A Middle Aged Man with a Big Head since Childhood

Introduction

A 55 year old man presented with h/o sudden fall in bathroom followed by loss of consciousness for an hour for which he was admitted to a hospital and underwent a CT scan of his head (Figure 1).

Figure 1.

55 year old man CT when admitted to hospital

Following this he was operated for a ventriculo-peritoneal shunt placement (in another hospital even before he presented to our centre) and his attendants reported a few days of improvement of sensorium following the surgery but soon after that the patient passed again into a coma.

When he was brought to our centre he was still comatose and slowly regained consciousness over a few days of conservative management followed by improvement in his cognitive and motor skills within one week and he was able to sit, recognise and talk to his family members.

Patient was again readmitted after a month with continuous partial seizure which was later well controlled on antiepileptic drugs.

His mother and wife informed us that patient had a big head since childhood (Figure 2) but was completely normal otherwise and worked as a clerk in an office.

Figure 2.

Profile of 55 year old man’s head

He had never ever been noticed to have a difficulty in walking or doing any other work with his hands. He has 2 children who are completely normal.

Although it was interesting to think that he had lived his 55 years of life with that strikingly less amount of brain tissue accidentally noticed for the first time we found that such a phenomenon had been reported earlier in the literature (“Man Lives Normal Life”,2007)

Since the past 1 year the patient’s wife related that he had 3 previous episodes of sudden fall that was interpreted by us as transient ischemic attacks. He has Diabetes and Hypertension since 12 years and was fairly controlled on medications.

Discussion

In addition to the large dilated ventricles in a thin layer of cerebral cortices (massive ventriculomegaly) along with increase of the cisterna magna and defect of the cerebellar vermis with a communication of the cyst with the fourth ventricle in his first CT head which was suggestive of the Dandy Walker syndrome, his postoperative CT head scans showed evidence of bilateral subdural hematoma along with the Ventriculo peritoneal shunt in situ. (Figure 3)

Figure 3.

CT scan of head after shunt surgery

Our final impression was stroke in a patient of pre-existing but asymptomatic Dandy Walker syndrome.

Most of the patients with Dandy-Walker syndrome, signs and symptoms caused by abnormal brain development appear within the first year of life with hydrocephalus that causes macrocephaly. Affected individuals typically have intellectual disability that ranges from mild to severe and often have delayed development, particularly a delay in motor skills such as crawling, walking, and coordinating movements.

People with Dandy-Walker syndrome frequently experience muscle stiffness and paralysis of the lower limbs (spastic paraplegia), and they may also have seizures. (Osenbach et al.,1991)

Our patient developed his first symptoms at the age of 55 years possibly due to a stroke related to his atherosclerosis risk factors in the form of hypertension and diabetes and unrelated to his congenital malformation. He also underwent a ventriculo-peritoneal shunt and developed acute bilateral subdural hematoma following the decompression which is again well described in the literature (Salomão et al., 1990).