Agenesis of the Corpus Callosum

Kimberly Burnham
13 min readOct 24, 2020

--

Consider Manual Therapy and Complementary and Alternative Medicine for Optimal Health

Healing can occur with alternative medicine approaches. Here we will look at some of the medical literature about what agenesis of the corpus callosum is and some of the signs and symptoms.

The first consideration is why does the person have agenesis or incomplete development of the corpus callosum. A common reason for a lack of development is insufficient blood flow. So, one consideration is how to encourage or increase blood flow to the brain. There are a number of manual therapy approaches and acupuncture techniques that address blood flow to the brain. Simply using manual therapy and massage to decrease the tension in the muscles of the neck can take pressure off the blood vessels and nerve running between the head and the rest of the body. This improves communication and flow between the heart and the brain.

Also a diagnosis of agenesis of the corpus callosum is not all bad news. The literature supports the idea of healing.

Sometimes people with agenesis [lack of development] of the corpus callosum [the area of the brain which connects the right and left sides] are “only slightly impaired relative to normal subjects.”

―Forster, B., Corballis, P. M., & Corballis, M. C. (2000). Effect of luminance on successiveness discrimination in the absence of the corpus callosum. Neuropsychologia, 38(4), 441–450.

Agenesis of the corpus callosum can be associated with hemangiomas, brain abnormalities, cranial cysts, cranial deformities, arterial dysfunction, eye abnormalities, myelination problems, autonomic nervous system, and olfactory bulb dysfunctions.

Manual Therapy approaches including Cranial Therapy, Integrative Manual Therapy and Osteopathic Manual Therapy have helped address dysfunction and encourage function in many children with brain dysfunctions.

The purpose of this review is to give a skilled manual therapist, not familiar with agenesis of the corpus callosum, an idea about the different anatomic presentations and clinical considerations.

The following looks at some of the cases presentations from the medical literature on agenesis of the corpus callosum, which may be addressed with manual therapies.

Brain Anatomy and Healing with Integrative Manual Therapy

Hemangiomas

“In one case a 2-month-old female had a combination of midfacial hemangioma, with recurrent hemorrhages, absent corpus callosum, hypoplastic internal carotid artery, and an abnormal tortuous dysplastic basilar artery and sinus pericranii.”

―Drosou, A., Benjamin, L., Linfante, I., Mallin, K., Trowers, A., Wakhloo, A. K., et al. (2006). Infantile midline facial hemangioma with agenesis of the corpus callosum and sinus pericranii: another face of the PHACE syndrome. J Am Acad Dermatol, 54(2), 348–352.

Manual therapists use techniques like Advanced Strain and Counterstrain to address the muscle tension in the blood vessel walls. This seems to improve blood flow and improves brain function.

Brain Abnormalities

“A male neonate was admitted because prenatal ultrasonography indicated central nervous system abnormalities. Neurological examination showed no abnormality except for electroencephalographic spike activities. Magnetic resonance imaging revealed a cystic lesion in the left interhemispheric fissure, agenesis of the corpus callosum, and microgyria in the left frontotemporal lobes. Cerebral blood flow (CBF) was diffusely reduced. The cyst wall was partially removed and a cyst-peritoneal shunt procedure was performed. The histological diagnosis was glioependymal cyst. The spike activity disappeared and CBF dramatically improved after the operation.”

―Tange, Y., Aoki, A., Mori, K., Niijima, S., & Maeda, M. (2000). Interhemispheric glioependymal cyst associated with agenesis of the corpus callosum — case report. Neurol Med Chir (Tokyo), 40(10), 536–542.

Cranial Deformities

Cranial Deformities are common (Higashi, 1972) along with cranial cysts such as one case with a large interhemispheric epithelial cyst with a motor paresis of the left arm and progressive enlargement of the cyst on MRI were revealed. The histological diagnosis was a neuroepithelial cyst with the feature of choroid plexus epithelia.”

