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BMJ Case Rep. 2010; 2010: bcr10.2009.2399.
Published online 2010 April 20. doi:  10.1136/bcr.10.2009.2399
PMCID: PMC3047383
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Ataxia, hypoacusis, and dementia

Description

A 54-year-old man fell from a scaffold without losing consciousness and suffered from headache. Neurological examination revealed right homonymous hemianopsia. Computed tomography (CT) scan of the brain showed left occipital hyperintensity indicating bleeding. He was discharged after 3 days. Six weeks later he lost appetite and slowly developed gait disturbances, hearing deficits, and disorientation. Neurological examination revealed bilateral sensorineural hearing and visual deficits, meningism (without fever), bilateral ataxic finger–nose and heel–shin test, ataxic gait, negative Romberg’s test, dementia (forgetfulness, non-attention, apathy), but no sensible or motor deficits.

CT (fig 1A) and magnetic resonance imaging (MRI) (fig 1B) showed a new, right occipital bleeding and latter a rim of haemosiderin indicating superficial central nervous system (CNS) siderosis (fig 1C). Cerebral angiography was regular. Cerebrospinal fluid (CSF) showed haemosiderophages, free haemoglobin (35 mg/dl), increased erythrocytes (42600/µl), leucocytes (139/µl, normal<5), protein (330 mg/dl, normal <45), lactate (55 mg/dl, normal<20) and decreased glucose (41 mg/dl, normal 50–75). Routine blood tests were regular (complete blood count (CBC), electrolytes, liver and kidney parameters). Pathogens in CSF and vasculitis screening (antinuclear antibody (ANA), antineutrophil cytoplasmic antibody (ANCA)) were negative. Coagulation studies were regular (partial thromboplastin time (PTT), international normalised ratio (INR), antithrombin (AT) III, protein C+S, factor VIII, activated protein C resistance, anticardiolipin antibodies).

Figure 1
Computed tomography (CT) scan (A) and magnetic resonance imaging (MRI) (B) of the patient’s brain demonstrate intracerebral bleeding and superficial central nervous system (CNS) siderosis. Bilateral occipital haemorrhages correspond to hyperdense ...

The patient was treated with antihypertensives, corticosteroids, vitamins C and E, and desferrioxamine.1,2 A third cerebral bleeding (left parietal) caused his death. Autopsy showed subarachnoid haemorrhage, brain herniation, and metastases of an undifferentiated carcinoma (left occipital and right parieto-occipital), but no primary tumour in the whole body.

Superficial CNS siderosis is caused by chronic bleeding into the subarachnoid space.3 The combination of ataxia, hypoacusis and dementia following head trauma has high diagnostic value for superficial CNS siderosis.

Footnotes

Competing interests: None.

Patient consent: Patient/guardian consent was obtained for publication.

REFERENCES

1. Leussink VI, Flachenecker P, Brechtelsbauer D, et al. Superficial siderosis of the central nervous system: pathogenetic heterogeneity and therapeutic approaches. Acta Neurol Scand 2003; 107: 54–61. [PubMed]
2. Levy M, Turtzo C, Llinas RH. Superficial siderosis: a case report and review of the literature. Nat Clin Pract Neurol 2007; 3: 54–8. [PubMed]
3. Koeppen AH, Hurwitz CG, Dearborn RE, et al. Experimental superficial siderosis of the central nervous system: biochemical correlates. J Neurol Sci 1992; 112: 38–45. [PubMed]

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