundoscopic examination is normal bilaterally. Visual acuity in the right eye is 20/20 and fields appear to be intact to controntation. She reports only being able to perceive light with her left eye. However, she does respond to visual threat in the left eye, and follows her image in a mirror with her right eye covered. There is no facial asymmetry. Facial sensation is intact. Hearing is grossly within normal limits. The tongue protrudes at midline and is normal in size and bulk. The palate elevates symmetrically. She has no dysarthria.

Motor: Strength appears approximately 5/5, although she does not fully cooperate with strength testing. Muscle tone is normal throughout. There is no muscle atrophy or fasiculations. The patient demonstrates a persistent left laterocollis. Passive range of motion of her neck is full. There is no associated head tremor, although there does appear to be some hypertrophy of the left sternocleidomastoid. Throughout the examination, she demonstrates frequent, episodic movements consisting of turning of the head in conjunction with abduction of both arms, right more so than the left. During these movements, she extends the elbows and the MCP joints, but flexes her fingers. Each movement lasts approximately two to three seconds, and appears relatively stereotyped. In sitting, there is no involvement of the legs. However, when she stands up, she has vigorous back arching movements with plantar flexion of her ankles, requiring restraint to prevent falling. These movements occurs in either leg, depending on which leg is bearing weight, but appear to be more prominent on the left.

Reflexes: Deep tendon reflexes are 2+ and symmetric. Plantar responses are flexor bilaterally.

Sensation: The patient did not cooperate with formal testing, but demonstrated symmetric withdrawal in all extremities to brief noxious stimulation.

Coordination: The patient demonstrates normal finger to nose testing, except when interrupted by involuntary abduction and extension of the arms.

Gait: As described above. She extends her back and pushes off her legs requiring assistance to prevent falling.

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