―Uematsu, Y., Kubo, K., Nishibayashi, T., Ozaki, F., Nakai, K., & Itakura, T. (2000). Interhemispheric neuroepithelial cyst associated with agenesis of the corpus callosum. A case report and review of the literature. Pediatr Neurosurg, 33(1), 31–36.

There can also be feeding and swallowing disorders.

―Ng, Y. T., McCarthy, C. M., Tarby, T. J., & Bodensteiner, J. B. (2004). Agenesis of the corpus callosum is associated with feeding difficulties. J Child Neurol, 19(6), 443–446.

The parents of a child with developmental delay reported after treatment with Integrative Manual Therapy (IMT), “these are the changes that I have observed in S.V. since he started treatment: Speech has improved, fine and gross motor is improved, potty training improved, sleep pattern has improved with less wakening at night, and eyes have improved.”

Manual therapy practitioners have also worked extensively with children with brain tumors, brain trauma, developmental brain dysfunctions and see improvements with the use of gentle hands-on cranial techniques to improve the mobility of the cranial bones.

Aicardi syndrome

“Aicardi syndrome is a rare disorder characterized by absent corpus callosum, infantile spasms, and choriorentinal lacunae. It is sporadic in nature and affects only females, resulting in severe mental and physical handicap.”

— Hoag, H. M., S. A. Taylor, et al. (1997). “Evidence that skewed X inactivation is not needed for the phenotypic expression of Aicardi syndrome.” Hum Genet 100(3–4): 459–64.

Arterial Dysfunctions

In this case a woman lived a relatively normal life despite having agenesis of the corpus callosum. Arterial anomalies, such as hypoplastic internal carotid artery and tortuous dysplastic basilar artery, coarctation of the aorta are reported. One case noted that the agenesis of the corpus callosum wasn’t diagnosed until the woman was 53 years old. The diagnosis was made when the woman was admitted to the hospital with sudden onset of severe headache.

“CT scan showed a marked subarachnoid hemorrhage with intracerebral and intraventricular hematoma, a separation of the lateral ventricles with enlargement of posterior horns, and deformity of anterior horn. By these findings, the patient was diagnosed as an agenesis of the corpus callosum with subarachnoid hemorrhage.”

―Nakamura, N., Ogawa, A., Kayama, T., Sakurai, Y., Yoshimoto, T., & Suzuki, J. (1986). [A case of agenesis of the corpus callosum accompanied by a ruptured azygos anterior cerebral artery aneurysm and lipoma — a case report]. No To Shinkei, 38(7), 701–705.

In conjunction with medical treatment, manual therapy approaches can enhance healing in heart and circulation related issues.

In a case report, the mother of a two month old IMT client with Down’s Syndrome and heart related complications, reports, “the surgeon said he no longer needs heart surgery, it was a miracle that the holes in the heart healed.”

Another study looked at myofascial trigger-point massage therapy to the head, neck and shoulder areas on cardiac autonomic tone. Researchers found, “in normal healthy subjects myofascial trigger-point massage therapy to the head, neck and shoulder areas is effective in increasing cardiac parasympathetic activity and improving measures of relaxation.”

―Delaney, J. P., K. S. Leong, et al. (2002). “The short-term effects of myofascial trigger point massage therapy on cardiac autonomic tone in healthy subjects.” J Adv Nurs 37(4): 364–71.

Reiki, an energetic technique has also been found to benefit autonomic nervous system function. The study found, “heart rate and diastolic blood pressure decreased significantly in the Reiki group compared to both placebo and control groups.” and concluded, “the study indicates that Reiki has some effect on the autonomic nervous system. The results justify further, larger studies to look at the biological effects of Reiki treatment.”

―Mackay, N., S. Hansen, et al. (2004). “Autonomic nervous system changes during Reiki treatment: a preliminary study.” J Altern Complement Med 10(6): 1077–81.

Autonomic Nervous System Dysfunctions

“Spontaneous recurrent hypothermia [low temperatures] and hyperhidrosis [excessive sweating] associated with agenesis of the corpus callosum was first described by Shapiro and Plum in 1967. (Shapiro’s syndrome).”

