Neurology: Case of the Month

History and Physical — Patient 84

History

Present Illness: Patient is a 15 year old male with diagnosis of acute lymphatic leukemia (ALL) now in remission after completing earlier course of chemotherapy. He is now referred for bilateral weakness of his limbs, with legs being weaker than arms.

He presented initially with weakness of both legs (distal and proximal) that within two weeks progressed to difficulty with standing and walking. He suffered occasional falling episodes. By the third week after symptom onset, the weakness further involved his arms, such that he showed grasping difficulty in his hands. He also experienced feelings of numbness as if "frozen" below the knees bilaterally, and complained of associated calf pain. In his upper extremities, he complained of tingling along the medial aspect of the hands. Gabapentin and amitriptyline were both tried with marginal benefits. He also reported a mild back discomfort with no radiating symptoms, and no bowel/bladder dysfunction. Furthermore, he denied difficulty with vision, hearing, or swallowing.

Few weeks later, he developed shingles on his right side of face accompanied by slight subjective worsening of his overall neurologic symptoms. His opioid requirement for pain control increased. Patient had an episode of unresponsiveness which responded to narcaine.

Treatment History for his ALL: He was diagnosed few months earlier with acute T cell lymphoblastic leukemia with 61.5% leukemic blast cells in the CSF. He previously had failed two earlier attempts with conventional induction, but has been in remission with his latest trial of nelarabine. His initial symptoms of leg weakness started around 2-3 weeks after completing his first cycle of nelarabine treatment. There was no evidence of peripheral blast cells, cell atypia, or CSF blast cells. He was given an antiviral regimen for shingles. Physical and occupational therapies were initiated and resulted in some improvement to the extent that he was able to perform some ambulation with a walker.

Past Medical History: T cell ALL; history of fungal infection involving the liver, spleen and kidneys; mild renal insufficiency with Type IV renal tubular acidosis on potassium replacements; known history of varicella zoster infection with prior reactivation involving right maxillary division of the trigerminal nerve; ADHD and depression; peripheral neuropathy; hypertension; and, Osgood-Schlatter disease.

Allergies: No known drug allergies.

Medications: gabapentin 300 mg t.i.d.; amitriptyline 10 mg at bedtime; sulfamethoxazole/trimethoprim; clonidine; carvedilol; polyethylene glycol (MiraLax); docusate p.r.n.; montelukast; amphotericin-B; pregabalin 100 mg t.i.d.; fluconazole; acyclovir; morphine 15 mg b.i.d.

Family History: No history of neurological diseases.

Social History: He lived with his mother (parents were divorced). He has been attending the 9th grade in school.

Physical Exam

General: He was awake, cooperative, and appropriate in conversation. He appeared frail, though comfortable. Breathing was unlabored.

Vital Signs: Temperature 98.2, Blood Pressure 100-130's/50-70's mmHg, Pulse 70-110, Respiratory Rate 20's.

HEENT: No facial weakness was noted. He had intact sensation in his face with resolving erythematous rashes on right side of face in the trigerminal distribution. Neck was supple.

Cardiovascular: Tachycardia was present with first and second heart sounds heard and no murmurs.

Abdomen: Soft with no organomegaly.

Extremities: Peripheral pulses were present with no edema.

Neurological Examination

Mental Status: Patient was awake, alert oriented to place, person, and situation. Language and speech were normal.

Cranial Nerves: Pupils were equal, round and reactive to light. Eyes appeared conjugate with full range of extraocular movements. Face was symmetric with normal strength of orbicularis oculi and oris. Palatal movements were symmetric with normal gag reflex, and tongue appeared midline. Face turns were strong bilaterally, and shoulder shrugging was symmetric and normal.

Motor: There was no apparent drift, but there was notable diminished fine finger dexterity bilaterally. There was wasting of muscles in the hands and feet. There were no contractures.

Strength

Right

Left

Upper Extremities

  
Deltoid4+4+
Biceps4+4+
Triceps4+4+
Wrist Extensors22
Wrist Flexors  
Finger Extensors22
Finger Flexors22
Hand Intrinsics2-32-3

Lower Extremities

  
Iliopsoas33
Knee Extensors3-43-4
Knee Flexors3-43-4
Ankle Extensors1-21-2
Ankle Flexors1-21-2

 

Reflexes: DTRs were absent in both lower extremities and were trace in both upper extremities.

Sensation: Pinprick and temperature sensations were essentially absent in "stocking" distribution from ankle and down without dysesthesia. Upper extremities paresthesias were described as tingling in both hands symmetrically. No sensory level was identified in the trunk. Position sense was absent distally in both arms and legs, while vibration sense was also impaired (to a lesser degree comparatively) across these same regions.

Coordination: He showed dysmetria upon finger nose testing, although no intention tremors were present. Patient could not perform heel-to-shin maneuver.

Gait: Per history from physical therapy, he was able to stand with one-person support and take few steps with walker.

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