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Sexual dysfunction in amyotrophic lateral sclerosis
In amyotrophic lateral sclerosis the neurones of Onuf’s nucleus in the sacral spinal cord innervating the pelvic floor muscles are relatively spared. Sensory and autonomic functions are also unaffected. Thus, urination defecation and sexual functions are normal, and sexual problems arise solely from the paralysis. Erection and ejaculation through psychogenic stimulation or partner stimulation is possible and the experience of orgasm is normal (Jokelainen & Palo 1976). Kaub-Wittemer et al (2003) found that sexuality was an important issue, even among ventilated patients. Decreased libido and impotence may occur in Kennedy´s syndrome (X-linked bulbospinal muscular atrophy) (Ertekin & Sirin 1993, Hokezu et al 1996).
Sexual dysfunction in spinal cord disorders
If there is a complete destruction of the genital reflex centre in the sacral part of the conus medullaris, reflex erection and reflex lubrication are usually lost and there is complete paresis of the striated ejaculatory muscles. With spinal cord lesion between the level of the lower thoracic segments and the conus, both cerebral and reflex erection and lubrication may be possible, despite the fact that the patient cannot feel the sexual organs. Loss of sacral sensation does not necessarily imply anorgasmia. Although with a complete lesion of the spinal cord above the conus ejaculatory contractions cannot be felt, autonomic components of the orgasm can be experienced. In spinal cord lesion there is often hyperaesthesia just above or at the segment of the lesion. This may be used as an erogenous zone. A meta-analysis of 24 studies (Lundberg et al 2000) of more than 2,500 men with spinal cord injuries showed that a median of 80% (range 54-95%) reported spontaneous erections. The percentage of SCI men reporting ejaculation without therapeutic assistance was much lower (median 15 %, range 0-52 %). Fewer (26 %) of the patients with complete lower sacral lesions had erectile capacity than those with complete upper cord lesions or incomplete lesions at any level (90-99 %) (Bors & Comarr 1960). In cauda equina lesions of various causation only 15% reported normal sexual function (Podnar et al 2002).

The semen of men with spinal cord injuries is characterised by small volume, low sperm count and low spermatic mobility. This is at least partly dependent on insufficient drainage. Ejaculation can be provoked in many paraplegic males through vibratory stimulation or electrostimulation. It has been shown that repeated vibration-induced ejaculations resulted in increased semen volume, a larger number of motile sperms and improved sperm penetration capacity. Insemination with autologous semen obtained in such a way has resulted in pregnancies. Collection of semen very early after the spinal cord injury makes it possible to store semen of good quality for future insemination.

Women with para- or tetraplegia are in a better sexual and reproductive situation than men. Deprived of sensation in the sacral segments they may still reach orgasm through stimulation of other erogenous zones, suprasegmental to the lesion, such as breasts, lips, ears and other areas. Preservation of sensory function in the T11-L2 dermatomes is associated with psychogenically mediated genital vasocongestion (Sipski et al 2001) Deep penetration may provide stimulus enough for orgasm through the sympathetic nervous system or possibly through the vagus nerve. Sexuality, pregnancy, motherhood and quality of life in women with traumatic spinal cord injuries are reviewed by Berard (1989) and Westgren (1999)

Of 224 consecutive male patients referred because of impotence, 17 (31-72 years old) were found to have an unrecognised myelopathy (Brattberg & Lundberg 1991, Lundberg & Brattberg 1992). Morning erections, psychogenic erections and reflex erections were disturbed in most of these men. Disturbances of ejaculation were reported by 10 patients and 7 reported disturbances of the experience of orgasm; in one orgasms were painful. In a study of nearly 2000 patients of both sexes with injuries of the cervical spinal cord without paralysis 85% reported sexual dysfunction (Perese et al 1976). 30% of patients with vitamin B12 deficiency reported sexual dysfunction (Kunze & Leitenmeier 1976).

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