―Segeren, C. M., Polderman, K. H., & Lips, P. (1997). Agenesis of the corpus callosum associated with paroxysmal hypothermia: Shapiro’s syndrome. Neth J Med, 50(1), 29–35.

IMT practitioners work extensively with the autonomic nervous system.

―Giammatteo, T. and S. Weiselfish-Giammatteo (1997). Integrative manual therapy for the autonomic nervous system and related disorders : utilizing advanced strain and counterstrain technique. Berkeley, Calif., North Atlantic

Noting a relationship to dopamine levels another study reported, “No neurologic or physical abnormalities were noted beside the callosal agenesis. Neuroimaging examinations could not reveal any structural abnormality of the diencephalon. Endocrinological examinations and electroencephalograms were found to be normal. The hyperthermia returned to normal by a small dose of levodopa, but a larger dose reversed the hyperthermia to hypothermia.”

―Hirayama, K., Hoshino, Y., Kumashiro, H., & Yamamoto, T. (1994). Reverse Shapiro’s syndrome. A case of agenesis of corpus callosum associated with periodic hyperthermia. Arch Neurol, 51(5), 494–496.

Parkinson’s disease and hyperthermia with callosal agenesis could have some similarities given the response to levodopa. People with Parkinson’s have improved their symptoms (walking better, improving fine motor control and decreased pain) with manual therapy, massage and acupuncture.

“Episodic hyperhidrosis and hypothermia are the primary symptoms of a rare central nervous system disorder of thermoregulation which is often associated with agenesis of the corpus callosum and can present in childhood or adult years. During attacks, patients may exhibit confused, withdrawn, and lethargic behavior and ataxia or other neurologic symptoms….Episodic thermoregulatory disturbance has been attributed to “vagal attacks” or “diencephalic epilepsy,” but the pathophysiology remains undefined.”

―LeWitt, P. A., R. P. Newman, et al. (1983). “Episodic hyperhidrosis, hypothermia, and agenesis of corpus callosum.” Neurology 33(9): 1122–9.

Olfactory Blub and a Sense of Smell

“A combination between agenesis of the olfactory bulb and agenesis of the corpus callosum has been reported.”

―Naruse, I., & Keino, H. (1993). Induction of agenesis of the corpus callosum by the destruction of anlage of the olfactory bulb using fetal laser surgery exo utero in mice. Brain Res Dev Brain Res, 71(1), 69–74.

Eye Abnormalities

Such as endothelial failure, band-shaped keratopathy and bilateral corneal clouding have been reported.

―Akhtar, S., Bron, A. J., Meek, K. M., & Bennett, K. (2001). Congenital hereditary endothelial dystrophy and band keratopathy in an infant with corpus callosum agenesis. Cornea, 20(5), 547–552.

One study asked, subjects to perform a simple reaction time task in which visual stimuli were either presented singly in one or other visual field, or in both visual fields simultaneously. “Reaction times were faster to double stimuli than to single ones, but in the normal subjects this ‘redundancy gain’ did not exceed that predicted by probability summation (the horse-race model). In the four subjects lacking the corpus callosum, the gain did exceed that predicted by probability summation when the stimuli were brighter than the background, implying subcortical neural summation. In the three surgical cases (L.B., J.W. and M.E.) the gain was greatly diminished when the stimuli were equiluminant with the background, suggesting that neural summation occurred at the collicular level. In normal subjects, callosal transfer may ensure that atleast some degree of interhemispheric neural summation occurs, even with unilateral input. The acallosal subject (R.B.) was anomalous in that neural summation was not diminished by equiluminance.”

―Corballis, M. C. (1998). Interhemispheric neural summation in the absence of the corpus callosum. Brain, 121 ( Pt 9), 1795–1807.

IMT practitioners have worked extensively with children and adults with visual problems and are getting positive changes in acuity (how well the person sees), hand-eye coordination, color vision, visual field improvements and decreases in the pain around the eyes and headache pain.

Another medical case looked at a 38-year-old patient, who had been complaining for some years about impairment in nasal breathing.

