A Woman With Granulomatous Lesions in the Lungs and a Peripheral Neuropathy

A 71-year-old woman presented initially to her internist with an acute episode of severe cough, mild fever, and weight loss. Chest x-ray and CT scan demonstrated bilateral granulomatous lesions ( Fig. 67-1 ).

Laboratory studies showed a white blood cell count of 4000 cells/μL (normal, 3800–10,000 cells/μL). Hematocrit was 32.3% (normal, 35%–46%). Thyroid-stimulating hormone, B ₁₂ , glycosylated hemoglobin, and fasting blood sugar were normal. Anti-PR3 (antiproteinase 3; circulating antineutrophil cytoplasmic antibody [C-ANCA]) IgG titer was 0.97 EU/mL (normal, <6 EU/mL). The antimyeloperoxidase (anti-MPO; perinuclear ANCA [P-ANCA]) IgG was 0.01 EU/mL (normal, <6 EU/mL).

A lung biopsy showed a granulomatous lesion compatible with Wegener granulomatosis ( Fig. 67-2 ).

She was started on oral cyclophosphamide 100 mg/day orally with the improvement of the cough and resolution of the lung lesions. The drug was discontinued after she developed severe leukopenia. She presented 6 months later with progressive numbness and tingling in the legs and hands.

Past medical history was positive for two C-sections, hemorrhoidectomy, cataract surgery, and hyperlipidemia for which she was taking simvastatin. She did not drink or smoke, and there was no family history of neurologic disorders.

Mentation and cranial nerve examinations were normal. Strength and reflexes were also normal, except for absent ankle jerks. She had decreased vibration sense in the toes, mildly decreased at the ankles, and normal at the knees, with decreased pinprick and pain sensations up to the midcalf. Hand sensation was normal. There were no Babinski signs. Coordination and gait were normal. There was no visceromegaly, and the rest of the examination was normal.

EMG and Quantitative Sensory Tests were Performed

Motor Nerve Studies

Nerve and Site	Latency (ms)	Amplitude (mV)	Conduction Velocity (m/s)
Peroneal Nerve R.	Normal ≤ 5.7	Normal ≥ 3	Normal ≥ 40
Ankle	6.5	7	–
Fibular head	14.3	6	40
Knee	16.3	6	38

Tibial Nerve L.	Normal ≤ 5.3	Normal ≥ 4	Normal ≥ 40
Ankle	4.4	13	–
Pop. fossa	15.7	7	35

Nerve and Site	Latency (ms)	Amplitude (mV)	Conduction Velocity (m/s)
Ulnar Nerve R.	Normal ≤ 3.6	Normal ≥ 8	Normal ≥ 50
Wrist	3.3	11	–
Below elbow	7.0	8	55
Above elbow	9.3	8	52
Axilla	11.4	8	61

F-Wave and Tibial H-Reflex Studies

Nerve	Latency (ms)	Normal Latency ≤ (ms)
Peroneal nerve R.	58.0	54
Tibial nerve L.	66.9	54
Ulnar nerve R.	27.4	30
H-reflex R.	38.1	34
H-reflex L.	NR	34

Sensory Nerve Studies

Nerve	Onset Latency (ms)	Normal Onset Latency ≤ (ms)	Peak Latency (ms)	Normal Peak Latency ≤ (ms)	Amp (μV)	Normal Amp ≥ (μV)	Conduction Velocity (m/s)	Normal Conduction Velocity ≥ (m/s)
Sural nerve R.	3.7	3.5	4.2	4.0	5	11	–	40
Superficial peroneal nerve R.	NR	3.5	NR	4.0	NR	10	NR	40
Ulnar nerve R.	2.3	2.6	2.8	3.1	23	13	52	50

Quantitative Sensory Testing

	Threshold JND	Normal/Abnormal
Vibration Detection Threshold
Hand: 40%	13.5	Normal
Foot: 98%	20.5	Abnormal
Cold Detection Threshold
Hand: 35%	6.75	Normal
Foot: 96%	22.5	Abnormal
Heat Pain Threshold
Hand: 45%	16.5	Normal
Foot: 97%	25.0	Abnormal