ABSTRACT
Concomitant central nervous system (CNS) involvement in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is rare. Although the spinal nerve roots may present MRI abnormalities in CIDP, hitherto, the spinal cord has been investigated in a single study. We retrospectively investigated clinically and with MRI a cohort of patients with definite CIDP diagnosis (EFNS/PNS criteria) for evidence of brain and spinal cord involvement, who were initially admitted in our department during the last 4 years. Among 12 patients with CIDP (men: 8, mean age: 59.3 years, mean disease duration: 3.8 years), nine patients had their MRI scan during a clinical relapse and three during remission. Brain MRI did not document typical multiple sclerosis lesions in any patient. We did not identify any MRI abnormalities in ten patients without clinical evidence of spinal cord involvement. Conversely, MRI disclosed extensive lesions of the thoracic cord in two patients with an overt spinal cord syndrome, whom we describe. This represents the biggest MRI study of CIDP patients who have been investigated for spinal cord involvement. Our data support earlier observations that a minority of CIDP patients may additionally develop CNS involvement of variable degree.
Subject(s)
Magnetic Resonance Imaging , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/pathology , Spinal Cord/pathology , Female , Humans , Image Processing, Computer-Assisted , Immunoglobulins, Intravenous/therapeutic use , Immunologic Factors/therapeutic use , Male , Middle Aged , Multiple Sclerosis/pathology , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/drug therapy , Steroids/therapeutic useABSTRACT
BACKGROUND: The association of headache with transient neurological deficits and cerebrospinal fluid lymphocytosis (HaNDL) is recognized as a distinct benign, self-limited headache syndrome. Aphasic, sensory and motor disturbances predominate the clinical picture and to our knowledge, only 2 detailed cases of confusion and agitation have been previously described. CASE: We present our recent experience with 2 cases of the HaNDL syndrome who, in addition to the focal neurological deficits, developed confusional state of variable degree, with no signs of aphasia that could jeopardize the clinical picture. An extensive laboratory and neuroimaging work-up excluded all other possible entities and both patients treated conservatively showed an excellent functional recovery. CONCLUSION: We suggest that, although the HaNDL syndrome has a focal plateau, explaining the focal deficits; diffuse manifestations in the form of confusion may well be part of the clinical spectrum of this disorder.