“Within the last few months the patient presented a rapid deterioration of his visual acuity. Radiological examinations revealed a sphenopharyngeal encephalocele as well as agenesis of corpus callosum.”

―Mauersberger, W., & Diaz, L. A. (1985). Sphenopharyngeal encephalocele associated with agenesis of the corpus callosum. Neurochirurgia (Stuttg), 28(6), 238–242.

Effect of Alcohol on Corpus Callosum

“Fetal alcohol spectrum disorders constitute a major public health problem. Heavy prenatal alcohol exposure can severely affect the physical and neurobehavioral development of a child. Autopsy and brain imaging studies indicate reductions and abnormalities in overall brain size and shape, specifically in structures such as the cerebellum, basal ganglia, and corpus callosum. A wide range of neuropsychological deficits have been found in children prenatally exposed to alcohol, including deficits in visuospatial functioning, verbal and nonverbal learning, attention, and executive functioning. These children also exhibit a variety of behavioral problems that can further affect their daily functioning. Children exposed to alcohol prenatally, with and without the physical features of fetal alcohol syndrome, display qualitatively similar deficits.”

— Riley, E. P. and C. L. McGee (2005). “Fetal alcohol spectrum disorders: an overview with emphasis on changes in brain and behavior.” Exp Biol Med (Maywood) 230(6).

One way to improve symptoms caused by featal alcohol syndrome is to improve liver function through manual therapy techniques and acupuncture.

Integrative Manual Therapist work with many kinds of visual problems. The following is a report from the parents of a boy with severe visual dysfunction and brain damage, “the special education therapist was amazed at how T.L.’s eyes were fixed to the toys that she had been showing him for months. He followed them with his eyes, and also he was moving his head back and forth to follow it! She also got a kick out of the way his arms could move more freely. She noticed he was very vocal. He held his head up straighter for her. The most important was the Teacher of the Vision Impaired who had identified the Cortical Visual Impairment. She had told us the day before Thanksgiving that T.L. would not be able to identify things he saw and labeled him legally blind. She observed him for an hour this past Tuesday and told us that she had a correction to make on T.L.. We asked her to explain herself. She said, I made a mistake, T.L. is now at the low end of the cortical impairment. She said she saw eye movement she never saw before, which told her his brain was sending the messages that were never there before. We could hardly control our excitement.”

Myelination Problems

A study on “glucocorticoids regulate oligodendrocyte maturation and the myelin biosynthetic pathways” found, “synthetic glucocorticoids, the corticosteroids have been successfully used in clinical practice as a single course to enhance lung maturation and reduce mortality and morbidity in preterm infants with no long-term neurologic or cognitive side effects. However, a trend has arisen to use repeated courses despite an absence of safety data from clinical trials. Our data suggest that repeated prenatal corticosteroid administration delays myelination of the corpus callosum and that further safety data are needed to evaluate clinical practice.”

―Huang, W. L., Harper, et al. (2001). Repeated prenatal corticosteroid administration delays myelination of the corpus callosum in fetal sheep. Int J Dev Neurosci, 19(4), 415–425.

One thing to consider with myelination problems is a gluten elimination diet. Gluten has recently been linked to a number of neurological problems.

Brain Function

“Knowledge concerning the role of the corpus callosum derives from the study of patients with lesions of spontaneous or surgical origin. Three major aspects are defined: interhemispheric elementary transfer of symmetrically organized messages, complex transfer of a symmetrically organized information, interhemispheric transfer and complex behaviors. Symptoms are both complex and rather limited; they can be missed if they are not specifically searched for.”

―Habib, M. (1998). [Corpus callosum disconnection syndromes and functional organization or the corpus callosum in adults]. Neurochirurgie, 44(1 Suppl), 102–109.

