We describe a man presented with left sided body weakness and pulmonary nodules and lobar atalectasis.
A 51- year- old Hispanic male, with a history of high blood pressure, was admitted to the hospital with a chief complaint of several day history of slurred speech and of left sided weakness. He recalled having a productive cough, decreased appetite and weight loss in the 2 month period prior to admission. Patient denies any tobacco use and family history was noncontributory. On examination speech was fluent with left sided body weakness with motor 3/5, along with a mild left facial droop. Air entry was good bilaterally along with a systolic murmur. Labs at admission showed mild elevation of WBC 12.5 (75% neutrophils), anemia with a Hb/Hct of 8.6/26.1, platelets count of 854, normal electrolytes/Urea & Creatinine. The urine showed trace protein with 23 RBC.
X-ray of the chest showing right & left lower lobe consolidation (fig 1). After the initial negative CT head the repeated scan in 24 hour showed ischemia in the distribution of the middle cerebral artery (fig 2), later CT scan chest showed multiple lung nodules (fig 3). Bronchoscopy was performed that appeared normal & BAL, transbronchial biopsies were negative for malignancy, PCP, AFB, and fungus. A CT guided biopsy of the lower lobe nodule was inconclusive with histological findings suggesting an inflammatory pseudotumor. Mean while the patient continues spiking and had one episode of mild Epistaxis. All cultures remained negative and additional lab tests revealed an ESR of 130 and a negative ANA, ACE and cardiolipin Ab. C-ANCA was elevated at 32 while the P- ANCA was normal. The patient was then referred for open lung biopsy that showed vasculitis with necrosis of the vessel wall, geographic necrosis and numerous multinucleated giant cells confirming the diagnosis of Wegener’s Granulomatosis. Cyclophosphamide and prednisone therapy were then instituted.
Wegener’s granulomatosis is a systemic vasculitis of the medium and small arteries, as well as the venules, arterioles, and occasionally large arteries. The upper airway disease is the most common presenting feature of WG. This includes sinusitis, otitis media, cough, epistaxis, and saddle nose deformity; Nonspecific complaints of fever, night sweats, anorexia, weight loss, and malaise may accompany upper or lower airway disease. In addition, signs and symptoms related to involvement of other organ systems may also be observed.
The reported frequency of neurological involvement in WG ranges from 22% to 54% and involve both the peripheral nervous system (PNS), manifested as mononeuropathy multiplex, distal symmetrical polyneuropathy and multiple cranial nerves It may manifest as numbness, tingling, shooting pains in the extremities, and sometimes to weakness in a foot, hand, arm, or leg. CNS involvement of WG is rare and literature showed cases with involvement of meninges, pituitary involvement and cerebral vasculitis. Drachman had recognized the following pathophysiologically relevant processes: (a) contiguous spread of granulomatous disease from the ear, nose, and throat tact into the brain and cranial nerves; (b) formation of granulomas primarily in the nervous system; and (c) vasculitis affecting the central nervous system and / or peripheral nervous system. Therapy has reduced mortality form this disease considerably. Multiple different agents and regimens have been proposed. Currently an induction phase of concurrent cyclophosphamide and prednisone is the standard approach.
We believe the neurologic presentation of this patient was secondary to Wegener’s Granulomatosis and to our knowledge this is the first case of WG with stroke manifestation. Tissue diagnosis via biopsy specimens is critical in making the diagnosis of WG.