Case Vignettes in Neurology

PATIENT CASE STUDIES

Case 42
Case discussion This 52 year-old man presented with an initial history of numbness and weakness of the lower extremities, progressive over the previous six months with intermittent numbness of both hands during the preceding two months. Examination revealed intact mental status and cranial nerves. Motor examination revealed weakness of the wrist and finger extensors on the left and weakness of the intrinsic hand muscles on the right. There was weakness of knee extension on the right and weakness of foot flexion, extension, inversion and eversion bilaterally. Reflexes were absent at the left brachioradialis, right knee and both ankles. Plantar responses were flexor. Sensory testing demonstrated hypalgesia and hypesthesia over the dorsum of the left hand, medial palm and two fingers on the right, the anterior right thigh and both legs and feet. Initial laboratory studies revealed WBC 12,500 with 7% eosinophilia, sedimentation rate of 48mm/hr. and hematuria. Nerve conduction studies of the multiple motor nerves in all extremities were abnormal. The day following admission, the patient's temperature rose to 101°F and he developed severe but transient lower abdominal pain. His right elbow became noticeably erythematous, swollen and painful. At this time, a more meticulous systemic and neurological history was obtained. Although he had complained initially of slowly progressive, generalized weakness and numbness, his first sign of illness had, in reality, occurred quite suddenly. The patient had been a long-distance trucker and his first neurological symptom had been the sudden numbness and paralysis of his right foot and lower leg that developed as he was driving an 18 wheeler down a steep grade in the mountains of West Virginia. He survived the experience but went on to suffer at least four additional episodes of sudden numbness and/or weakness involving: a) right anterior thigh; b) left ankle flexors with numbness on the lateral aspect of his left foot; c) right intrinsic hand muscles with numbness of the fourth and fifth fingers and d) left wrist extensors with numbness of the left hand dorsum. Over the three months following hospital admission, the patient continued to be intermittently febrile. Fundoscopy showed several retinal hemorrhages and a variety of skin eruptions developed. Electrocardiograms showed intermittent conduction defects. Pathologic Case Correlate: Neuropath Case 13
Summarize the Case in 1-2 sentences. This is a 52-year-old man presenting with a history of several episodes over several months of sudden numbness of a portion of a limb or sudden weakness of a muscle group with absent reflexes, corresponding to the distribution of a large peripheral nerve. On admission, he had fever, leukocytosis, eosinophilia, and elevated ESR. good; note a striking feature of the presentation is asymmetrical involvement of individual nerves Discuss lesion localization on the basis of the physical examination. weak wrist & finger extensors, sensory changes on dorsum of hand: left radial n. weak intrinsic hand muscles & sensory changes on medial palm: right ulnar n. weak knee extension, sensory changes on thigh: right femoral n. weak foot flexion, extension, inversion, eversion and sensory changes in legs and feet: bilateral common peroneal n. and tibial n. Leukocytosis and elevated ESR: inflammation hematuria: kidney retinal hemorrhages: vessels good Discuss underlying pathogenesis on the basis of clinical course. Acute onset of deficits suggests a vascular or traumatic cause. Progressive involvement of increasing numbers of peripheral nerves over months suggests an infectious, inflammatory, or metabolic underlying process. good (note course is initially intermittent, acute subsequently step wise) Indicate one likely clinical diagnosis. List (or classify) alternative diagnoses. Mononeuritis multiplex secondary to polyarteritis nodosa Differential diagnosis: * diabetes, sarcoid, RA, SLE, subacute combined degeneration * Sjogren’s syndrome, Wegener’s granulomatosis, Churg-Strauss (eosinophilia) * infectious: Lyme disease, HIV/AIDS good Indicate 2 ancillary tests that would assist in confirming or refuting the clinical diagnosis. Indicate the test results that would confirm the clinical diagnosis. * EMG--denervation changes: fibrillation, increased insertional activity * biopsy of affected nerve, muscle and vessels: inflammatory infiltrate of vessel wall, necrosis of nerve fibers good Indicate complications of the disease and ancillary tests that would help evaluate them. * kidney failure * GI : bowel infarcts & perforations * cardiovascular: arrhythmias, infarction * stroke good Discuss how the underlying pathophysiology is relevant in the management of this patient. Occlusion of small arteries of peripheral nerve and other tissues (esp. GI, kidney) causing infarction and necrosis of nerve fibers. The occlusion is due to inflammation of vessel walls due to immune complex deposition in the vessels. PAN is associated in ~20% of cases with Hep B, suggesting that as one possible antigen causing immune complex formation. Treatment with prednisone and cyclophosphamide induces remission in ~90% of cases, even when treatment is discontinued after remission. Untreated, PAN carries a bleak progrnosis. good

Case 42

Case discussion
This 52 year-old man presented with an initial history of numbness and weakness of the lower extremities, progressive over the previous six months with intermittent numbness of both hands during the preceding two months. Examination revealed intact mental status and cranial nerves. Motor examination revealed weakness of the wrist and finger extensors on the left and weakness of the intrinsic hand muscles on the right. There was weakness of knee extension on the right and weakness of foot flexion, extension, inversion and eversion bilaterally. Reflexes were absent at the left brachioradialis, right knee and both ankles. Plantar responses were flexor. Sensory testing demonstrated hypalgesia and hypesthesia over the dorsum of the left hand, medial palm and two fingers on the right, the anterior right thigh and both legs and feet.

