[Surgical management of syringomyelia associated with Arnold-Chiari malformation, primary IgA deficiency and chromosomal abnormality--a case report].
A rare case of syringomyelia associated with Arnold-Chiari malformation, primary IgA deficiency and sex chromosomal abnormality is reported. A 26-year-old Ethiopian black male was admitted with a complaint of hypalagesia of his left arm and face for 10 years. Neurological examination on admission revealed dissociated sensory loss of his left arm and face. Mild motor weakness of his hand and rotatory nystagmus on left gaze were also noticed. Plain craniogram of lateral view showed small posterior cranial fossa with low positioned inion and platybasia. MRI with T1-weighted images in sagittal plane revealed tonsillar herniation reaching C1 and syrinx extending from C2 to lumbar region. Although no episode of infectious disease nor allergy were experienced, blood analysis disclosed low serum level of IgA (7 mg/dl). The values of other immunoglobulins were within normal range. IgA in saliva was not detected, too. According to the clinical history and symptoms, a diagnosis is of primary asymptomatic IgA deficiency was obtained. Karyotype analysis showed inversion of Y chromosome. In an attempt to avoid anaphylactic shock on blood transfusion in a patient with IgA deficiency, autologous blood was prepared before surgery. Decompressive craniectomy of the posterior fossa with posterior arch of C1 and C2 was performed together with syringosubarachnoid shunt at Th 6-7 level. Postoperative course was successful and slight improvement of sensory disturbance was obtained. No respiratory or wound infection was occurred. The association of these three anomalies is very rare and genetical relationship is not known. From surgical point of view, it is conceivable that preoperative management in a case of asymptomatic IgA deficiency is uneventful.
Keyaki
A
A
Dpartment of Neurosurgery and Neurology, Tenri Hospital, Nara, Japan.
Makita
Y
Y
Nabeshima
S
S
Tei
T
T
Takahashi
J
J
Nioka
H
H
Kawamura
J
J
D000328:Adult; D001139:Arnold-Chiari Malformation / Q000150:complications*; D007446:Chromosome Inversion; D004406:Dysgammaglobulinemia / Q000150:complications*; D006801:Humans; D007070:Immunoglobulin A; D008297:Male; D012729:Sex Chromosome Aberrations / Q000235:genetics*; D013595:Syringomyelia / Q000150:complications* / Q000601:surgery; D014998:Y Chromosome
D007070:Immunoglobulin A