A. Ammendola

Peripheral neuropathy in hepatitis-related mixed cryoglobulinemia: electrophysiologic follow-up study.

A retrospective study was performed on 27 patients with hepatitis C (HCV)–related mixed cryoglobulinemia (purpura, arthralgia, hepatitis, glomerulonephritis, peripheral neuropathy) to assess peripheral nerve involvement during follow‐up of up to 8 years. All patients had the same degree of organ/system involvement initially and were clinically evaluated at least annually. All 27 patients received steroids; 15 also received recombinant interferon‐α2b (rIFN‐α2b). At first examination, neurological signs and electrodiagnostic findings consistent with peripheral neuropathy were found in 20 (74%) and in 24 (88.8%) patients, respectively. Neurological evaluation and electrodiagnostic data at 3 and 8 years revealed worsening of neuropathy, whereas the other manifestations of mixed cryoglobulinemia (MC) were stable. At the last examination, clinical and electrodiagnostic signs of neuropathy were found in 25 patients (92.5%), occurring in 1 of 3 patients with normal initial findings, and worsened in 8. A more severe neuropathy was observed in 3 (25%) of the patients treated with prednisone alone and in 6 (40%) of the patients additionally treated with rIFN‐α2b. Our data confirm that in patients with HCV‐related MC, peripheral nerve involvement is frequent, is progressive, and does not seem to benefit by addition of rIFN‐α2b to steroid treatment. Muscle Nerve, 2005

Hirayama disease: three cases assessed by F wave, somatosensory and motor evoked potentials and magnetic resonance imaging not supporting flexion myelopathy

Traumatic chronic injury of the cervical spinal cord caused by neck flexion (“flexion myelopathy”) is one of the suggested pathogenetic mechanisms for Hirayama disease (HD). Neurophysiological data, especially reporting particularly N13 cervical somatosensory response, are scarce and conflicting in HD. F wave, somatosensory evoked potentials (SEP), motor evoked potentials (MEP) and magnetic resonance imaging (MRI) studies were assessed in 3 HD male patients (aged 22, 36, and 51 years) with the aim of evaluating the functional effects of neck flexion in HD. Median and ulnar F waves, median, ulnar and posterior tibial SEP, and upper and lower limb MEP were performed bilaterally in standard conditions and during neck flexion in the patients. Cervical spinal MRI study was performed in standard position in two patients and both in standard and flexed positions in the third patient. F wave, SEP, and MEP findings did not show statistically significant differences in standard conditions and during neck flexion both in HD patients and controls. MRI with neck in standard position was normal in two patients, while in the third patient revealed cervical anterior horns signal changes and cord atrophy. In this patient, MRI with the neck in flexion showed that the spinal cord was normally located, was not compressed within the cervical canal and that there were no abnormalities of the dural sac. These findings suggest that in a complex disorder like HD no definite conclusions can be drawn from the present paper and some cases of HD without evidence of “flexion myelopathy” might have a different pathogenetic mechanism.SommarioLa mielopatia cervicale indotta dalla flessione traumatica del collo è uno dei meccanismi patogenetici ipotizzato per la malattia di Hirayama (MH). In questa malattia i dati neurofisiologici sono scarsi e contraddittori. Lo studio della onda F, dei potenziali evocati somatosensoriali (PES) e motori (PEM) e della risonanza magnetica (RM) spinale cervicale erano valutati in 3 pazienti (rispettivamente di 22, 36, e 51 anni di età) maschi affetti da MH con lo scopo di valutare gli effetti funzionali della flessione del collo nella MH. Lo studio neurofisiologico (onda F, PES e PEM) era attuato bilateralmente con collo in posizione standard ed in flessione. Lo studio della RM spinale cervicale veniva attuato in due pazienti con collo in posizione standard e nel terzo paziente con collo in posizione standard ed in flessione. I risultati degli studi neurofisiologici non mostrano differenze statisticamente significative con collo in posizione standard ed in flessione nei pazienti con MH e nel gruppo di controllo. La RM eseguita con collo in posizione standard era normale in due pazienti, mentre rivelava alterazioni del segnale a livello delle corna anteriori e atrofia del midollo a livello cervicale nel terzo paziente. In questo paziente, la RM attuata con il collo in flessione mostrava il midollo cervicale normalmente localizzato e non compresso nel canale spinale ed il sacco durale senza anormalità. In conclusione, nei nostri pazienti la flessione del collo non mostra significativi cambiamenti dello studio neurofisiologico (onda F, PES e PEM). Questi dati propongono che in un disordine complesso come la MH nessuna definitiva conclusione può essere tratta dall’ attuale lavoro e che alcuni casi di MH senza elettrofisiologiche ed neuroradiologiche evidenze di mielopatia cervicale possono avere un differente meccanismo patogenetico.