Initial laboratory studies revealed WBC 12,500 with 7% eosinophilia, sedimentation rate of 48mm/hr. and hematuria. Nerve conduction studies of the multiple motor nerves in all extremities were abnormal. The day following admission, the patient's temperature rose to 101°F and he developed severe but transient lower abdominal pain. His right elbow became noticeably erythematous, swollen and painful.

At this time, a more meticulous systemic and neurological history was obtained. Although he had complained initially of slowly progressive, generalized weakness and numbness, his first sign of illness had, in reality, occurred quite suddenly. The patient had been a long-distance trucker and his first neurological symptom had been the sudden numbness and paralysis of his right foot and lower leg that developed as he was driving an 18 wheeler down a steep grade in the mountains of West Virginia. He survived the experience but went on to suffer at least four additional episodes of sudden numbness and/or weakness involving: a) right anterior thigh; b) left ankle flexors with numbness on the lateral aspect of his left foot; c) right intrinsic hand muscles with numbness of the fourth and fifth fingers and d) left wrist extensors with numbness of the left hand dorsum.

Over the three months following hospital admission, the patient continued to be intermittently febrile. Fundoscopy showed several retinal hemorrhages and a variety of skin eruptions developed. Electrocardiograms showed intermittent conduction defects.

Pathologic Case Correlate: Neuropath Case 13

Summarize the Case in 1-2 sentences.

This is a 52-year-old man presenting with a history of several episodes over several months of sudden numbness of a portion of a limb or sudden weakness of a muscle group with absent reflexes, corresponding to the distribution of a large peripheral nerve. On admission, he had fever, leukocytosis, eosinophilia, and elevated ESR.

good; note a striking feature of the presentation is asymmetrical involvement of individual nerves

Discuss lesion localization on the basis of the physical examination.

weak wrist & finger extensors, sensory changes on dorsum of hand: left radial n. weak intrinsic hand muscles & sensory changes on medial palm: right ulnar n. weak knee extension, sensory changes on thigh: right femoral n. weak foot flexion, extension, inversion, eversion and sensory changes in legs and feet: bilateral common peroneal n. and tibial n. Leukocytosis and elevated ESR: inflammation hematuria: kidney retinal hemorrhages: vessels

good

Discuss underlying pathogenesis on the basis of clinical course.

Acute onset of deficits suggests a vascular or traumatic cause. Progressive involvement of increasing numbers of peripheral nerves over months suggests an infectious, inflammatory, or metabolic underlying process.

good (note course is initially intermittent, acute subsequently step wise)

Indicate one likely clinical diagnosis. List (or classify) alternative diagnoses.

Mononeuritis multiplex secondary to polyarteritis nodosa

Differential diagnosis: * diabetes, sarcoid, RA, SLE, subacute combined degeneration * Sjogren’s syndrome, Wegener’s granulomatosis, Churg-Strauss (eosinophilia) * infectious: Lyme disease, HIV/AIDS

good

Indicate 2 ancillary tests that would assist in confirming or refuting the clinical diagnosis. Indicate the test results that would confirm the clinical diagnosis.

* EMG--denervation changes: fibrillation, increased insertional activity
* biopsy of affected nerve, muscle and vessels: inflammatory infiltrate of vessel wall, necrosis of nerve fibers

good

Indicate complications of the disease and ancillary tests that would help evaluate them.

* kidney failure
* GI : bowel infarcts & perforations
* cardiovascular: arrhythmias, infarction
* stroke

good

Discuss how the underlying pathophysiology is relevant in the management of this patient.

Occlusion of small arteries of peripheral nerve and other tissues (esp. GI, kidney) causing infarction and necrosis of nerve fibers. The occlusion is due to inflammation of vessel walls due to immune complex deposition in the vessels. PAN is associated in ~20% of cases with Hep B, suggesting that as one possible antigen causing immune complex formation. Treatment with prednisone and cyclophosphamide induces remission in ~90% of cases, even when treatment is discontinued after remission. Untreated, PAN carries a bleak progrnosis.

good