A 51-year-old woman was diagnosed as
Crow-Fukase syndrome on July 1997, presenting with lymph node swelling,
polyneuropathy,
hepatomegaly,
hypothyroidism, renal dysfunction,
edema and skin change. Lymph node swelling and
polyneuropathy improved in some degree after
chemotherapy. She was admitted to our hospital on march 6, 1998 because of consciousness disturbance, right
hemiparesis and non-
fluent aphasia after
fever and
hypotension. The next day of admission, consciousness disturbance, right
hemiparesis and non-
fluent aphasia disappeared. MR images of the brain revealed low intensity on a T1-weighted image and high intensity on a T2-weighted image in the left parietal lobe. Furthermore, MR images also revealed diffuse hypertrophic dura matter with enhancement by
Gd-DTPA, which made the diagnosis of chronic cranial
pachymeningitis. The cerebral angiographies showed bilateral internal carotid artery occlusion. The cerebrospinal fluid showed normal cell count, total
protein level of 82 mg/dl, and
IgG level of 18 mg/dl. Since there has been very few case reports describing intimate relationship between
Crow-Fukase syndrome and
pachymeningitis, and between carotid occlusion and
pachymeningitis, we speculated that the
pachymeningitis might be associated with
Crow-Fukase syndrome. Furthermore,
pachymeningitis might be a cause of her bilateral carotid occlusion. The number of cases of
Crow-Fukase syndrome associated with
cerebrovascular disease was very rare. This is the first case which had bilateral internal carotid artery occlusion probably caused by chronic cranial
pachymeningitis. Therefore, it is necessary to pay attention to
cerebrovascular disease when the patient of
Crow-Fukase syndrome is associated with
pachymeningitis.