Corpus callosum lesions can have a variety of effects on brain function. “The symptoms of dyscopia or dysgraphia could appear in isolation from each other. Combined lesions of the medial parts of the brain (cingulate cortex, frontal lobes) and the corpus callosum significantly increased the level of dysfunction of these medial parts. Patients with lesions to the anterior parts of the corpus callosum showed symptoms of frontal lobe dysfunction. Lesions to the corpus callosum led to dysfunction of the right hemisphere in the spheres of emotion, perception, and spatial activity. Previous studies have demonstrated that the right hemisphere integrates impulses from both sides of the space and is the first zone involved in activity, performing its initial stages. The author believes that this synthetic activity of the right hemisphere, with tight connections with the “conscious” left hemisphere, is required for the formation of the overall conceptualization of both individual objects and particular types of activity. From this point of view, it is the right hemisphere that can, in a sense, be regarded as dominant, rather than the left hemisphere.”

―Buklina, S. B. (2005). The corpus callosum, interhemisphere interactions, and the function of the right hemisphere of the brain. Neurosci Behav Physiol, 35(5), 473–480.

Another study investigated, “the role of the corpus callosum in cognitive and sensorimotor functioning as measured by a neuropsychological test battery.

Researchers concluded, “taken together, the results of the reviewed and personal studies suggest that absence of the corpus callosum does not necessarily impede cognitive functioning. However, samples drawn from clinical populations tend to show a larger variability as to their mental abilities. In keeping with previous findings, our results indicate that the corpus callosum does play a role in bimanual motor coordination although other pathways (probably ipsilateral and/or subcortical) may provide adequate compensation in many cases. The data further suggest that the corpus callosum may be important for interhemispheric transfer of tactuo-motor learning when a spatial component is involved. Finally, our results are consistent with a facilitatory role of the corpus callosum in cognitive and sensorimotor functioning which allows for interhemispheric compensation.”

―Sauerwein, H. C., & Lassonde, M. (1994). Cognitive and sensori-motor functioning in the absence of the corpus callosum: neuropsychological studies in callosal agenesis and callosotomized patients. Behav Brain Res, 64(1–2), 229–240.

Seizures

Partial complex epilepsy has also been reported with a lesion of the corpus callosum: a cavernoma in one case and a lesion of undetermined nature in the other. Two mechanisms could explain such epilepsies: either the lesion extends to the cingulate gyrus whence the epileptic discharge may reach internal temporal structures (our case n. 1), or a temporal focus becomes autonomic after interruption of inhibitory callosal fibres (our case n. 2).”

―Gastaut, J. L., & Bartolomei, F. (1993). [Partial epilepsy and corpus callosum involvement]. Rev Neurol (Paris), 149(6–7), 416–418.

“The relationship between tuberous sclerosis (TS) and epilepsy on one hand and between agenesis of the corpus callosum (ACC) and epilepsy on the other has been recognized for a long time. Until now, a syndrome characterized by TS, ACC and Lennox-Gastaut syndrome (LGS) has not been described in the literature. Three cases of children, all young male patients, who suffer from TS, ACC and LGS, were presented. The ACC was total in two cases and partial in the third. In all subjects tonic and astatic seizures were frequent. Antiepileptic therapy improved the clinical picture in one case, while in the other two it remained unchanged.”

―DeMarco, P. (1992). Tuberous sclerosis, agenesis of the corpus callosum and Lennox-Gastaut syndrome: mere chance or a new syndrome? Clin Electroencephalogr, 23(1), 7–9.

IMT practitioners work with seizures and have seen a decrease in seizure activity in people who are not well controlled by seizure medications. There seems to be a number of things in the brain and in the body that can trigger off seizures. The knowledge and skill of IMT practitioners working with a wide variety of nervous system problems makes Integrative Manual Therapy a good choice of treatment for anyone looking for help improving brain and physical function in agenesis of the corpus callosum and any other nervous system dysfunction.

Originally published in The Burnham Review June 2008.

--

--

Kimberly Burnham
Kimberly Burnham

Written by Kimberly Burnham

(She/Her) Writer, Poet, currently working on a memoir, Mistaken for a Man, a Story for Anyone Struggling to Feel Comfortable in Their Own Skin, Clothes, & ...