Autonomic neuropathy in mixed cryoglobulinemia

A retrospective, cross-sectional study was performed on a series of HCV-related mixed cryoglobulinemia (HCV-MC) patients to assess autonomic neuropathy (AN) and its relation to peripheral neuropathy (PN). Thirty consecutive patients affected by HCV-MC underwent clinical, neurological and electrodiagnostic examinations. Autonomic nervous system (ANS) involvement was assessed by functional cardiovascular tests and sympathetic skin response (SSR) evaluation. Sural nerve biopsy was performed in 10 patients with PN. All patients received steroids, 15 also received recombinant interferon-α2b (RIfn-α2b). PN occurred in 27 patients (90.0%) and AN in 4 (13.3 %) all with signs of PN. SSR was the autonomic test more frequently altered. Biopsy disclosed axonal degeneration more evident in the 4 patients with AN. Three out of 4 patients with AN received steroids and rIFN-α2b and 1 steroids alone. In our study on HCV-MC, it was concluded that AN can occur also without dysautonomic symptoms, SSR appears to be one of the optional tests to use together with dysautonomic tests to identify AN and finally PN and AN do not seem to be positively influenced by addition of rIFN-α2b to steroid treatment.

Clinical and electrodiagnostic follow-up of an adolescent poisoned with thallium

We report a six-year clinical and electrodiagnostic follow-up of an adolescent patient with acute thallium poisoning from attempted suicide. During the acute stage the patient showed gastrointestinal disturbances, alopecia, and clinical and electrodiagnostic signs of severe polyneuropathy. Three years after poisoning, his neurological symptomatology was making progress, and electrophysiologic signs of peripheral neuropathy were mainly confined to lower limbs. Six years after intoxication, he was still complaining of weakness and sensory disturbances at the level of distal lower extremities; his neurologic and electrodiagnostic abnormalities affected mainly the feet. In this case report we underline the importance of early diagnosis and treatment to prevent neurological damage and the role of serial electromyographic and nerve conduction studies in thallium poisoning. These investigations allowed the authors to depict the electrophysiologic course of peripheral nervous system involvement over six years following poisoning.SommarioViene riportato uno studio clinico ed elettrodiagnostico di un giovane paziente con intossicazione acuta da tallio a scopo suicida, seguito per sei anni. Durante la fase acuta il paziente presentava disturbi gastrointestinali, alopecia, e segni clinici ed elettrofisiologici di una severa polineuropatia. Tre anni dopo l’avvelenamento, la sua sintomatologia neurologica migliorava ed i segni elettrofisiologici di neuropatia periferica erano principalmente confinati agli arti inferiori. Sei anni dopo l’intossicazione, il paziente lamentava ancora debolezza e disturbi sensitivi a carico dei tratti distali degli arti inferiori; dove persistevano segni neurologici ed elettrodiagnostici di danno neurologico. In questo caso noi evidenziamo l’importanza di una diagnosi e trattamento precoci per prevenire il danno neurologico ed il ruolo degli studi elettromiografico e di conduzione nervosa nell’avvelenamento da tallio. Queste indagini ci permettevano di descrivere il decorso elettrofisiologico del coinvolgimento del sistema nervoso e di confermare segni elettrodiagnostici di danno neurologico anche dopo sei anni dalla intossicazione.